Choroba kawasakiego

Choroba kawasakiego
Leczenie

Choroba Kawasakiego (CK) to ostre zapalenie naczyń średniej wielkości, które może prowadzić do poważnych powikłań sercowo-naczyniowych, zwłaszcza tętniaków tętnic wieńcowych. Standardowe leczenie obejmuje dożylne podanie immunoglobulin (IVIG) w dawce 2 g/kg masy ciała w infuzji trwającej 8-12 godzin oraz aspirynę, stosowaną w fazie ostrej w dawkach 80-100 mg/kg/dobę lub 30-50 mg/kg/dobę, a następnie w dawce przeciwpłytkowej 3-5 mg/kg/dobę przez 6-8 tygodni. Wczesne podanie IVIG, najlepiej w ciągu pierwszych 7-10 dni od wystąpienia gorączki, zmniejsza ryzyko rozwoju tętniaków z około 25% do poniżej 5%. Aspiryna pełni rolę przeciwzapalną i przeciwpłytkową, choć jej wpływ na powstawanie tętniaków jest dyskusyjny. Leczenie odbywa się w warunkach szpitalnych ze względu na konieczność monitorowania i ryzyko powikłań.

Leczenie choroby Kawasakiego

Podstawowa terapia
Leczenie choroby Kawasakiego opornej na IVIG
Leczenie powikłań sercowo-naczyniowych
Monitorowanie i opieka długoterminowa

Skuteczność leczenia
Podsumowanie terapii choroby Kawasakiego

Kolejne rozdziały

Leczenie choroby Kawasakiego

Choroba Kawasakiego (CK) to ostre, samoograniczające się zapalenie naczyń średniej wielkości, które może prowadzić do poważnych powikłań sercowo-naczyniowych, w tym tętniaków tętnic wieńcowych. Wczesne rozpoznanie i leczenie są kluczowe dla zmniejszenia ryzyka powikłań sercowo-naczyniowych. Leczenie powinno rozpocząć się jak najszybciej po postawieniu diagnozy, najlepiej w ciągu pierwszych 10 dni od wystąpienia gorączki, aby zminimalizować ryzyko uszkodzenia tętnic wieńcowych.¹²³

Standardowe leczenie choroby Kawasakiego ma na celu:⁴⁵
– zmniejszenie stanu zapalnego
– zapobieganie lub ograniczenie uszkodzeń tętnic
– zapobieganie powstawaniu zakrzepów u pacjentów z problemami tętnic wieńcowych
– zapobieganie powikłaniom sercowym

Leczenie choroby Kawasakiego zwykle odbywa się w warunkach szpitalnych ze względu na ryzyko powikłań i konieczność monitorowania stanu pacjenta.⁶⁷

Podstawowa terapia

Standardowe leczenie choroby Kawasakiego obejmuje dożylne podanie immunoglobulin (IVIG) w połączeniu z aspiryną.⁸⁹¹⁰

Immunoglobuliny dożylne (IVIG)

IVIG jest podstawą leczenia choroby Kawasakiego i znacząco zmniejsza ryzyko rozwoju tętniaków tętnic wieńcowych.¹¹¹² Według standardów American Heart Association (AHA) i American Academy of Pediatrics (AAP), IVIG powinien być podany w pojedynczej dawce 2 g/kg masy ciała w infuzji trwającej 8-12 godzin.¹³¹⁴¹⁵

Efektywność IVIG w zapobieganiu tętniakom tętnic wieńcowych jest najwyższa, gdy leczenie rozpoczyna się w ciągu pierwszych 10 dni od wystąpienia objawów, najlepiej w ciągu pierwszych 7 dni. Leczenie IVIG zmniejsza ryzyko rozwoju zmian wieńcowych z około 25% u nieleczonych pacjentów do mniej niż 5%.¹⁶¹⁷

Mechanizm działania IVIG w chorobie Kawasakiego nie jest do końca poznany, ale wiadomo, że zmniejsza on stan zapalny w naczyniach krwionośnych.¹⁸¹⁹ Badania wykazały, że IVIG może obniżać poziom prozapalnych cytokin, takich jak IL-1β, we krwi, co prowadzi do zmniejszenia stanu zapalnego.²⁰

Po podaniu IVIG, u większości dzieci następuje istotna poprawa stanu klinicznego w ciągu 24-36 godzin. Ustępuje gorączka oraz inne objawy choroby.²¹²²

Kwas acetylosalicylowy (aspiryna)

Aspiryna jest drugim podstawowym lekiem w terapii choroby Kawasakiego, stosowanym w połączeniu z IVIG.²³ Działa przeciwzapalnie w fazie ostrej choroby oraz przeciwpłytkowo w fazie podostrej i przewlekłej.²⁴²⁵

W fazie ostrej choroby Kawasakiego stosuje się aspirynę w wysokich lub średnich dawkach:²⁶
– Wysokie dawki: 80-100 mg/kg/dobę w 4 dawkach podzielonych
– Średnie dawki: 30-50 mg/kg/dobę w 4 dawkach podzielonych

Leczenie wysokimi dawkami aspiryny zwykle kontynuuje się do momentu ustąpienia gorączki na co najmniej 48-72 godziny lub do 14 dni od początku choroby.²⁷²⁸

Po ustąpieniu gorączki i objawów ostrej fazy, dawka aspiryny jest zmniejszana do dawki przeciwpłytkowej (3-5 mg/kg raz dziennie). Leczenie niskimi dawkami aspiryny kontynuuje się przez 6-8 tygodni od początku choroby, a u pacjentów z nieprawidłowościami tętnic wieńcowych – do czasu ich ustąpienia.²⁹³⁰³¹

Należy podkreślić, że stosowanie aspiryny u dzieci zazwyczaj jest przeciwwskazane ze względu na ryzyko wystąpienia zespołu Reye’a (rzadkiego, ale zagrażającego życiu schorzenia mogącego wpływać na mózg i wątrobę). W przypadku choroby Kawasakiego korzyści przewyższają jednak potencjalne ryzyko, ale podawanie aspiryny powinno odbywać się wyłącznie pod ścisłym nadzorem lekarza.³²³³

Warto zaznaczyć, że chociaż aspiryna jest standardowym elementem leczenia choroby Kawasakiego, niektóre badania sugerują, że nie ma ona wpływu na rozwój tętniaków tętnic wieńcowych, a jej główną rolą jest przeciwdziałanie tworzeniu się zakrzepów.³⁴³⁵³⁶

Leczenie choroby Kawasakiego opornej na IVIG

Około 10-20% pacjentów z chorobą Kawasakiego nie odpowiada na początkowe leczenie IVIG i aspiryną. Mówi się wówczas o chorobie opornej na IVIG (ang. IVIG-resistant/refractory Kawasaki disease). Jest ona definiowana jako utrzymująca się lub nawracająca gorączka 36 godzin po zakończeniu infuzji IVIG.³⁷³⁸³⁹

Pacjenci z chorobą oporną na IVIG mają zwiększone ryzyko rozwoju tętniaków tętnic wieńcowych, dlatego wymagają dodatkowego leczenia.⁴⁰ Opcje terapeutyczne w tej sytuacji obejmują:

Druga dawka IVIG

Najczęściej stosowaną metodą leczenia choroby Kawasakiego opornej na IVIG jest podanie drugiej dawki IVIG w tej samej dawce (2 g/kg).⁴¹⁴²⁴³

Glikokortykosteroidy

Glikokortykosteroidy są często stosowane jako terapia wspomagająca w leczeniu choroby Kawasakiego opornej na IVIG. Mogą być podawane w połączeniu z drugą dawką IVIG lub jako alternatywa.⁴⁴⁴⁵

Stosowane schematy leczenia glikokortykosteroidami obejmują:⁴⁶⁴⁷
– Metyloprednizolon dożylnie w pulsach (30 mg/kg/dobę) przez 1-3 dni
– Prednizolon doustnie (2 mg/kg/dobę) ze stopniowym zmniejszaniem dawki

Badania wykazały, że dodanie kortykosteroidów do IVIG w przypadku opornej choroby Kawasakiego zmniejsza liczbę pacjentów, których stan nie reaguje na leczenie, oraz obniża ryzyko nieprawidłowości tętnic wieńcowych po miesiącu.⁴⁸⁴⁹

Warto zaznaczyć, że rola kortykosteroidów w początkowym leczeniu choroby Kawasakiego pozostaje kontrowersyjna. Wczesne retrospektywne analizy sugerowały, że kortykosteroidy wiązały się ze zwiększonym ryzykiem tętniaków tętnic wieńcowych. Jednak nowsze badania, w tym metaanaliza przeprowadzona przez Chen i wsp., wykazały, że u pacjentów otrzymujących IVIG i kortykosteroidy rozwinęło się znacznie mniej tętniaków tętnic wieńcowych niż u tych otrzymujących samo IVIG (7,6% vs 18,9%).⁵⁰

Leki biologiczne

W przypadku niepowodzenia leczenia IVIG i glikokortykosteroidami, coraz częściej stosuje się leki biologiczne, w szczególności inhibitory TNF-α.⁵¹⁵²

Infliksymab (Remicade), chimeryczne przeciwciało monoklonalne skierowane przeciwko TNF-α, okazał się skuteczny w leczeniu choroby Kawasakiego opornej na IVIG. Zwykle podaje się go w dawce 5-10 mg/kg w pojedynczej infuzji dożylnej.⁵³⁵⁴

Badanie KIDCARE, największe badanie kliniczne w Stanach Zjednoczonych porównujące metody leczenia choroby Kawasakiego opornej na IVIG, wykazało, że infliksymab w porównaniu z drugą dawką IVIG skuteczniej i szybciej obniżał gorączkę oraz skracał czas hospitalizacji.⁵⁵⁵⁶

Inne leki biologiczne stosowane w terapii choroby Kawasakiego opornej na leczenie to inhibitory IL-1, takie jak anakinra.⁵⁷

Inne metody leczenia

W przypadkach opornych na standardowe metody leczenia stosuje się również inne leki immunosupresyjne:⁵⁸⁵⁹⁶⁰
– Cyklosporyna – inhibitor kalcyneuryny
– Cyklofosfamid – lek cytostatyczny hamujący podziały komórkowe
– Metotreksat – antymetabolit hamujący reduktazę dihydrofolianową

