Zespół churga-straussa
Charakterystyka, pielęgnacja i opieka

Zespół Churga-Straussa, obecnie określany jako eozynofilowe ziarniniakowate zapalenie naczyń z poliangiitis (EGPA), to rzadkie autoimmunologiczne zapalenie małych i średnich naczyń, charakteryzujące się eozynofilią obwodową, astmą oraz zapaleniem błony śluzowej nosa i zatok. Choroba przebiega w trzech fazach: zapalenie zatok i alergie, ostra astma oraz zapalenie naczyń narządowych (płuca, przewód pokarmowy, nerwy obwodowe). Bez leczenia pięcioletnia przeżywalność wynosi około 25%, natomiast przy odpowiedniej terapii wzrasta do 90%. Diagnostyka i leczenie opierają się na ocenie ciężkości choroby, m.in. za pomocą Five Factor Score (FFS), gdzie FFS=0 wskazuje na łagodny przebieg, a FFS≥1 na cięższy, zagrażający życiu. Podstawą terapii są kortykosteroidy (prednizon 1 mg/kg/dobę, max 60 mg przez 2-3 tygodnie w łagodnych przypadkach) oraz leki immunosupresyjne, takie jak cyklofosfamid (w ciężkich postaciach) i leczenie podtrzymujące (rituksymab, azatiopryna 2-3 mg/kg/dobę, metotreksat 0,3 mg/kg/tydzień przez 18-24 miesiące). W przypadkach opornych stosuje się plazmaferezę, mepolizumab (anty-IL-5) oraz omalizumab.

Ogólne informacje o Zespole Churga-Straussa

Zespół Churga-Straussa, obecnie nazywany eozynofilowym ziarniniakowatym zapaleniem naczyń z poliangiitis (EGPA), jest rzadkim schorzeniem autoimmunologicznym charakteryzującym się zapaleniem małych i średnich naczyń krwionośnych12. Zapalenie to może ograniczać przepływ krwi do ważnych narządów i tkanek, powodując ich trwałe uszkodzenie3. Choroba często przebiega w trzech fazach: pierwsza obejmuje zapalenie zatok i pojawienie się lub nasilenie alergii, druga charakteryzuje się wystąpieniem ostrej astmy, a trzecia dotyczy zapalenia naczyń różnych układów narządowych, w tym płuc, przewodu pokarmowego i nerwów obwodowych4.

EGPA należy do grupy zapaleń naczyń związanych z przeciwciałami przeciwko cytoplazmie neutrofilów (ANCA), jednak wyróżnia się spośród innych schorzeń z tej grupy przez związek z astmą, zapaleniem błony śluzowej nosa i zatok oraz eozynofilią obwodową5. Bez leczenia wskaźnik pięcioletniego przeżycia wynosi około 25%, natomiast przy odpowiednim i terminowym leczeniu wskaźnik ten wzrasta do 90%6.

Podstawowe zasady leczenia i opieki

Chociaż nie ma leku na zespół Churga-Straussa, odpowiednie leczenie może skutecznie kontrolować objawy7. Celem terapii jest zwiększenie remisji, zmniejszenie częstości nawrotów oraz minimalizacja dawki kortykosteroidów doustnych8. Leczenie powinno być dostosowane do ciężkości choroby, biorąc pod uwagę obecność zagrożeń dla narządów lub życia (np. krwawienie pęcherzykowe, kłębuszkowe zapalenie nerek, niedokrwienie kończyn)9.

Podstawą leczenia jest kombinacja steroidów i leków immunosupresyjnych10. W przypadku pacjentów z łagodnymi objawami (Five Factor Score = 0), sam kortykosteroid może być wystarczający, jednak osoby z cięższymi objawami mogą wymagać dodania innego leku do tłumienia układu odpornościowego1112.

Kortykosteroidy jako podstawa leczenia

Prednizon, który zmniejsza stan zapalny, jest najczęściej przepisywanym lekiem w zespole Churga-Straussa13. Leczenie EGPA zazwyczaj rozpoczyna się od wysokich dawek kortykosteroidów w celu zmniejszenia stanu zapalnego i liczby eozynofilów14. U pacjentów z niecięzkiej postaci EGPA (FFS = 0), zaleca się rozpoczęcie od prednizonu w dawce 1 mg/kg (maksymalna dawka 60 mg) przez 2-3 tygodnie15.