W niektórych przypadkach stosuje się również plazmaferezę, która usuwa cytokiny zapalne z krwi, co może zmniejszyć stan zapalny i zapobiec rozwojowi tętniaków tętnic wieńcowych.⁶¹⁶²

Leczenie powikłań sercowo-naczyniowych

Powikłania sercowo-naczyniowe, szczególnie tętniaki tętnic wieńcowych, są najpoważniejszymi konsekwencjami choroby Kawasakiego. Strategie leczenia tych powikłań zależą od ich nasilenia i obejmują:⁶³⁶⁴

Leczenie przeciwzakrzepowe

Pacjenci z małymi lub umiarkowanymi tętniakami są leczeni aspiryną w niskich dawkach, samodzielnie lub w połączeniu z innymi lekami przeciwpłytkowymi, takimi jak klopidogrel (Plavix) lub dipirydamol.⁶⁵⁶⁶

W przypadku dużych tętniaków (≥8 mm) lub złożonych tętniaków średniej wielkości w pojedynczej tętnicy wieńcowej, oprócz aspiryny stosuje się:⁶⁷⁶⁸⁶⁹
– Heparynę drobnocząsteczkową
– Warfarynę (Coumadin)
– Bezpośrednie doustne antykoagulanty (DOAC)

Najnowsze badania sugerują, że zastosowanie bezpośrednich doustnych antykoagulantów w leczeniu pacjentów z dużymi tętniakami tętnic wieńcowych może być bardziej skuteczne i bezpieczniejsze, wymagać mniejszego monitorowania i powodować mniej działań niepożądanych, jednak konieczne są dalsze badania w celu potwierdzenia tych ustaleń.⁷⁰

W przypadku zakrzepicy tętnic wieńcowych stosuje się leki trombolityczne, takie jak rekombinowany tkankowy aktywator plazminogenu (rtPA), w połączeniu z aspiryną i heparyną.⁷¹⁷²

Leczenie inwazyjne

W ciężkich przypadkach z powikłaniami sercowymi może być konieczne:⁷³
– Angioplastyka tętnic wieńcowych – procedura polegająca na otwarciu zwężonej tętnicy poprzez nadmuchanie małego balonu wewnątrz tętnicy
– Implantacja stentu w niedrożnej tętnicy w celu utrzymania jej drożności
– Pomostowanie aortalno-wieńcowe (CABG) – operacja, podczas której przepływ krwi jest przekierowywany wokół chorej tętnicy wieńcowej poprzez wszczepienie fragmentu naczynia krwionośnego z klatki piersiowej, ramienia lub nogi

Wybór metody leczenia kardiologicznego lub kardiochirurgicznego powinien być dokonywany przez doświadczony zespół kardiologii i kardiochirurgii dziecięcej.⁷⁴

Monitorowanie i opieka długoterminowa

Pacjenci po przebytej chorobie Kawasakiego wymagają regularnego monitorowania w celu wykrycia ewentualnych powikłań sercowo-naczyniowych.⁷⁵⁷⁶

U wszystkich pacjentów zaleca się wykonanie badania echokardiograficznego 6-8 tygodni po początku choroby, a następnie po 6 miesiącach. Dalsze badania kontrolne zależą od obecności lub braku nieprawidłowości tętnic wieńcowych.⁷⁷⁷⁸

Dzieci, u których rozwinęły się tętniaki tętnic wieńcowych, powinny pozostawać pod opieką kardiologa dziecięcego i wymagają długoterminowego leczenia oraz regularnych badań kontrolnych.⁷⁹⁸⁰

Pacjenci, którzy przebyli chorobę Kawasakiego, nawet bez powikłań wieńcowych, powinni prowadzić zdrowy tryb życia, obejmujący:⁸¹⁸²
– Dietę zdrową dla serca
– Regularną aktywność fizyczną
– Kontrolę ciśnienia tętniczego
– Unikanie otyłości i palenia tytoniu

Zaleca się również kontrolę poziomu cholesterolu co 5 lat.⁸³

Skuteczność leczenia

Przy odpowiednim i wczesnym leczeniu, większość dzieci z chorobą Kawasakiego wraca do pełnego zdrowia bez długotrwałych powikłań.⁸⁴⁸⁵⁸⁶

Wczesne leczenie IVIG (w ciągu pierwszych 10 dni od wystąpienia gorączki) zmniejsza ryzyko rozwoju tętniaków tętnic wieńcowych z około 25% u nieleczonych pacjentów do 3-5%.⁸⁷⁸⁸

Bez leczenia śmiertelność choroby Kawasakiego wynosi około 1%, natomiast przy odpowiednim leczeniu ryzyko zgonu zmniejsza się do 0,17%.⁸⁹

Większość dzieci zaczyna czuć się lepiej w ciągu kilku dni po rozpoczęciu leczenia. Pełny powrót do zdrowia zwykle następuje w ciągu 6-8 tygodni od początku choroby.⁹⁰⁹¹

Pacjenci, u których nie rozwinęły się tętniaki tętnic wieńcowych, mają doskonałe rokowanie długoterminowe. Natomiast pacjenci z tętniakami wymagają długotrwałej opieki i mają zwiększone ryzyko powikłań sercowo-naczyniowych w późniejszym życiu.⁹²⁹³

Podsumowanie terapii choroby Kawasakiego

Leczenie choroby Kawasakiego powinno być rozpoczęte jak najszybciej po rozpoznaniu, najlepiej w ciągu pierwszych 10 dni od wystąpienia gorączki. Standardowe leczenie obejmuje dożylne podanie immunoglobulin (IVIG) w dawce 2 g/kg masy ciała oraz aspirynę. IVIG znacząco zmniejsza ryzyko rozwoju tętniaków tętnic wieńcowych, głównego powikłania choroby Kawasakiego.⁹⁴⁹⁵

W przypadku choroby opornej na IVIG, definiowanej jako utrzymująca się lub nawracająca gorączka 36 godzin po infuzji IVIG, stosuje się drugą dawkę IVIG, glikokortykosteroidy, leki biologiczne (infliksymab) lub inne leki immunosupresyjne.⁹⁶⁹⁷

Pacjenci z powikłaniami sercowo-naczyniowymi wymagają długoterminowego leczenia przeciwzakrzepowego oraz regularnego monitorowania kardiologicznego.⁹⁸⁹⁹

Dzięki odpowiedniemu leczeniu większość dzieci z chorobą Kawasakiego wraca do pełnego zdrowia bez długotrwałych powikłań. Kluczowe znaczenie ma wczesna diagnoza i szybkie wdrożenie leczenia, co znacząco zmniejsza ryzyko powikłań sercowo-naczyniowych.¹⁰⁰¹⁰¹

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Materiały źródłowe

#1 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
It’s best to start treatment for Kawasaki disease as early as possible, when your child still has a fever. Treatment for Kawasaki disease often happens in a hospital. The goals of treatment are to lower fever, reduce swelling and prevent heart damage. […] Treatment for Kawasaki disease can include: […] Gamma globulin. A protein called gamma globulin is given through a vein. This treatment lowers inflammation in the blood vessels. It can lower the risk of problems with the heart artery. […] Aspirin. High doses of aspirin might help treat inflammation. Aspirin also can decrease pain, joint swelling and fever. The aspirin dose will likely be lowered once the fever has been gone for 48 hours. […] Once the fever goes down, a child might need to take low-dose aspirin for at least six weeks. This can be longer if a there are problems with the heart artery. Aspirin helps prevent blood clotting.
#2 Kawasaki disease – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598
Kawasaki disease is often treatable. With early treatment, most children get better and have no long-lasting problems. […] Treating Kawasaki disease within 10 days of when it began may reduce the chances of lasting damage to the arteries that supply the heart. […] But, with treatment, few children have lasting damage.
#3 Kawasaki Disease: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/965367-overview
The principal goal of treatment is to prevent coronary artery disease. Intravenous immunoglobulin (IVIG), a purified preparation of gamma globulin, and aspirin are the mainstays of treatment. Patients should be treated with IVIG within 10 days after the onset of fever to prevent the development of cardiac sequelae. […] Other medications that are used variably as adjunctive treatments or for IVIG-resistant KD include corticosteroids, infliximab, cyclophosphamide, methotrexate, and ulinastatin. In addition to aspirin, other anticoagulants are sometimes utilized, including clopidogrel, dipyridamole, warfarin, and heparin. […] Therapy with IVIG should be started within 10 days, and ideally within 7 days, of fever onset to prevent cardiac complications.
#4 Kawasaki Disease: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/13457-kawasaki-disease
With prompt treatment, most children recover in about two months. […] Treatment for Kawasaki disease includes: Immune globulin (IVIG), or human blood proteins you receive by IV. About 10% of children may not respond to the first dose of IVIG and will need a second dose or other medications. […] The goals of Kawasaki disease treatment are to: Reduce inflammation. Prevent or lessen damage to arteries. Prevent blood clots in people with coronary artery issues. Prevent heart complications. […] Yes. But with prompt treatment, most children with Kawasaki disease can recover completely.
#5
https://link.springer.com/article/10.1007/s11908-021-00746-1
Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). […] Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. […] The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80100 mg/kg/day and 3050 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper.
#6
https://www.nhs.uk/conditions/kawasaki-disease/
Kawasaki disease is always treated in hospital. […] It’s best if treatment begins as soon as possible. The sooner treatment starts, the quicker the recovery time and there’s less risk of complications developing. […] Intravenous immunoglobulin (IVIG), a solution of antibodies, and aspirin are the 2 main medicines used to treat Kawasaki disease.
#7 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
The principal goal of treatment for KD is to prevent coronary artery aneurysms and other cardiac complications. The mainstays of treatment for KD are IVIG and aspirin. All patients with KD should be admitted to the hospital for administration of IVIG, echocardiography, initiation of aspirin, and for observation until fevers have resolved. […] IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. […] About 10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended.
#8 Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies
https://pmc.ncbi.nlm.nih.gov/articles/PMC5542821/
AHA and AAP (American Academy of Pediatrics) recommend a combination of aspirin and IVIG for treating acute KD. […] The first description of the use of IVIG in treating KD dates back to the 1980s by Furusho et al. Since then it has been used successfully in treating majority of the cases of KD and has become the standard of treatment. The best response is seen if IVIG treatment is started within 10 days of the onset of symptoms at a single dose of 2g/kg infusion over 12 hours as per AAP and AHA guidelines. […] Aspirin is used in conjunction with IVIG for its anti-inflammatory effect in the acute phase of KD and for its anti-platelet activity during the sub-acute phase. […] AHA and AAP define refractory KD as the one where fever persists beyond 36 hours of treatment with IVIG. These patients can then be treated with either a second dose of IVIG or steroids.
#9 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
The principal goal of treatment for KD is to prevent coronary artery aneurysms and other cardiac complications. The mainstays of treatment for KD are IVIG and aspirin. All patients with KD should be admitted to the hospital for administration of IVIG, echocardiography, initiation of aspirin, and for observation until fevers have resolved. […] IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. […] About 10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended.
#10 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Treatment for acute disease is intravenous immunoglobulin and aspirin. […] If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. […] Corticosteroids may be helpful as adjunctive therapy to IVIG for preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given IVIG, 2 g per kg in a single dose, to prevent coronary artery abnormalities. […] IVIG has a dose-dependent effect, with higher doses given in a single infusion having the greatest effect in preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given high-dose aspirin, 80 to 100 mg per kg per day in four divided doses, until afebrile for 48 to 72 hours. […] Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose).
#11 Kawasaki disease: Initial treatment and prognosis – UpToDate
https://www.uptodate.com/contents/kawasaki-disease-initial-treatment-and-prognosis
Kawasaki disease: Initial treatment and prognosis […] The frequency of CA aneurysm development and associated morbidity and mortality have dramatically decreased as a result of treatment with intravenous immune globulin (IVIG). […] The initial treatment of KD is discussed in this topic review. […] Once the diagnosis of KD has been made (algorithm 1), the next step is to determine the risk of intravenous immune globulin (IVIG) resistance since it is associated with a higher risk of coronary artery (CA) abnormalities (algorithm 2). Patients who do not respond to IVIG treatment may benefit from more aggressive initial therapy for KD. […] No single risk factor identifies a majority of children likely to have an incomplete response to the initial dose of IVIG; therefore, scoring models based upon combinations of risk factors have been developed to predict initial response to IVIG therapy.
#12 Kawasaki Disease in Children | Boston Children’s Hospital
https://www.childrenshospital.org/conditions/kawasaki-disease
The standard treatment for Kawasaki disease is IVIG (intravenous immunoglobulin). It is most effective in preventing long-term heart damage when started within 10 days of the onset of illness. IVIG can shorten the duration of the fever and decrease the risk of developing an abnormal enlargement of the coronary vein (aneurysm). Because IVIG is given intravenously (through a vein), your child will need to stay in the hospital during treatment and until their fever goes away. […] Your childâs clinician may also recommend high doses of aspirin to control inflammation and fever. […] Most children respond to their first treatment with IVIG. Some children continue to have a fever despite IVIG treatment, and may need additional treatment. […] If your child develops coronary problems from Kawasaki disease, they may need to take blood-thinning (anticoagulant) medications â either on a short-term or lifelong basis â to reduce the risk of a clot in the coronary vein.
#13 Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies
https://pmc.ncbi.nlm.nih.gov/articles/PMC5542821/
AHA and AAP (American Academy of Pediatrics) recommend a combination of aspirin and IVIG for treating acute KD. […] The first description of the use of IVIG in treating KD dates back to the 1980s by Furusho et al. Since then it has been used successfully in treating majority of the cases of KD and has become the standard of treatment. The best response is seen if IVIG treatment is started within 10 days of the onset of symptoms at a single dose of 2g/kg infusion over 12 hours as per AAP and AHA guidelines. […] Aspirin is used in conjunction with IVIG for its anti-inflammatory effect in the acute phase of KD and for its anti-platelet activity during the sub-acute phase. […] AHA and AAP define refractory KD as the one where fever persists beyond 36 hours of treatment with IVIG. These patients can then be treated with either a second dose of IVIG or steroids.
#14 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
The principal goal of treatment for KD is to prevent coronary artery aneurysms and other cardiac complications. The mainstays of treatment for KD are IVIG and aspirin. All patients with KD should be admitted to the hospital for administration of IVIG, echocardiography, initiation of aspirin, and for observation until fevers have resolved. […] IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. […] About 10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended.
#15 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. […] Treatment for acute disease is intravenous immunoglobulin and aspirin. If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. […] Corticosteroids may be helpful as adjunctive therapy to IVIG for preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given IVIG, 2 g per kg in a single dose, to prevent coronary artery abnormalities. […] IVIG has a dose-dependent effect, with higher doses given in a single infusion having the greatest effect in preventing coronary abnormalities. […] Acute disease is also marked by inflammation and platelet activation; aspirin is believed to modify the inflammatory state and prevent thrombosis, but it does not appear to impact the development of coronary aneurysms.
#16 Kawasaki Disease | Symptoms, Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/k/kawasaki
Children are usually admitted to the hospital for two to five days. […] Treatment includes intravenous gamma globulin (IVIG), which is an infusion through your childs IV and high-dose aspirin every six hours. […] These medicines help reduce the swelling and inflammation in the blood vessels. IVIG can decrease, but NOT eliminate, the risk of developing coronary aneurysms. […] The infusion is most effective if given within the first 10 days of the illness, decreasing the risk of developing coronary changes from 25% to less than 5%. […] After discharge, your child will continue to take high-dose aspirin until the signs of inflammation and fever have resolved. […] Children continue taking a low-dose aspirin every day for usually six to eight weeks or more. […] Your child will continue to be followed by a cardiologist after Kawasaki disease resolves.
#17 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. […] The main goal of treatment in the acute phase of KD is suppressing systemic inflammation and minimizing the risk of developing CAA. Administration of IVIG is overall recognized as the first-line treatment for KD both in terms of efficacy and safety. […] IVIG at the dose of 2g/kg of body weight is the treatment of choice for KD, preferably given within the 10th day, better if within the 7th day of illness, but as soon as possible after diagnosis. […] Treatment of KD is completed by ASA given at a daily dosage of 30-50mg/kg in the acute phase of KD until 48h after the disappearance of fever, then switched to the anti-platelet dose (3-5mg/kg once daily).
#18 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