Pacjenci z FFS ≥1 lub z zagrażającymi życiu objawami powinni być leczeni dożylnym metyloprednizolonem 1 g/dzień przez 3 dni wraz z indukcyjnymi lekami immunosupresyjnymi16. Gdy liczba eozynofilów zostanie wystarczająco zmniejszona, aby złagodzić objawy, choroba znajduje się w remisji, a lekarz może zacząć zmniejszać dawkę kortykosteroidów17.

Leki immunosupresyjne w terapii EGPA

W przypadkach ciężkich lub zagrażających życiu podawany jest cyklofosfamid18. Niektórzy pacjenci są leczeni, albo początkowo, albo w późniejszej fazie terapii, kombinacją codziennego doustnego prednizonu i cyklofosfamidu19. Aby zmniejszyć toksyczność cyklofosfamidu, należy rozważyć ochronę pęcherza moczowego poprzez nawodnienie i ewentualnie profilaktykę mesną20.

Po zakończeniu indukcji leczenia, zaleca się leczenie podtrzymujące rituximabem, azatiopryną 2-3 mg/kg/dzień lub metotreksatem 0,3 mg/kg/tydzień przez następne 18-24 miesiące21. Inne leki immunosupresyjne stosowane w EGPA to m.in. dapson, takrolimus i metotreksat2223.

Terapie biologiczne i alternatywne metody leczenia

W przypadkach opornych na standardowe leczenie stosuje się plazmaferezę, mepolizumab (przeciwciało monoklonalne anty-IL-5) i omalizumab (rekombinowane humanizowane przeciwciało monoklonalne anty-IVIG E)24. NUCALA (mepolizumab) jest warunkowo zalecany jako dodatkowa opcja leczenia dla odpowiednich pacjentów z EGPA w wytycznych ACR/VF z 2021 roku dotyczących postępowania w zapaleniach naczyń związanych z ANCA25.

Dożylna immunoglobulina (IVIG) może być stosowana jako leczenie drugiego rzutu u pacjentów z ciężkim zespołem Churga-Straussa26. IVIG pomaga zmniejszyć poziom eozynofilów we krwi, blokując ich aktywność, co zapobiega nadmiernemu gromadzeniu się eozynofilów w tkankach27.

Niektórzy pacjenci wykazali znaczną poprawę kliniczną, której towarzyszył spadek miana krążących pANCA, po leczeniu dożylną immunoglobuliną28. IVIG jest podawany drogą dożylną w warunkach szpitalnych lub w domu, a proces ten trwa kilka godzin i może wymagać wielu sesji, w zależności od odpowiedzi pacjenta z EGPA i nasilenia objawów29.

Opieka pielęgniarska w Zespole Churga-Straussa

Pielęgniarki odgrywają kluczową rolę w opiece i edukacji pacjentów z EGPA ze względu na rzadkość choroby, konieczność długotrwałego przyjmowania leków i zagrażające życiu powikłania30. Do klinicznych obowiązków pielęgniarki w leczeniu pacjenta z EGPA należą: monitorowanie poziomu eozynofilów, kontrola parametrów życiowych w przypadku gorączki lub astmy, codzienne podawanie leków pomagających w kontrolowaniu choroby oraz nawiązanie relacji poprzez komunikację, aby pomóc złagodzić strach u pacjenta i jego rodziny31.

Monitorowanie stanu pacjenta

Pacjenci z EGPA muszą być ściśle obserwowani w poradni reumatologicznej lub innej poradni specjalistycznej32. Obserwacja polega na monitorowaniu objawów aktywnej choroby i wykonywaniu odpowiednich badań obrazowych lub czynnościowych (np. testy czynności płuc, elektromiografia/elektroneurografia, echokardiografia), wraz z rutynowymi badaniami laboratoryjnymi33.

Pielęgniarki powinny być świadome skutków ubocznych długotrwałego stosowania glikokortykoidów i oceniać powikłania u pacjentów z EGPA34. Pacjenci hospitalizowani w celu leczenia EGPA powinni być oceniani pod kątem ryzyka zakrzepicy żył głębokich, zatorowości płucnej, a jeśli stwierdzono dysfunkcję serca, również zatoru kardiogennego35.

Edukacja pacjenta i profilaktyka powikłań

Lepsza wiedza na temat choroby i jej leczenia pomoże pielęgniarkom edukować pacjentów w zakresie choroby i zapobiegania dalszym powikłaniom poprzez przyjęcie zdrowego stylu życia, takiego jak regularne ćwiczenia, suplementacja witaminy D, kontrola diety, zaprzestanie palenia i regularne wizyty kontrolne36.

Długotrwałe leczenie kortykosteroidami może powodować wiele skutków ubocznych, takich jak utrata kości, wysoki poziom cukru we krwi, przyrost masy ciała, zaćma i trudne do leczenia infekcje37. Aby zminimalizować te problemy, zaleca się następujące kroki3839:

  • Zapytanie lekarza o ilość witaminy D i wapnia potrzebną w diecie oraz omówienie, czy należy przyjmować suplementy
  • Ćwiczenia pomagające utrzymać zdrową wagę, co jest ważne podczas przyjmowania leków kortykosteroidowych, które mogą powodować przyrost masy ciała
  • Spożywanie produktów, które pomagają utrzymać stabilny poziom cukru we krwi, takich jak owoce, warzywa i pełnoziarniste produkty

40

Podawanie leków przeciwwydzielniczych lub blokerów histaminy w celu zmniejszenia ryzyka krwawienia z przewodu pokarmowego powinno być kontynuowane tak długo, jak pacjenci przyjmują doustne kortykosteroidy41. Długotrwałe podawanie kortykosteroidów wiąże się z ryzykiem zaburzeń elektrolitowych, infekcji i złamań z powodu zmniejszonej masy kostnej, dlatego ryzyko to należy stale oceniać w trakcie terapii42.

Wsparcie psychologiczne i współpraca interprofesjonalna

Wiedza o chorobie może pomóc przygotować się do radzenia sobie z powikłaniami lub nawrotami43. Rodzina i przyjaciele mogą bardzo pomóc, a pacjenci mogą również rozmawiać z doradcą lub pracownikiem socjalnym zaznajomionym z chorobą lub innymi osobami z zespołem Churga-Straussa44.

W celu optymalizacji leczenia EGPA konieczna jest współpraca z interprofesjonalnym zespołem opieki zdrowotnej, zapewniającym kompleksowe podejście do opieki nad pacjentem45. Ścisła współpraca z reumatologami w leczeniu tak złożonej choroby, w tym szybka diagnostyka poprzez wykonanie biopsji nerwu przez chirurga ortopedycznego i szybkie leczenie medyczne przez reumatologa, jest kluczem do skutecznego postępowania46.

Powikłania i rokowanie

Zespół Churga-Straussa jest poważną chorobą, która może być śmiertelna. Nieleczona jest niezwykle niebezpieczna i zagraża zajętym narządom47. Powikłania zapalenia naczyń zależą od zajęcia konkretnego układu narządów48. Powikłania sercowe i neurologiczne są szczególnie poważne i bardziej prawdopodobne u pacjentów z opóźnioną diagnozą49.

Nieleczony zespół Churga-Straussa może powodować potencjalnie śmiertelne powikłania, takie jak tętniaki, choroby serca i udar50. Wczesna diagnostyka i rozpoczęcie leczenia mogą pomóc zmniejszyć stan zapalny i zapobiec postępowi choroby do poważniejszych objawów51.

Chociaż całkowite wskaźniki śmiertelności pacjentów z zespołem Churga-Straussa są dobre, obserwuje się nieakceptowalnie wysoki wskaźnik zachorowalności i nawrotów52. Wyzwania związane z leczeniem zespołu Churga-Straussa koncentrują się wokół zapobiegania zachorowalności i poprawy jakości życia, czemu może sprzyjać przedłużenie stosowania kortykosteroidów w małych dawkach i leków immunosupresyjnych53.

Przy agresywnym leczeniu i monitorowaniu chorobę można dość dobrze kontrolować. Możliwa jest również całkowita inaktywacja choroby (remisja)54. Ogólna przeżywalność pacjentów z CSS jest doskonała, ale nawroty nie są rzadkie i wymagają leczenia podtrzymującego lub oszczędzającego steroidy, w zależności od pierwotnej prognozy opartej na FFS w momencie rozpoznania55.