It’s best to start treatment for Kawasaki disease as early as possible, when your child still has a fever. Treatment for Kawasaki disease often happens in a hospital. The goals of treatment are to lower fever, reduce swelling and prevent heart damage. […] Treatment for Kawasaki disease can include: […] Gamma globulin. A protein called gamma globulin is given through a vein. This treatment lowers inflammation in the blood vessels. It can lower the risk of problems with the heart artery. […] Aspirin. High doses of aspirin might help treat inflammation. Aspirin also can decrease pain, joint swelling and fever. The aspirin dose will likely be lowered once the fever has been gone for 48 hours. […] Once the fever goes down, a child might need to take low-dose aspirin for at least six weeks. This can be longer if a there are problems with the heart artery. Aspirin helps prevent blood clotting.
#19 IVIG Treatment for Kawasaki Disease | AmeriPharmaâ¢ Specialty
https://ameripharmaspecialty.com/ivig/exploring-ivig-treatment-for-kawasaki-disease/
Fortunately, IVIG treatment is available for Kawasaki disease. With early detection and the correct therapy, most children can recover fully from this condition. […] Treatments for this condition include: […] IVIG or immune globulin injections to prevent heart damage. […] IVIG is an effective treatment for Kawasaki disease. It reduces the severity of symptoms to help your child recover. IVIG treatment can be more effective when paired with a course of aspirin. […] This study concluded that PBL cell death might be involved in the development of Kawasaki disease. It also showed that IVIG helps treat Kawasaki disease by affecting this mechanism. […] After receiving IVIG treatment for Kawasaki disease, the level of this cytokine dropped significantly. These results show that IVIG can help treat KD by reducing IL-1Î² in the blood and lowering inflammation.
#20 IVIG Treatment for Kawasaki Disease | AmeriPharmaâ¢ Specialty
https://ameripharmaspecialty.com/ivig/exploring-ivig-treatment-for-kawasaki-disease/
Fortunately, IVIG treatment is available for Kawasaki disease. With early detection and the correct therapy, most children can recover fully from this condition. […] Treatments for this condition include: […] IVIG or immune globulin injections to prevent heart damage. […] IVIG is an effective treatment for Kawasaki disease. It reduces the severity of symptoms to help your child recover. IVIG treatment can be more effective when paired with a course of aspirin. […] This study concluded that PBL cell death might be involved in the development of Kawasaki disease. It also showed that IVIG helps treat Kawasaki disease by affecting this mechanism. […] After receiving IVIG treatment for Kawasaki disease, the level of this cytokine dropped significantly. These results show that IVIG can help treat KD by reducing IL-1Î² in the blood and lowering inflammation.
#21 Kawasaki Disease – UF Health
https://ufhealth.org/conditions-and-treatments/kawasaki-disease
Children with Kawasaki disease need hospital treatment. Treatment must be started right away to prevent damage to the coronary arteries and heart. […] Intravenous gamma globulin is the standard treatment. It is given in high doses as a single infusion. The child’s condition often gets much better within 24 hours of treatment with IV gamma globulin. […] High-dose aspirin is often given along with IV gamma globulin. […] Even with standard treatment, up to 1 in 4 children may still develop problems in their coronary arteries. In sicker children or those with signs of heart disease, adding corticosteroids is recommended. Tumor necrosis factor (TNF) inhibitors such as infliximab (Remicade) or etanercept (Enbrel) are not recommended for initial treatment. However, there still needs to be better tests to tell which children will benefit from these medicines.
#22 Kawasaki Disease – Pediatrics – Merck Manual Professional Edition
https://www.merckmanuals.com/professional/pediatrics/miscellaneous-disorders-in-infants-and-children/kawasaki-disease
Treatment is aspirin and IV immune globulin. […] Therapy is started as soon as possible, optimally within the first 10 days of illness, with a combination of high-dose IVIG (single dose of 2 g/kg given over 10 to 12 hours) and oral high-dose aspirin 20 to 25 mg/kg orally 4 times a day. […] The aspirin dose is reduced to 3 to 5 mg/kg once a day after the child has been afebrile for 4 to 5 days; some authorities prefer to continue high-dose aspirin until the 14th day of illness. […] Most patients have a brisk response over the first 24 hours of therapy. […] An alternative regimen may benefit patients with cardiac dysfunction who could not tolerate the volume of a 2 g/kg IGIV infusion but may lead to slightly slower resolution of symptoms. […] After the child’s symptoms have abated for 4 to 5 days, aspirin 3 to 5 mg/kg once a day is continued for at least 8 weeks after onset until repeated echocardiographic testing is completed.
#23 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Treatment for acute disease is intravenous immunoglobulin and aspirin. […] If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. […] Corticosteroids may be helpful as adjunctive therapy to IVIG for preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given IVIG, 2 g per kg in a single dose, to prevent coronary artery abnormalities. […] IVIG has a dose-dependent effect, with higher doses given in a single infusion having the greatest effect in preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given high-dose aspirin, 80 to 100 mg per kg per day in four divided doses, until afebrile for 48 to 72 hours. […] Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose).
#24 Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies
https://pmc.ncbi.nlm.nih.gov/articles/PMC5542821/
AHA and AAP (American Academy of Pediatrics) recommend a combination of aspirin and IVIG for treating acute KD. […] The first description of the use of IVIG in treating KD dates back to the 1980s by Furusho et al. Since then it has been used successfully in treating majority of the cases of KD and has become the standard of treatment. The best response is seen if IVIG treatment is started within 10 days of the onset of symptoms at a single dose of 2g/kg infusion over 12 hours as per AAP and AHA guidelines. […] Aspirin is used in conjunction with IVIG for its anti-inflammatory effect in the acute phase of KD and for its anti-platelet activity during the sub-acute phase. […] AHA and AAP define refractory KD as the one where fever persists beyond 36 hours of treatment with IVIG. These patients can then be treated with either a second dose of IVIG or steroids.
#25 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#26 Kawasaki Disease or Incomplete Kawasaki Disease â Antiplatelet Therapy â Clinical Pathway: Emergency, Inpatient | Children’s Hospital of Philadelphia
https://www.chop.edu/clinical-pathway/kawasaki-disease-incomplete-kawasaki-disease-aspirin
Aspirin has been used in the treatment of KD for its anti-inflammatory activity at high doses (early in the disease) and anti-platelet activity at low doses (for chronic thromboprophylaxis). […] There is no evidence that aspirin decreases the incidence of coronary artery abnormalities, and there is no data to suggest that a high vs. moderate dose of aspirin is superior. […] The AHA guidelines state it is reasonable to administer moderate- or high-dose aspirin until the patient is afebrile, although there is no evidence it reduces coronary artery aneurysms. […] This pathway does not recommend high-dose aspirin in those already receiving steroids, since steroids offer a powerful anti-inflammatory effect. […] This pathway suggests transitioning to low-dose ASA after 24 hours to allow for a better assessment of fever and treatment resistance prior to discharge.
#27 Kawasaki Disease | Symptoms, Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/k/kawasaki
Children are usually admitted to the hospital for two to five days. […] Treatment includes intravenous gamma globulin (IVIG), which is an infusion through your childs IV and high-dose aspirin every six hours. […] These medicines help reduce the swelling and inflammation in the blood vessels. IVIG can decrease, but NOT eliminate, the risk of developing coronary aneurysms. […] The infusion is most effective if given within the first 10 days of the illness, decreasing the risk of developing coronary changes from 25% to less than 5%. […] After discharge, your child will continue to take high-dose aspirin until the signs of inflammation and fever have resolved. […] Children continue taking a low-dose aspirin every day for usually six to eight weeks or more. […] Your child will continue to be followed by a cardiologist after Kawasaki disease resolves.
#28 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#29 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. […] The main goal of treatment in the acute phase of KD is suppressing systemic inflammation and minimizing the risk of developing CAA. Administration of IVIG is overall recognized as the first-line treatment for KD both in terms of efficacy and safety. […] IVIG at the dose of 2g/kg of body weight is the treatment of choice for KD, preferably given within the 10th day, better if within the 7th day of illness, but as soon as possible after diagnosis. […] Treatment of KD is completed by ASA given at a daily dosage of 30-50mg/kg in the acute phase of KD until 48h after the disappearance of fever, then switched to the anti-platelet dose (3-5mg/kg once daily).
#30 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Low-dose ASA must be continued until 6-to-8 weeks in children without CAA and continued in children with CAA until the resolution of coronary artery lesions. […] In non-responder patients with KD treatment requires a second infusion of IVIG and – in case of failure – pulses of methylprednisolone (30mg/kg/day) for 3 consecutive days, followed by oral prednisone (2mg/kg/day, then gradually tapered). […] Current evidence supports the use of infliximab as rescue therapy in IVIG- and methylprednisolone-refractory patients with KD; IL-1 blockade with anakinra is highly promising in treating the most dramatically severe multi-refractory patients with KD, with potential benefits also on the cardiovascular complications. […] Recombinant tissue plasminogen activator (rtPA) is the first-choice thrombolytic drug in children with KD complicated by coronary artery thrombosis; the glycoprotein IIb/IIIa inhibitor abciximab may be used in case of thrombosis with high risk of occlusion. […] First-choice cardiologic interventional treatment in patients with KD should be chosen in the shortest time based on the specific experience of a pediatric cardiac surgery team.
#31 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
It’s best to start treatment for Kawasaki disease as early as possible, when your child still has a fever. Treatment for Kawasaki disease often happens in a hospital. The goals of treatment are to lower fever, reduce swelling and prevent heart damage. […] Treatment for Kawasaki disease can include: […] Gamma globulin. A protein called gamma globulin is given through a vein. This treatment lowers inflammation in the blood vessels. It can lower the risk of problems with the heart artery. […] Aspirin. High doses of aspirin might help treat inflammation. Aspirin also can decrease pain, joint swelling and fever. The aspirin dose will likely be lowered once the fever has been gone for 48 hours. […] Once the fever goes down, a child might need to take low-dose aspirin for at least six weeks. This can be longer if a there are problems with the heart artery. Aspirin helps prevent blood clotting.
#32 Kawasaki Disease | MedlinePlus
https://medlineplus.gov/kawasakidisease.html
Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG) antibodies. Antibodies are proteins that your immune system makes to fight infections. […] Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver. […] Treatment usually works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child’s heart, they might need additional medicines, surgery, or other medical procedures.
#33 Kawasaki Disease: Diagnosis & Treatment | NewYork-Presbyterian
https://www.nyp.org/pediatrics/rheumatology/kawasaki-disease/treatment
Kawasaki disease is treated in a hospital where doctors can monitor the child. Treatment should begin as soon as possible to speed up recovery and reduce the risk of complications. Intravenous (IV) immunoglobulin (a solution of antibodies), and aspirin are the main medicines used to treat Kawasaki disease. […] Gamma globulin – Infusion of an immune protein (gamma globulin) through a vein (intravenously or IV) used to reduce inflammation in blood vessels and lower the risk of coronary artery problems. […] Aspirin is usually not given to children because it has been linked to Reye’s syndrome, a rare but life-threatening condition in children. Because of this risk, aspirin is given to children with Kawasaki disease only under the supervision of a health care provider. […] After your child is discharged from the hospital, your healthcare provider will provide recommendations for your childs continued care at home. The child should continue taking any prescribed medicines. Full recovery could take around six to eight weeks after the disease started.