Podsumowanie opieki nad pacjentem z Zespołem Churga-Straussa

Zespół Churga-Straussa, obecnie nazywany eozynofilowym ziarniniakowatym zapaleniem naczyń z poliangiitis (EGPA), wymaga kompleksowego podejścia do leczenia i opieki nad pacjentem. Podstawą leczenia są kortykosteroidy, często w połączeniu z lekami immunosupresyjnymi w przypadku cięższego przebiegu choroby. Wczesna diagnoza i leczenie znacznie poprawiają rokowanie56.

Pielęgniarki odgrywają kluczową rolę w monitorowaniu stanu pacjenta, edukacji na temat choroby i zapobieganiu powikłaniom związanym z leczeniem. Regularne wizyty kontrolne, ćwiczenia i kontynuacja przyjmowania leków mogą zminimalizować powikłania i poprawić jakość życia pacjentów57.

Współpraca między różnymi specjalistami, w tym reumatologami, pulmonologami, nefrologami i ortopedami, jest niezbędna dla skutecznego leczenia tej złożonej choroby5859. Z odpowiednim leczeniem i monitorowaniem większość pacjentów może osiągnąć remisję i prowadzić aktywne życie60.

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Eosinophilic granulomatosis with polyangiitis formerly known as ChurgStrauss syndrome (CSS) is a multisystemic rare autoimmune disorder which results in restriction of blood flow to the vital organs, particularly the respiratory tract. […] Better knowledge about the disease condition and its treatment will assist nurses to educate the patients regarding the illness and prevention of further complication by adapting a healthy lifestyle such as regular exercise, Vitamin D supplement, diet control, smoking cessation and regular follow-up. […] Nurses play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication. To promote their wellness, increase in patient safety measures and teaching is needed. The clinical responsibilities of a nurse in treating a patient with EGPA are to monitor their eosinophil levels, monitor their vital signs in case of fever or asthma, administer medications every day to help with management of their disease and establish the relationship by communication to help alleviate fear in patient and family.
  • #2 Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment – PubMed
    https://pubmed.ncbi.nlm.nih.gov/21674415/
    Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. […] Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. […] All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses.
  • #3 Churg-Strauss syndrome | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/churg-strauss-syndrome
    Churg-Strauss syndrome is a disorder marked by blood vessel inflammation. This inflammation can restrict blood flow to organs and tissues, sometimes permanently damaging them. This condition is also known as eosinophilic granulomatosis with polyangiitis (EGPA). […] Churg-Strauss syndrome is rare and has no cure. Symptoms can usually be controlled with steroids and other powerful immunosuppressant drugs. […] There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly.
  • #4 Churg-Strauss Syndrome Mnemonic
    https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/vasculitides-39285/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome_6055
    Churg-Strauss syndrome typically presents in three stages. The first stage of disease involves the sinuses and the onset or worsening of pre-existing allergies. The second stage involves the onset of acute asthma. The third stage involves various organ systems including vessels of the lungs, GI tract, and peripheral nerves. […] Treatment of this disease begins with corticosteroid administration. […] Treatment for this syndrome consists of high-dose corticosteroid administration. Other modalities of treatment include immunosuppressive drugs (such as azathioprine and cyclophosphamide), along with monoclonal antibodies (mepolizumab).
  • #5 CE Activity | Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | NPs
    https://www.statpearls.com/nursepractitioner/ce/activity/94228
    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). EGPA is distinguished from other conditions in this group by its association with asthma, rhinosinusitis, and peripheral eosinophilia. […] The management of EGPA also differs from other AAVs, requiring tailored treatment strategies. This activity reviews the presentation, evaluation, and management of EGPA, highlighting the crucial role of an interprofessional healthcare team in optimizing patient care, particularly given the variability in symptoms and treatment responses. […] Collaborate with an interprofessional healthcare team to optimize the management of eosinophilic granulomatosis with polyangiitis, ensuring a comprehensive approach to patient care. […] Outline the pharmacologic therapy as it applies to Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome).
  • #6 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    Plasmapheresis, mepolizumab (an anti-IL-5 monoclonal antibody) and omalizumab (recombinant humanised monoclonal anti-IVIG E antibody) have been found to be useful in refractory cases. […] Complications of vasculitis depend on the specific organ system involvement. […] Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. […] However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. […] Relapses are not uncommon.
  • #7 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] High doses of corticosteroids can cause serious side effects, so your doctor will decrease the dose gradually until you’re taking the smallest amount that will keep your disease under control. Even lower doses taken for extended periods can cause side effects. […] Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts and hard-to-treat infections.
  • #8 About EGPA | NUCALA (mepolizumab) for HCPs
    https://nucalahcp.com/egpa/egpa-patient-types/about/
    The current standard of care (SOC) is oral corticosteroids (OCS) with or without an immunosuppressant. According to EGPA guidelines, the goal of therapy in EGPA is to10,11: […] increase remission […] reduce relapse rates […] minimize OCS dose. […] NUCALA is conditionally recommended as an add-on treatment option for appropriate patients with EGPA in the 2021 ACR/VF Guideline for the Management of ANCA-Associated Vasculitis. […] NUCALA is indicated for the: […] treatment of adult patients with eosinophilic granulomatosis with polyangiitis (EGPA). […] Hypersensitivity reactions (eg, anaphylaxis, angioedema, bronchospasm, hypotension, urticaria, rash) have occurred with NUCALA. […] Do not discontinue systemic or inhaled corticosteroids abruptly upon initiation of therapy with NUCALA. […] Treat patients with pre-existing helminth infections before initiating therapy with NUCALA.
  • #9 Eosinophilic granulomatosis with polyangiitis – Knowledge @ AMBOSS
    https://www.amboss.com/us/knowledge/eosinophilic-granulomatosis-with-polyangiitis/
    Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules […] Management of EGPA is based on disease severity, taking into account the presence of organ- and/or life-threatening manifestations (e.g., alveolar hemorrhage, glomerulonephritis, limb ischemia). […] Consult rheumatology and other specialties (e.g., nephrology, pulmonology) as required. […] Goal of pharmacotherapy (e.g., glucocorticoids PLUS cyclophosphamide) is induction of remission […] Prevent complications of glucocorticoid therapy. […] Monitor for adverse effects of immunosuppressants. […] Consider pneumocystis pneumonia prophylaxis.
  • #10 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    The mainstay of treatment of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is combination of steroids and immunosuppressants agents. Medical management of cardiovascular, cardiac, renal, and gastrointestinal complications of EGPA, falls under the purview of subspecialty consultation. […] Patients hospitalized for treatment of EGPA, should be assessed, as should any hospitalized patient, for the risk of deep vein thrombosis, pulmonary embolus and, if cardiac dysfunction has been noted, cardiogenic embolus. […] The recommended initial medications for treatment of severe manifestations of eosinophilic granulomatosis with polyangiitis (EGPA), including patients with EGPA-related peripheral neuritis, are corticosteroids, which are administered at high doses.
  • #11 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: […] Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. […] Exercise can help you maintain a healthy weight, which is important when you’re taking corticosteroid medications that can cause weight gain. […] Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains.
  • #12 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Systemic corticosteroid therapy is the cornerstone of management of eosinophilic granulomatosis with polyangiitis (EGPA). Selection of the agent and regimen is guided by the patient’s five factor score (FFS); see Workup/Five Factor Score. […] Patients with EGPA also require management of asthma. Treatment is the same as for asthma in the general population, with a stepwise approach tailored to asthma severity. […] In patients with non-severe EGPA (FFS = 0), the French Vasculitis Study Group recommends starting prednisone at a dosage of 1 mg/kg (maximum dose, 60 mg) for 2-3 weeks. […] Patients with an FFS 1 or any life-threatening manifestations should be treated with intravenous methylprednisolone 1 g/day for 3 days along with induction immunosuppressive agents. […] To decrease cyclophosphamide toxicity, bladder protection with hydration and possibly mesna prophylaxis must be considered.
  • #13 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] High doses of corticosteroids can cause serious side effects, so your doctor will decrease the dose gradually until you’re taking the smallest amount that will keep your disease under control. Even lower doses taken for extended periods can cause side effects. […] Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts and hard-to-treat infections.
  • #14 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa
    EGPA (formerly Churg-Strauss syndrome) begins with respiratory symptoms, but it can go on to affect you all over. […] Treatment for EGPA begins with a high dose of corticosteroids to reduce inflammation and eosinophils. When they’ve reduced enough to relieve your symptoms, your disease is in remission. At this point, your provider will begin reducing your dose. Most people continue to take a low dose of corticosteroids to maintain remission. […] Medications for EGPA reduce your immunity, which makes it easier for you to get sick and harder for you to get better. When you’re taking these medications, you’ll need to take extra care to protect yourself from common illnesses, because you won’t be able to bounce back as easily as before. […] Long-term treatment with corticosteroids can also cause side effects, including high blood sugar and weight gain, bone thinning and mood changes. Your provider will work with you to manage side effects while managing EGPA. Stay in touch with your provider about all your symptoms, especially new ones.
  • #15 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Systemic corticosteroid therapy is the cornerstone of management of eosinophilic granulomatosis with polyangiitis (EGPA). Selection of the agent and regimen is guided by the patient’s five factor score (FFS); see Workup/Five Factor Score. […] Patients with EGPA also require management of asthma. Treatment is the same as for asthma in the general population, with a stepwise approach tailored to asthma severity. […] In patients with non-severe EGPA (FFS = 0), the French Vasculitis Study Group recommends starting prednisone at a dosage of 1 mg/kg (maximum dose, 60 mg) for 2-3 weeks. […] Patients with an FFS 1 or any life-threatening manifestations should be treated with intravenous methylprednisolone 1 g/day for 3 days along with induction immunosuppressive agents. […] To decrease cyclophosphamide toxicity, bladder protection with hydration and possibly mesna prophylaxis must be considered.
  • #16 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Systemic corticosteroid therapy is the cornerstone of management of eosinophilic granulomatosis with polyangiitis (EGPA). Selection of the agent and regimen is guided by the patient’s five factor score (FFS); see Workup/Five Factor Score. […] Patients with EGPA also require management of asthma. Treatment is the same as for asthma in the general population, with a stepwise approach tailored to asthma severity. […] In patients with non-severe EGPA (FFS = 0), the French Vasculitis Study Group recommends starting prednisone at a dosage of 1 mg/kg (maximum dose, 60 mg) for 2-3 weeks. […] Patients with an FFS 1 or any life-threatening manifestations should be treated with intravenous methylprednisolone 1 g/day for 3 days along with induction immunosuppressive agents. […] To decrease cyclophosphamide toxicity, bladder protection with hydration and possibly mesna prophylaxis must be considered.
  • #17 EGPA (Formerly Churg-Strauss Syndrome): Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/churg-strauss-syndrome-eosinophilic-granulomatosis-with-polyangiitis-egpa
    EGPA (formerly Churg-Strauss syndrome) begins with respiratory symptoms, but it can go on to affect you all over. […] Treatment for EGPA begins with a high dose of corticosteroids to reduce inflammation and eosinophils. When they’ve reduced enough to relieve your symptoms, your disease is in remission. At this point, your provider will begin reducing your dose. Most people continue to take a low dose of corticosteroids to maintain remission. […] Medications for EGPA reduce your immunity, which makes it easier for you to get sick and harder for you to get better. When you’re taking these medications, you’ll need to take extra care to protect yourself from common illnesses, because you won’t be able to bounce back as easily as before. […] Long-term treatment with corticosteroids can also cause side effects, including high blood sugar and weight gain, bone thinning and mood changes. Your provider will work with you to manage side effects while managing EGPA. Stay in touch with your provider about all your symptoms, especially new ones.
  • #18 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    EGPA (eosinophilic granulomatosis with polyangiitis), formerly known as Churg-Strauss syndrome, is a rare diffuse vasculitic disease affecting coronary, pulmonary, cerebral, abdominal visceral and skin circulations. The vasculitis affects small- and medium-sized arteries and veins and is associated with asthma. […] High-dose steroids are usually adequate for treatment. […] Cyclophosphamide is administered in patients with severe or life-threatening complications. Either azathioprine or methotrexate is also used. […] Other treatments include intravenous immune globulin, interferon-alpha and plasma exchange. […] Successful use of rituximab has been reported. […] Oral tacrolimus in combination with methylprednisolone and cyclophosphamide was used successfully in the treatment of a child severely ill with EGPA.
  • #19 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    Some patients have been treated, either initially or during a subsequent phase of therapy, with the combination of daily oral prednisone and cyclophosphamide. […] The combination of high-dose corticosteroids and dapsone has been used in patients with severe Churg-Strauss disease (CSD) and has proven effective in instances of Churg-Strauss myocarditis. […] Consultations for patients with of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), depend on the manifestations of the disease. […] A number of authorities believe that the administration of cysteinyl leukotriene-receptor antagonists for the treatment of asthma may provoke development of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), in some individuals.