#34 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. […] Treatment for acute disease is intravenous immunoglobulin and aspirin. If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. […] Corticosteroids may be helpful as adjunctive therapy to IVIG for preventing coronary abnormalities. […] Patients with acute Kawasaki disease should be given IVIG, 2 g per kg in a single dose, to prevent coronary artery abnormalities. […] IVIG has a dose-dependent effect, with higher doses given in a single infusion having the greatest effect in preventing coronary abnormalities. […] Acute disease is also marked by inflammation and platelet activation; aspirin is believed to modify the inflammatory state and prevent thrombosis, but it does not appear to impact the development of coronary aneurysms.
#35 Kawasaki Disease or Incomplete Kawasaki Disease â Antiplatelet Therapy â Clinical Pathway: Emergency, Inpatient | Children’s Hospital of Philadelphia
https://www.chop.edu/clinical-pathway/kawasaki-disease-incomplete-kawasaki-disease-aspirin
Aspirin has been used in the treatment of KD for its anti-inflammatory activity at high doses (early in the disease) and anti-platelet activity at low doses (for chronic thromboprophylaxis). […] There is no evidence that aspirin decreases the incidence of coronary artery abnormalities, and there is no data to suggest that a high vs. moderate dose of aspirin is superior. […] The AHA guidelines state it is reasonable to administer moderate- or high-dose aspirin until the patient is afebrile, although there is no evidence it reduces coronary artery aneurysms. […] This pathway does not recommend high-dose aspirin in those already receiving steroids, since steroids offer a powerful anti-inflammatory effect. […] This pathway suggests transitioning to low-dose ASA after 24 hours to allow for a better assessment of fever and treatment resistance prior to discharge.
#36
https://link.springer.com/article/10.1007/s40674-019-00115-z
The primary aim of therapy of Kawasaki disease (KD) is to halt inflammation and prevent development of coronary artery aneurysms (CAAs). […] High-dose aspirin has not been found to have significant effect on development of CAAs in KD. Role of corticosteroids has been suggested in patients with high-risk disease and a recent Cochrane review has recommended addition of oral corticosteroids to all children with KD. […] Intravenous immunoglobulin (2 g/kg) is the standard of care in all patients with KD. However, a proportion of patients may need additional therapy with corticosteroids or infliximab. Infliximab is also the drug of choice for IVIg-resistant KD. […] Infliximab plus infliximab may be considered for patients with KD and CAAs. Recent literature has also suggested the role of infliximab, ant-IL-1 antagonist (anakinra), and cyclosporine in IVIg-resistant KD. […] Patients with giant CAAs or multiple complex medium-sized aneurysms in a single coronary artery need anticoagulation and aspirin.
#37 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#38 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
The principal goal of treatment for KD is to prevent coronary artery aneurysms and other cardiac complications. The mainstays of treatment for KD are IVIG and aspirin. All patients with KD should be admitted to the hospital for administration of IVIG, echocardiography, initiation of aspirin, and for observation until fevers have resolved. […] IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. […] About 10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended.
#39 Refractory Kawasaki disease – UpToDate
https://www.uptodate.com/contents/refractory-kawasaki-disease
Refractory Kawasaki disease […] The treatment of refractory KD is discussed in this review. This population differs from the high-risk KD population who warrant primary intensification of therapy (ie, adjunctive therapy with the first dose of IVIG). […] The major sequelae of KD are dramatically decreased by therapy with intravenous immune globulin (IVIG). […] Persistent or recurrent fever 36 hours after completion of initial intravenous immune globulin (IVIG) therapy up to two weeks after the start of treatment. […] […] […] Approach to treatment of Kawasaki disease after initial treatment with IVIG.
#40 Refractory Kawasaki disease: diagnostic and management challenges | PHMT
https://www.dovepress.com/refractory-kawasaki-disease-diagnostic-and-management-challenges-peer-reviewed-fulltext-article-PHMT
Kawasaki disease (KD), an acute, self-limiting, medium-sized arterial vasculitis, is now the most common cause of acquired heart disease in childhood in the developed world. […] We discuss the diagnosis of KD, predicting resistance to traditional therapy and treatment options in refractory or high-risk disease. […] The mainstay of traditional therapy is IVIG and aspirin therapy. However, there is an increasing incidence of patients who do not respond to IVIG therapy. IVIG-resistant or refractory KD is defined as recrudescent or persistent fever at 24-48 hrs following first IVIG infusion and affects 10-20% of patients. […] Due to the significant morbidity associated with CAA, there has been increasing emphasis on earlier diagnosis and a search for adjunctive and second line therapies in order to decrease treatment failure.
#41 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#42 Kawasaki disease
https://www.rch.org.au/clinicalguide/guideline_index/Kawasaki_Disease/
Kawasaki disease (KD) is a clinical diagnosis that requires prompt recognition and management […] Early treatment with intravenous immunoglobulin (IVIg) has been shown to reduce morbidity and mortality […] INTRAVENOUS IMMUNOGLOBULIN (IVIg): 2 g/kg as a single IV infusion on diagnosis […] IVIg should be given within the first 10 days of illness but should also be given to children diagnosed after 10 days if there is evidence of ongoing fever and/or inflammation […] A second dose of 2 g/kg IVIg should be given to children who do not respond to the first dose, as demonstrated by persistent or recurrent fevers 36 hours after the end of the first IVIg infusion. Seek specialist advice […] CORTICOSTEROIDS: Evidence for indication and optimal dose/duration of adjunctive steroids in the primary treatment of KD is limited
#43 Refractory Kawasaki disease: diagnostic and management challenges | PHMT
https://www.dovepress.com/refractory-kawasaki-disease-diagnostic-and-management-challenges-peer-reviewed-fulltext-article-PHMT
The goals of the management of the acute phase of KD are to stop the inflammation as early as possible in order to limit the risk and severity of coronary artery lesions and to prevent coronary artery thrombosis. […] Intravenous immunoglobulin is initially given as a single dose of 2 g/kg over 8-12 hrs. […] The exact mechanism of IVIG in KD is poorly understood. […] Aspirin was traditionally thought to have two roles in the management of KD; an initial anti-inflammatory effect at high dosage and then an anti-thrombotic effect at lower dosage. […] The current AHA guidelines do not recommend routine use of adjunctive corticosteroids, but rather consideration for high-risk patients. […] An appropriate and established initial therapy is a second 2 mg/kg infusion of IVIG. […] Corticosteroids can be used as a second-line treatment alone or in conjunction with a second dose of IVIG.
#44 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#45 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#46 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
In addition to their use in treatment of IVIG-resistant KD, corticosteroids have been proposed as part of primary therapy. This indication is controversial, however. […] Guidelines from the ACR and the Vasculitis Foundation conditionally recommend the use of adjunctive glucocorticoids with IVIG as initial therapy for patients with acute KD who are at high risk for IVIG resistance or the development of coronary artery aneurysms. […] The roles of other adjunctive therapies, including pentoxifylline and abciximab, have not yet been definitively determined.
#47 Management of Kawasaki disease | Archives of Disease in Childhood
https://adc.bmj.com/content/99/1/74
We suggest that corticosteroids should be considered for patients who have already declared themselves as IVIG-resistant, that is, with ongoing fever, and/or persistent inflammation or clinical signs 48h after receiving IVIG as a single dose of 2g/kg. […] There are emerging animal data case reports suggesting a role for anti-tumour necrosis factor (TNF)- therapy for the treatment of KD. […] Other immunosuppressive agents such as ciclosporin, cyclophosphamide, methotrexate, and plasma exchange, have occasionally been used to treat patients who do not respond to IVIG, steroids and anti-TNF. […] In the convalescent phase of the condition, if aneurysms persist, antiplatelet therapy in the form of low-dose aspirin (25 mg/kg) should be continued long-term until the aneurysms resolve. […] The recommendation regarding timing of immunisations after KD remains unchanged from our 2002 guideline.
#48 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#49 Advances in the treatment of Kawasaki disease – PubMed
https://pubmed.ncbi.nlm.nih.gov/23283289/
Intravenous immunoglobulin (IVIG) and aspirin is the standard initial therapy in the treatment of Kawasaki disease. […] A recent study in a Japanese population of Kawasaki disease patients at high risk for IVIG resistance found that the group receiving steroids, in addition to IVIG and aspirin, had fewer coronary artery abnormalities than the group receiving IVIG and aspirin alone. […] Although most practitioners in the USA use IVIG as a second-line therapy for those Kawasaki disease patients who have persistent or recrudescent fever, promising new therapies are under study. Infliximab and steroids are currently the two agents that have been most studied. However, larger studies and studies in genetically diverse populations are needed.
#50 Management of Kawasaki disease | Archives of Disease in Childhood
https://adc.bmj.com/content/99/1/74
Currently, aspirin at a dose of 30-50 mg/kg/day is recommended during the acute phase of the illness, as this may be better tolerated than higher doses in terms of gastrointestinal and other side effects. […] Corticosteroids are effective treatment for other forms of vasculitis, but early retrospective analyses suggested that corticosteroids were associated with increased risk of CAA. […] Clinical trials evaluating the use of corticosteroids plus IVIG have produced seemingly confusing results. […] Chen et al recently reported a meta-analysis comparing the frequency of CAA in patients treated with IVIG plus corticosteroids or IVIG alone for the primary treatment of KD. […] They found that significantly fewer patients receiving IVIG+corticosteroids developed CAA than those receiving IVIG alone (7.6% vs 18.9%; OR: 0.3; 95% CI 0.20 to 0.46).
#51 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#52 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#53 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#54 Kawasaki Disease
https://www.e-kd.org/archive/view_article?pid=kd-2-1-1
Infliximab, a tumor necrosis factor (TNF)- blocking monoclonal antibody, is regarded as an effective and safe treatment modality for Kawasaki disease (KD). In clinical practice, infliximab administration in patients with KD has been associated with a shortened duration of fever and reduced hospital stays. Patients with KD who were treated with infliximab also showed improved outcomes in terms of resistance to intravenous immunoglobulin (IVIG) and coronary artery lesions (CALs). Many studies suggest that infliximab is beneficial not only as a second-line treatment for refractory KD but also as part of first-line intensification treatment with IVIG. Infliximab at a dose of 10 mg/kg/day appears to be more effective in treating refractory KD and reducing CALs compared to a 5 mg/kg/day dose. […] Infliximab has been used both in combination with IVIG as an intensification of initial treatment and as a second-line option for refractory KD. However, no consensus exists regarding the optimal treatment for high-risk refractory KD patients.
#55 Comparing Two Treatments for Resistant Kawasaki Disease – Evidence Update for Parents | PCORI
https://www.pcori.org/evidence-updates/comparing-two-treatments-resistant-kawasaki-disease
Compared with children who received a second dose of IVIG, children who received infliximab were more likely to have their fever end within 24 hours. Children who received infliximab also had shorter fevers and spent less time in the hospital than children who received a second dose of IVIG. […] More children who took infliximab (77%) had their fever end within 24 hours compared with the children who had a second dose of IVIG (51%). […] About 33 percent of children who had IVIG as their second treatment developed anemia, or a low red blood cell count. […] If your child has resistant KD, their clinician will try to start treatment as quickly as possible to prevent damage to your child’s heart. You may want to ask the clinician these questions: What treatment options are available for my child? Can you go over how the two treatments – infliximab or a second dose of IVIG – differ in their possible harms and side effects?
#56 Kawasaki Disease – Children’s Hospital of Orange County
https://choc.org/heart/kawasaki-disease/
CHOC participated in a recent study comparing the effectiveness and safety of Infliximab and a second infusion of intravenous immunoglobulin (IVIG) for patients who do not respond to initial IVIG treatment. The study found that Infliximab resulted in a shorter duration of fever, reduced need for additional therapy, less severe anemia, and shorter hospitalization compared to a second IVIG infusion. We are hopeful about the new insights this study has uncovered and look forward to being a part of continued advancements in treating Kawasaki disease.
#57 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Low-dose ASA must be continued until 6-to-8 weeks in children without CAA and continued in children with CAA until the resolution of coronary artery lesions. […] In non-responder patients with KD treatment requires a second infusion of IVIG and – in case of failure – pulses of methylprednisolone (30mg/kg/day) for 3 consecutive days, followed by oral prednisone (2mg/kg/day, then gradually tapered). […] Current evidence supports the use of infliximab as rescue therapy in IVIG- and methylprednisolone-refractory patients with KD; IL-1 blockade with anakinra is highly promising in treating the most dramatically severe multi-refractory patients with KD, with potential benefits also on the cardiovascular complications. […] Recombinant tissue plasminogen activator (rtPA) is the first-choice thrombolytic drug in children with KD complicated by coronary artery thrombosis; the glycoprotein IIb/IIIa inhibitor abciximab may be used in case of thrombosis with high risk of occlusion. […] First-choice cardiologic interventional treatment in patients with KD should be chosen in the shortest time based on the specific experience of a pediatric cardiac surgery team.
#58 Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies
https://pmc.ncbi.nlm.nih.gov/articles/PMC5542821/
In view of the role of TNF- in the pathogenesis of KD as well as coronary artery dilatations, it is plausible to use its inhibitors in the primary therapy for KD or in refractory cases. […] In KD, inflammatory mediators are elevated and their elevation is a risk factor for developing CAAs. So, plasma exchange (PE), which removes various inflammatory cytokines could serve as a promising treatment option for preventing CAAs. […] Cyclosporine acts as a calcineurin inhibitor. Since recent studies have shown the involvement of IPTKC/calcineurin pathway in the pathogenesis of KD, therefore, blocking of this pathway could be a useful treatment option for refractory KD patients. […] Cyclophosphamide is an immunosuppressive drug that is believed to reduce inflammation in KD. It inhibits DNA synthesis and cross linking of DNA strands, thereby preventing cell division. […] Methotrexate (MTX) is primarily used as an anticancer drug. It is an antimetabolite that inhibits the enzyme Dihydrofolate reductase required for DNA synthesis and replication.
#59 Refractory Kawasaki disease: diagnostic and management challenges | PHMT
https://www.dovepress.com/refractory-kawasaki-disease-diagnostic-and-management-challenges-peer-reviewed-fulltext-article-PHMT
Statins lower low-density lipoprotein cholesterol, improve endothelial function, reduce oxidative stress and inhibit inflammation. […] Cyclophosphamide, methotrexate and plasma exchange have also been used in treatment-resistant cases of KD. […] The diagnosis of KD remains a challenge and it should always be considered in cases of prolonged fever, particularly in the infant with irritability. […] Identifying patients at high risk of refractory KD and thus CAAs is a research priority in order to identify high-risk patients early in the disease course who may benefit from more aggressive treatment.
#60 Management of Kawasaki disease | Archives of Disease in Childhood
https://adc.bmj.com/content/99/1/74
We suggest that corticosteroids should be considered for patients who have already declared themselves as IVIG-resistant, that is, with ongoing fever, and/or persistent inflammation or clinical signs 48h after receiving IVIG as a single dose of 2g/kg. […] There are emerging animal data case reports suggesting a role for anti-tumour necrosis factor (TNF)- therapy for the treatment of KD. […] Other immunosuppressive agents such as ciclosporin, cyclophosphamide, methotrexate, and plasma exchange, have occasionally been used to treat patients who do not respond to IVIG, steroids and anti-TNF. […] In the convalescent phase of the condition, if aneurysms persist, antiplatelet therapy in the form of low-dose aspirin (25 mg/kg) should be continued long-term until the aneurysms resolve. […] The recommendation regarding timing of immunisations after KD remains unchanged from our 2002 guideline.
#61 Kawasaki Disease: Etiopathogenesis and Novel Treatment Strategies
https://pmc.ncbi.nlm.nih.gov/articles/PMC5542821/
In view of the role of TNF- in the pathogenesis of KD as well as coronary artery dilatations, it is plausible to use its inhibitors in the primary therapy for KD or in refractory cases. […] In KD, inflammatory mediators are elevated and their elevation is a risk factor for developing CAAs. So, plasma exchange (PE), which removes various inflammatory cytokines could serve as a promising treatment option for preventing CAAs. […] Cyclosporine acts as a calcineurin inhibitor. Since recent studies have shown the involvement of IPTKC/calcineurin pathway in the pathogenesis of KD, therefore, blocking of this pathway could be a useful treatment option for refractory KD patients. […] Cyclophosphamide is an immunosuppressive drug that is believed to reduce inflammation in KD. It inhibits DNA synthesis and cross linking of DNA strands, thereby preventing cell division. […] Methotrexate (MTX) is primarily used as an anticancer drug. It is an antimetabolite that inhibits the enzyme Dihydrofolate reductase required for DNA synthesis and replication.
#62 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy (adjusted OR = 0.16; 95% CI, 0.09 to 0.31) and lowered the risk of coronary artery abnormalities at one month (adjusted OR = 0.40; 95% CI, 0.27 to 0.90). […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] A case series of 125 patients with Kawasaki disease refractory to IVIG who were later treated with plasma exchange found that patients without coronary artery abnormalities at the start of therapy remained lesion free during follow-up, whereas 12 of 14 patients with coronary dilatation and two of six patients with aneurysms at the start of exchange experienced symptom resolution.
#63 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#64 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Heparin and warfarin (Coumadin) are reserved for treating larger aneurysms, and coronary thrombosis is treated with thrombolytic agents in conjunction with aspirin and heparin. […] The 2004 AHA guideline provides recommendations for the long-term management and surveillance of cardiovascular risk in individuals with Kawasaki disease; these are echoed in the 2010 Japanese Circulation Society Joint Working Group guidelines.
#65 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#66 Kawasaki Disease or Incomplete Kawasaki Disease â Antiplatelet Therapy â Clinical Pathway: Emergency, Inpatient | Children’s Hospital of Philadelphia
https://www.chop.edu/clinical-pathway/kawasaki-disease-incomplete-kawasaki-disease-aspirin
If coronary artery z-score measures 5, discuss with cardiology about increasing anti-platelet therapy with clopidogrel (Plavixreg) in addition to aspirin. […] If coronary artery z-score measures 10, discuss with cardiology about the initiation of systemic anticoagulation (LMWH or warfarin). […] For patients with increased risk of thrombosis (large or giant aneurysms and recent history of coronary artery thrombosis), triple therapy with antiplatelet medications, 2nd antiplatelet agent and anticoagulation may be considered. […] Ibuprofen and other NSAIDs may be harmful in patients taking ASA for its antiplatelet effects and should be avoided. […] There is no evidence that ASA decreases aneurysms or that outcomes are different with low- vs. high-dose ASA. […] High-dose ASA may help with inflammation initially. […] Low-dose ASA is necessary for thrombosis prevention and should be continued through discharge, with length of therapy.
#67
https://link.springer.com/article/10.1007/s40674-019-00115-z
The primary aim of therapy of Kawasaki disease (KD) is to halt inflammation and prevent development of coronary artery aneurysms (CAAs). […] High-dose aspirin has not been found to have significant effect on development of CAAs in KD. Role of corticosteroids has been suggested in patients with high-risk disease and a recent Cochrane review has recommended addition of oral corticosteroids to all children with KD. […] Intravenous immunoglobulin (2 g/kg) is the standard of care in all patients with KD. However, a proportion of patients may need additional therapy with corticosteroids or infliximab. Infliximab is also the drug of choice for IVIg-resistant KD. […] Infliximab plus infliximab may be considered for patients with KD and CAAs. Recent literature has also suggested the role of infliximab, ant-IL-1 antagonist (anakinra), and cyclosporine in IVIg-resistant KD. […] Patients with giant CAAs or multiple complex medium-sized aneurysms in a single coronary artery need anticoagulation and aspirin.
#68 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Heparin and warfarin (Coumadin) are reserved for treating larger aneurysms, and coronary thrombosis is treated with thrombolytic agents in conjunction with aspirin and heparin. […] The 2004 AHA guideline provides recommendations for the long-term management and surveillance of cardiovascular risk in individuals with Kawasaki disease; these are echoed in the 2010 Japanese Circulation Society Joint Working Group guidelines.
#69 Early detection, intensive treatment critical for high-risk patients with Kawasaki Disease | American Heart Association
https://newsroom.heart.org/news/early-detection-intensive-treatment-critical-for-high-risk-patients-with-kawasaki-disease
Intravenous immunoglobulin (IVIG), a mixture of antibodies and proteins to help fight infections, remains the standard of care for patients with Kawasaki Disease. Studies have shown, however, that dosing adjustments based on lean body mass in patients with obesity can help reduce the risks of complications. […] Recent studies have found adding other therapies, such as corticosteroids or infliximab, can help children with Kawasaki Disease who are at high risk for IVIG resistance. These new options may be effective in preventing coronary artery complications and offer health care professionals more options in managing treatment-resistant cases. […] For children with Kawasaki Disease who have larger coronary aneurysms, blood-thinning medications like aspirin (typically 81 mg daily) combined with anticoagulants, such as warfarin or low molecular weight heparin, may help prevent dangerous blood clots. Recent evidence indicates that using direct oral anticoagulants in the treatment of patients with large coronary artery aneurysms may be more effective and safer, require less monitoring and have fewer side effects, however, further research is needed to confirm these findings.
#70 Early detection, intensive treatment critical for high-risk patients with Kawasaki Disease | American Heart Association
https://newsroom.heart.org/news/early-detection-intensive-treatment-critical-for-high-risk-patients-with-kawasaki-disease
Intravenous immunoglobulin (IVIG), a mixture of antibodies and proteins to help fight infections, remains the standard of care for patients with Kawasaki Disease. Studies have shown, however, that dosing adjustments based on lean body mass in patients with obesity can help reduce the risks of complications. […] Recent studies have found adding other therapies, such as corticosteroids or infliximab, can help children with Kawasaki Disease who are at high risk for IVIG resistance. These new options may be effective in preventing coronary artery complications and offer health care professionals more options in managing treatment-resistant cases. […] For children with Kawasaki Disease who have larger coronary aneurysms, blood-thinning medications like aspirin (typically 81 mg daily) combined with anticoagulants, such as warfarin or low molecular weight heparin, may help prevent dangerous blood clots. Recent evidence indicates that using direct oral anticoagulants in the treatment of patients with large coronary artery aneurysms may be more effective and safer, require less monitoring and have fewer side effects, however, further research is needed to confirm these findings.