  • #20 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Systemic corticosteroid therapy is the cornerstone of management of eosinophilic granulomatosis with polyangiitis (EGPA). Selection of the agent and regimen is guided by the patient’s five factor score (FFS); see Workup/Five Factor Score. […] Patients with EGPA also require management of asthma. Treatment is the same as for asthma in the general population, with a stepwise approach tailored to asthma severity. […] In patients with non-severe EGPA (FFS = 0), the French Vasculitis Study Group recommends starting prednisone at a dosage of 1 mg/kg (maximum dose, 60 mg) for 2-3 weeks. […] Patients with an FFS 1 or any life-threatening manifestations should be treated with intravenous methylprednisolone 1 g/day for 3 days along with induction immunosuppressive agents. […] To decrease cyclophosphamide toxicity, bladder protection with hydration and possibly mesna prophylaxis must be considered.
  • #21 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Once an induction regimen has been completed, maintenance treatment with rituximab, azathioprine 2-3 mg/kg/day, or methotrexate 0.3 mg/kg/week is recommended for the next 18-24 months. […] Patients with EGPA must be followed up very closely at a rheumatology clinic or another specialty clinic. These patients usually need long-term immunosuppressive medications. […] Follow-up consists of monitoring for signs and symptoms of active disease and performing appropriate imaging or functional studies (eg, pulmonary function tests, electromyographyelectroneurography, echocardiography), along with routine laboratory tests.
  • #22 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    EGPA (eosinophilic granulomatosis with polyangiitis), formerly known as Churg-Strauss syndrome, is a rare diffuse vasculitic disease affecting coronary, pulmonary, cerebral, abdominal visceral and skin circulations. The vasculitis affects small- and medium-sized arteries and veins and is associated with asthma. […] High-dose steroids are usually adequate for treatment. […] Cyclophosphamide is administered in patients with severe or life-threatening complications. Either azathioprine or methotrexate is also used. […] Other treatments include intravenous immune globulin, interferon-alpha and plasma exchange. […] Successful use of rituximab has been reported. […] Oral tacrolimus in combination with methylprednisolone and cyclophosphamide was used successfully in the treatment of a child severely ill with EGPA.
  • #23 Churg-Strauss Syndrome Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/churg-strauss-sydrome-4174962
    Churg-Strauss syndrome can be fatal if left untreated. Complications from chronic vasculitis can increase a person’s risk of developing serious conditions such as aneurysms, heart disease, and stroke. Diagnosing the condition early and beginning treatment can help reduce inflammation and prevent the progression to more serious symptoms. […] Treatment for Churg-Strauss depends on the phase the condition is in at the time of diagnosis, which organ systems are affected, and the individual symptoms. Most people will begin treatment with medications that suppress their immune system, called corticosteroids. One of the most commonly prescribed corticosteroid or steroid medications is prednisone. […] People with advanced disease may also need to take cytotoxic medications such as cyclophosphamide or methotrexate.
  • #24 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    Plasmapheresis, mepolizumab (an anti-IL-5 monoclonal antibody) and omalizumab (recombinant humanised monoclonal anti-IVIG E antibody) have been found to be useful in refractory cases. […] Complications of vasculitis depend on the specific organ system involvement. […] Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. […] However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. […] Relapses are not uncommon.
  • #25 About EGPA | NUCALA (mepolizumab) for HCPs
    https://nucalahcp.com/egpa/egpa-patient-types/about/
    The current standard of care (SOC) is oral corticosteroids (OCS) with or without an immunosuppressant. According to EGPA guidelines, the goal of therapy in EGPA is to10,11: […] increase remission […] reduce relapse rates […] minimize OCS dose. […] NUCALA is conditionally recommended as an add-on treatment option for appropriate patients with EGPA in the 2021 ACR/VF Guideline for the Management of ANCA-Associated Vasculitis. […] NUCALA is indicated for the: […] treatment of adult patients with eosinophilic granulomatosis with polyangiitis (EGPA). […] Hypersensitivity reactions (eg, anaphylaxis, angioedema, bronchospasm, hypotension, urticaria, rash) have occurred with NUCALA. […] Do not discontinue systemic or inhaled corticosteroids abruptly upon initiation of therapy with NUCALA. […] Treat patients with pre-existing helminth infections before initiating therapy with NUCALA.