#71 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Heparin and warfarin (Coumadin) are reserved for treating larger aneurysms, and coronary thrombosis is treated with thrombolytic agents in conjunction with aspirin and heparin. […] The 2004 AHA guideline provides recommendations for the long-term management and surveillance of cardiovascular risk in individuals with Kawasaki disease; these are echoed in the 2010 Japanese Circulation Society Joint Working Group guidelines.
#72 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Low-dose ASA must be continued until 6-to-8 weeks in children without CAA and continued in children with CAA until the resolution of coronary artery lesions. […] In non-responder patients with KD treatment requires a second infusion of IVIG and – in case of failure – pulses of methylprednisolone (30mg/kg/day) for 3 consecutive days, followed by oral prednisone (2mg/kg/day, then gradually tapered). […] Current evidence supports the use of infliximab as rescue therapy in IVIG- and methylprednisolone-refractory patients with KD; IL-1 blockade with anakinra is highly promising in treating the most dramatically severe multi-refractory patients with KD, with potential benefits also on the cardiovascular complications. […] Recombinant tissue plasminogen activator (rtPA) is the first-choice thrombolytic drug in children with KD complicated by coronary artery thrombosis; the glycoprotein IIb/IIIa inhibitor abciximab may be used in case of thrombosis with high risk of occlusion. […] First-choice cardiologic interventional treatment in patients with KD should be chosen in the shortest time based on the specific experience of a pediatric cardiac surgery team.
#73 Kawasaki disease: Symptoms, causes, and treatment
https://www.medicalnewstoday.com/articles/164533
If there are any indications of heart problems, the doctor may order follow-up tests, usually 6 to 8 weeks after symptoms started. […] The following may be necessary: anticoagulant medications, such as warfarin, heparin, or aspirin, to prevent blood clots; coronary artery angioplasty, a procedure opens up an artery that has narrowed by inflating a small balloon inside the artery; alongside the angioplasty, a stent may be placed in a clogged artery to help prop it open, reducing the risk of it blocking again. […] In a coronary artery bypass graft, blood flow is rerouted round a diseased coronary artery by grafting a section of blood vessel from the chest, arm or leg to use as the alternate route.
#74 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Low-dose ASA must be continued until 6-to-8 weeks in children without CAA and continued in children with CAA until the resolution of coronary artery lesions. […] In non-responder patients with KD treatment requires a second infusion of IVIG and – in case of failure – pulses of methylprednisolone (30mg/kg/day) for 3 consecutive days, followed by oral prednisone (2mg/kg/day, then gradually tapered). […] Current evidence supports the use of infliximab as rescue therapy in IVIG- and methylprednisolone-refractory patients with KD; IL-1 blockade with anakinra is highly promising in treating the most dramatically severe multi-refractory patients with KD, with potential benefits also on the cardiovascular complications. […] Recombinant tissue plasminogen activator (rtPA) is the first-choice thrombolytic drug in children with KD complicated by coronary artery thrombosis; the glycoprotein IIb/IIIa inhibitor abciximab may be used in case of thrombosis with high risk of occlusion. […] First-choice cardiologic interventional treatment in patients with KD should be chosen in the shortest time based on the specific experience of a pediatric cardiac surgery team.
#75 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
If your child has any signs of heart problems, the healthcare professional might suggest follow-up tests to check your child’s heart health. Tests are often done 6 to 8 weeks after the illness began, and then again after six months. […] If heart problems keep on, your child might be sent to a specialist who treats heart disease in children, called a pediatric cardiologist. Treatment for heart issues linked to Kawasaki disease depends on the type of heart condition.
#76
https://www.healthychildren.org/English/health-issues/conditions/heart/Pages/Kawasaki-Disease.aspx
Children treated for Kawasaki Disease are sent home from the hospital on a low dose of aspirin to take by mouth every day for 6 to 8 weeks. […] It is very important to closely monitor children who have had Kawasaki Disease to make sure they are improving and to check for the development of coronary aneurysms. […] Children treated for Kawasaki disease who do not develop aneurysms have an excellent long-term outcome. However, it is important for them to follow a heart healthy diet and lifestyle. Cholesterol levels should be checked every 5 years. […] Children who do have coronary aneurysms should be under the care of a pediatric cardiologist as they grow and will require special long-term care and follow-up.
#77 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
If your child has any signs of heart problems, the healthcare professional might suggest follow-up tests to check your child’s heart health. Tests are often done 6 to 8 weeks after the illness began, and then again after six months. […] If heart problems keep on, your child might be sent to a specialist who treats heart disease in children, called a pediatric cardiologist. Treatment for heart issues linked to Kawasaki disease depends on the type of heart condition.
#78 Kawasaki disease: Symptoms, causes, and treatment
https://www.medicalnewstoday.com/articles/164533
If there are any indications of heart problems, the doctor may order follow-up tests, usually 6 to 8 weeks after symptoms started. […] The following may be necessary: anticoagulant medications, such as warfarin, heparin, or aspirin, to prevent blood clots; coronary artery angioplasty, a procedure opens up an artery that has narrowed by inflating a small balloon inside the artery; alongside the angioplasty, a stent may be placed in a clogged artery to help prop it open, reducing the risk of it blocking again. […] In a coronary artery bypass graft, blood flow is rerouted round a diseased coronary artery by grafting a section of blood vessel from the chest, arm or leg to use as the alternate route.
#79
https://www.healthychildren.org/English/health-issues/conditions/heart/Pages/Kawasaki-Disease.aspx
Children treated for Kawasaki Disease are sent home from the hospital on a low dose of aspirin to take by mouth every day for 6 to 8 weeks. […] It is very important to closely monitor children who have had Kawasaki Disease to make sure they are improving and to check for the development of coronary aneurysms. […] Children treated for Kawasaki disease who do not develop aneurysms have an excellent long-term outcome. However, it is important for them to follow a heart healthy diet and lifestyle. Cholesterol levels should be checked every 5 years. […] Children who do have coronary aneurysms should be under the care of a pediatric cardiologist as they grow and will require special long-term care and follow-up.
#80 Kawasaki Disease Clinic – Seattle Children’s
https://www.seattlechildrens.org/clinics/heart/treatments-and-services/kawasaki-clinic/
Your childs doctor will work with you to create a plan of care for your child. We carefully manage your childs treatment so they get the medicines and other care they need. […] All children who have had Kawasaki disease need regular check-ups to see if any new problems develop. Some may need ongoing care for long-term issues. […] Seattle Childrens Kawasaki disease research program, led by Dr. Michael Portman, is working to improve diagnosis and treatment so more children receive timely, effective care and recover fully.
#81 Kawasaki Disease Symptoms, Cause, Criteria, Treatment & Complications
https://www.emedicinehealth.com/kawasaki_disease/article_em.htm
Kawasaki disease is the most common cause of acquired heart disease in children in the developed world. When diagnosed and treated early with gamma globulin, the incidence of coronary artery lesions decreases from 20% to 3%. It is very uncommon for patients who have no evidence of coronary abnormalities at 2-3 months after the acute illness to develop coronary abnormalities. Patients with larger coronary lesions have the greatest risk, and it has been shown that patients with giant aneurysms (8mm) have a highest risk of developing future heart attacks (myocardial infarctions). The long-term risk of patients with small aneurysms is currently unknown. Establishment and adherence to a heart healthy diet, daily physical exercise, control of blood pressure, and avoiding obesity and smoking are goals for all but especially those with potential risk factors for cardiovascular disease.
#82
https://www.healthychildren.org/English/health-issues/conditions/heart/Pages/Kawasaki-Disease.aspx
Children treated for Kawasaki Disease are sent home from the hospital on a low dose of aspirin to take by mouth every day for 6 to 8 weeks. […] It is very important to closely monitor children who have had Kawasaki Disease to make sure they are improving and to check for the development of coronary aneurysms. […] Children treated for Kawasaki disease who do not develop aneurysms have an excellent long-term outcome. However, it is important for them to follow a heart healthy diet and lifestyle. Cholesterol levels should be checked every 5 years. […] Children who do have coronary aneurysms should be under the care of a pediatric cardiologist as they grow and will require special long-term care and follow-up.
#83
https://www.healthychildren.org/English/health-issues/conditions/heart/Pages/Kawasaki-Disease.aspx
Children treated for Kawasaki Disease are sent home from the hospital on a low dose of aspirin to take by mouth every day for 6 to 8 weeks. […] It is very important to closely monitor children who have had Kawasaki Disease to make sure they are improving and to check for the development of coronary aneurysms. […] Children treated for Kawasaki disease who do not develop aneurysms have an excellent long-term outcome. However, it is important for them to follow a heart healthy diet and lifestyle. Cholesterol levels should be checked every 5 years. […] Children who do have coronary aneurysms should be under the care of a pediatric cardiologist as they grow and will require special long-term care and follow-up.
#84 Kawasaki Disease: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/13457-kawasaki-disease
With prompt treatment, most children recover in about two months. […] Treatment for Kawasaki disease includes: Immune globulin (IVIG), or human blood proteins you receive by IV. About 10% of children may not respond to the first dose of IVIG and will need a second dose or other medications. […] The goals of Kawasaki disease treatment are to: Reduce inflammation. Prevent or lessen damage to arteries. Prevent blood clots in people with coronary artery issues. Prevent heart complications. […] Yes. But with prompt treatment, most children with Kawasaki disease can recover completely.
#85 Kawasaki disease – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598
Kawasaki disease is often treatable. With early treatment, most children get better and have no long-lasting problems. […] Treating Kawasaki disease within 10 days of when it began may reduce the chances of lasting damage to the arteries that supply the heart. […] But, with treatment, few children have lasting damage.
#86 About Kawasaki Disease | Kawasaki Disease | CDC
https://www.cdc.gov/kawasaki/about/index.html
Treatment for KD is available, and it must be given at the hospital. It typically combines a mixture of antibodies given through your veins (intravenous immunoglobulin) and aspirin. The treatment can help make symptoms less severe and reduce the risk of serious complications. Healthcare professionals may recommend additional treatments. […] Most children recover with proper treatment.
#87 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
The principal goal of treatment for KD is to prevent coronary artery aneurysms and other cardiac complications. The mainstays of treatment for KD are IVIG and aspirin. All patients with KD should be admitted to the hospital for administration of IVIG, echocardiography, initiation of aspirin, and for observation until fevers have resolved. […] IVIG relieves acute inflammation and has been shown to reduce the rate of CAAs from approximately 25% in untreated patients to 3-5% in treated patients. Maximal benefits are seen when IVIG is given within the first 10 days after the onset of fever. […] About 10-15% of patients will fail initial treatment with IVIG; treatment failure is defined as a fever occurring 36 hours or longer after IVIG is administered. In many of these cases, a second treatment with IVIG at the original dose is recommended.
#88 Kawasaki Disease | Symptoms, Diagnosis & Treatment
https://www.cincinnatichildrens.org/health/k/kawasaki
Children are usually admitted to the hospital for two to five days. […] Treatment includes intravenous gamma globulin (IVIG), which is an infusion through your childs IV and high-dose aspirin every six hours. […] These medicines help reduce the swelling and inflammation in the blood vessels. IVIG can decrease, but NOT eliminate, the risk of developing coronary aneurysms. […] The infusion is most effective if given within the first 10 days of the illness, decreasing the risk of developing coronary changes from 25% to less than 5%. […] After discharge, your child will continue to take high-dose aspirin until the signs of inflammation and fever have resolved. […] Children continue taking a low-dose aspirin every day for usually six to eight weeks or more. […] Your child will continue to be followed by a cardiologist after Kawasaki disease resolves.
#89 Kawasaki disease – Wikipedia
https://en.wikipedia.org/wiki/Kawasaki_disease
Typically, initial treatment of Kawasaki disease consists of high doses of aspirin and immunoglobulin. […] Without treatment, coronary artery aneurysms occur in up to 25% and about 1% die. […] With treatment, the risk of death is reduced to 0.17%. […] The largest clinical study to date on treatment of resistant Kawasaki disease has shown that infliximab is more effective and safer than a second dose of IVIG in treating children with IVIG-resistant Kawasaki Disease, offering faster fever resolution and reduced hospitalization times without significant adverse events. […] Salicylate therapy, particularly aspirin, remains an important part of the treatment (though questioned by some) but salicylates alone are not as effective as IVIG. […] High-dose aspirin is associated with anemia and does not confer benefit to disease outcomes.
#90 Kawasaki Disease: Diagnosis & Treatment | NewYork-Presbyterian
https://www.nyp.org/pediatrics/rheumatology/kawasaki-disease/treatment
Kawasaki disease is treated in a hospital where doctors can monitor the child. Treatment should begin as soon as possible to speed up recovery and reduce the risk of complications. Intravenous (IV) immunoglobulin (a solution of antibodies), and aspirin are the main medicines used to treat Kawasaki disease. […] Gamma globulin – Infusion of an immune protein (gamma globulin) through a vein (intravenously or IV) used to reduce inflammation in blood vessels and lower the risk of coronary artery problems. […] Aspirin is usually not given to children because it has been linked to Reye’s syndrome, a rare but life-threatening condition in children. Because of this risk, aspirin is given to children with Kawasaki disease only under the supervision of a health care provider. […] After your child is discharged from the hospital, your healthcare provider will provide recommendations for your childs continued care at home. The child should continue taking any prescribed medicines. Full recovery could take around six to eight weeks after the disease started.
#91 Kawasaki Disease: Treatment, Procedure, Cost, Recovery, Side Effects And More
https://www.lybrate.com/topic/kawasaki-disease
Treatment of Kawasaki disease usually yields permanent results within 6-8 weeks of recovery. However, in some cases where the disease has not been diagnosed in its initial stage, it may lead to complications related to coronary artery damage and heart ailments. People showing symptoms of the disease must consult a doctor as early as possible because treating the disease within 10 days of its onset normally reduces the chances of complication. The patient is therefore advised frequent follow ups with the doctor after the treatment.
#92 Kawasaki Disease Symptoms, Cause, Criteria, Treatment & Complications
https://www.emedicinehealth.com/kawasaki_disease/article_em.htm
Kawasaki disease is the most common cause of acquired heart disease in children in the developed world. When diagnosed and treated early with gamma globulin, the incidence of coronary artery lesions decreases from 20% to 3%. It is very uncommon for patients who have no evidence of coronary abnormalities at 2-3 months after the acute illness to develop coronary abnormalities. Patients with larger coronary lesions have the greatest risk, and it has been shown that patients with giant aneurysms (8mm) have a highest risk of developing future heart attacks (myocardial infarctions). The long-term risk of patients with small aneurysms is currently unknown. Establishment and adherence to a heart healthy diet, daily physical exercise, control of blood pressure, and avoiding obesity and smoking are goals for all but especially those with potential risk factors for cardiovascular disease.
#93 Kawasaki Disease: Complications and Treatment | American Heart Association
https://www.heart.org/en/health-topics/kawasaki-disease/kawasaki-disease-complications-and-treatment
Once diagnosed, patients are treated in the hospital. The standard initial treatment is intravenous immunoglobulin (IVIG) and aspirin. When given early in the illness, IVIG can reduce the risk of coronary artery problems. Aspirin reduces fever, joint swelling, pain and the rash. It can also help prevent blood clots from forming. […] With proper treatment, most children with Kawasaki disease who donât develop aneurysms recover completely. They donât typically require long-term follow-up. But it may take one to two months for them to be back to normal. Rest is very important. […] Most important, people with KD and documented and persistent moderate or large aneurysms require lifelong cardiology follow-up with yearly or biyearly assessments.
#94 Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease | Italian Journal of Pediatrics | Full Text
https://ijponline.biomedcentral.com/articles/10.1186/s13052-021-00962-4
Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. […] The main goal of treatment in the acute phase of KD is suppressing systemic inflammation and minimizing the risk of developing CAA. Administration of IVIG is overall recognized as the first-line treatment for KD both in terms of efficacy and safety. […] IVIG at the dose of 2g/kg of body weight is the treatment of choice for KD, preferably given within the 10th day, better if within the 7th day of illness, but as soon as possible after diagnosis. […] Treatment of KD is completed by ASA given at a daily dosage of 30-50mg/kg in the acute phase of KD until 48h after the disappearance of fever, then switched to the anti-platelet dose (3-5mg/kg once daily).
#95 Kawasaki disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/diagnosis-treatment/drc-20354603
It’s best to start treatment for Kawasaki disease as early as possible, when your child still has a fever. Treatment for Kawasaki disease often happens in a hospital. The goals of treatment are to lower fever, reduce swelling and prevent heart damage. […] Treatment for Kawasaki disease can include: […] Gamma globulin. A protein called gamma globulin is given through a vein. This treatment lowers inflammation in the blood vessels. It can lower the risk of problems with the heart artery. […] Aspirin. High doses of aspirin might help treat inflammation. Aspirin also can decrease pain, joint swelling and fever. The aspirin dose will likely be lowered once the fever has been gone for 48 hours. […] Once the fever goes down, a child might need to take low-dose aspirin for at least six weeks. This can be longer if a there are problems with the heart artery. Aspirin helps prevent blood clotting.
#96 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html
Approximately 10% of patients have refractory disease that does not respond to initial therapy (i.e., fever persists or recurs 36 hours after initial IVIG dose). These patients usually receive a second infusion of IVIG at 2 g per kg. […] A retrospective study (n = 359) found that adding corticosteroids to IVIG for refractory Kawasaki disease decreased the number of patients whose condition did not respond to therapy and lowered the risk of coronary artery abnormalities at one month. […] TNF- antagonists have also been used for disease that does not respond to IVIG. […] Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Long-term low-dose aspirin; warfarin or low-molecular-weight heparin if giant aneurysm persists; consider use of -blockers to reduce myocardial O2 consumption.
#97 Kawasaki Disease Treatment & Management: Approach Considerations, Intravenous Immunoglobulin, Treatment of IVIG-Resistant Disease
https://emedicine.medscape.com/article/965367-treatment
Treatment of patients in which IVIG fails after the first and/or second dose remains controversial and is variable across institutions and providers. Guidelines from the AHA recommend a second dose of IVIG, methylprednisolone, a longer tapering course of prednisolone or prednisone plus IVIG, or infliximab be considered for patients resistant to IVIG. […] Infliximab is a chimeric mouse-human monoclonal antibody directed against soluble and membrane bound tumor necrosis factor-alpha. Several studies have found infliximab at a dose of 5 mg/kg to be useful in treating KD that is refractory to IVIG. […] Aspirin has long been a standard part of therapy for KD. However, its use has been called into question, as it does not impact the development of CAAs. […] Medium- (30-50 mg/kg/day) to high- (80-100 mg/kg/day) dose aspirin divided four times daily is typically given in the acute phase for its anti-inflammatory effects. It is continued until day 14 of the illness or until the patient has been afebrile for 48-72 hours.
#98
https://link.springer.com/article/10.1007/s40674-019-00115-z
The primary aim of therapy of Kawasaki disease (KD) is to halt inflammation and prevent development of coronary artery aneurysms (CAAs). […] High-dose aspirin has not been found to have significant effect on development of CAAs in KD. Role of corticosteroids has been suggested in patients with high-risk disease and a recent Cochrane review has recommended addition of oral corticosteroids to all children with KD. […] Intravenous immunoglobulin (2 g/kg) is the standard of care in all patients with KD. However, a proportion of patients may need additional therapy with corticosteroids or infliximab. Infliximab is also the drug of choice for IVIg-resistant KD. […] Infliximab plus infliximab may be considered for patients with KD and CAAs. Recent literature has also suggested the role of infliximab, ant-IL-1 antagonist (anakinra), and cyclosporine in IVIg-resistant KD. […] Patients with giant CAAs or multiple complex medium-sized aneurysms in a single coronary artery need anticoagulation and aspirin.
#99 Diagnosis and Management of Kawasaki Disease | AAFP
https://www.aafp.org/pubs/afp/issues/2015/0315/p365.html/1000
Patients with mild to moderate aneurysms are treated with aspirin alone or in combination with other anti-platelet agents, such as clopidogrel (Plavix) or dipyridamole (Persantine). […] Heparin and warfarin (Coumadin) are reserved for treating larger aneurysms, and coronary thrombosis is treated with thrombolytic agents in conjunction with aspirin and heparin. […] The 2004 AHA guideline provides recommendations for the long-term management and surveillance of cardiovascular risk in individuals with Kawasaki disease; these are echoed in the 2010 Japanese Circulation Society Joint Working Group guidelines.
#100 Kawasaki Disease: Symptoms, Causes & Treatment
https://my.clevelandclinic.org/health/diseases/13457-kawasaki-disease
With prompt treatment, most children recover in about two months. […] Treatment for Kawasaki disease includes: Immune globulin (IVIG), or human blood proteins you receive by IV. About 10% of children may not respond to the first dose of IVIG and will need a second dose or other medications. […] The goals of Kawasaki disease treatment are to: Reduce inflammation. Prevent or lessen damage to arteries. Prevent blood clots in people with coronary artery issues. Prevent heart complications. […] Yes. But with prompt treatment, most children with Kawasaki disease can recover completely.
#101 Kawasaki disease – Symptoms and causes – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598
Kawasaki disease is often treatable. With early treatment, most children get better and have no long-lasting problems. […] Treating Kawasaki disease within 10 days of when it began may reduce the chances of lasting damage to the arteries that supply the heart. […] But, with treatment, few children have lasting damage.