  • #26 IVIG for Churg-Strauss Syndrome: How This Therapy Helps When Standard Treatments Fall Short – AmeriPharma® Specialty Care
    https://ameripharmaspecialty.com/ivig/ivig-for-churg-strauss-syndrome-how-this-therapy-helps-when-standard-treatments-fall-short/
    Various case reports have shown that IVIG therapy can be used as a second-line treatment in patients with severe Churg-Strauss syndrome. […] Standard treatments like corticosteroids or surgery are used to treat Churg-Strauss syndrome. However, in some cases, IVIG therapy is recommended as a second-line treatment when the conventional therapies are not sufficient. […] Various case reports have shown that IVIG therapy can effectively treat patients with CSS when other standard treatments do not yield promising results. […] IVIG therapy is known to have anti-inflammatory properties. It can help reduce the inflammation in the blood vessels and eventually reduce the risk of severe organ damage. […] IVIG also helps to neutralize or block the activity of autoantibodies. […] In CSS patients, IVIG helps reduce the eosinophil level in the blood. It does this by blocking the activity of eosinophils. This prevents the excessive buildup of eosinophils in the tissues.
  • #27 IVIG for Churg-Strauss Syndrome: How This Therapy Helps When Standard Treatments Fall Short – AmeriPharma® Specialty Care
    https://ameripharmaspecialty.com/ivig/ivig-for-churg-strauss-syndrome-how-this-therapy-helps-when-standard-treatments-fall-short/
    Various case reports have shown that IVIG therapy can be used as a second-line treatment in patients with severe Churg-Strauss syndrome. […] Standard treatments like corticosteroids or surgery are used to treat Churg-Strauss syndrome. However, in some cases, IVIG therapy is recommended as a second-line treatment when the conventional therapies are not sufficient. […] Various case reports have shown that IVIG therapy can effectively treat patients with CSS when other standard treatments do not yield promising results. […] IVIG therapy is known to have anti-inflammatory properties. It can help reduce the inflammation in the blood vessels and eventually reduce the risk of severe organ damage. […] IVIG also helps to neutralize or block the activity of autoantibodies. […] In CSS patients, IVIG helps reduce the eosinophil level in the blood. It does this by blocking the activity of eosinophils. This prevents the excessive buildup of eosinophils in the tissues.
  • #28 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    Administration of antacids or histamine-blocking agents to reduce the risk for gastrointestinal hemorrhage should be continued for as long as patients are administered oral corticosteroids. Long-term corticosteroid administration entails risk for electrolyte disturbances, infections, and fractures because of diminished bone mass. These risks must be reassessed continually during the course of therapy. […] Many patients with EGPA manifest a favorable response to this monotherapeutic approach within a few days; however, in many cases, persistence of asthma prevents oral prednisone from being tapered to doses lower than 10-15 mg/d. […] Corticosteroid treatment, whether oral or intravenous, has been combined with plasma exchange or plasmapheresis for cases that were difficult to treat. […] Some patients have demonstrated marked clinical improvement, accompanied by declining circulating pANCA titers, after treatment with intravenous immunoglobulin (IVIg).
  • #29 IVIG for Churg-Strauss Syndrome: How This Therapy Helps When Standard Treatments Fall Short – AmeriPharma® Specialty Care
    https://ameripharmaspecialty.com/ivig/ivig-for-churg-strauss-syndrome-how-this-therapy-helps-when-standard-treatments-fall-short/
    IVIG is administered through an intravenous (IV) infusion in a hospital setting or at home. This process takes several hours and may require multiple sessions, depending on the CSS patient’s response and the severity of the symptoms. […] Not all CSS patients can receive IVIG therapy. It is generally considered for patients who showed poor response to standard treatments of CSS, patients with compromised immune function, and patients with relapses or severe organ involvement. […] Currently, only a few studies have reported the beneficial effects of IVIG in CSS patients. Based on these studies’ results, IVIG is considered an effective second-line treatment for CSS when conventional therapies are insufficient. […] Hence, IVIG therapy can be considered a second-line treatment for CSS patients when standard treatments do not give promising results.
  • #30
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Eosinophilic granulomatosis with polyangiitis formerly known as ChurgStrauss syndrome (CSS) is a multisystemic rare autoimmune disorder which results in restriction of blood flow to the vital organs, particularly the respiratory tract. […] Better knowledge about the disease condition and its treatment will assist nurses to educate the patients regarding the illness and prevention of further complication by adapting a healthy lifestyle such as regular exercise, Vitamin D supplement, diet control, smoking cessation and regular follow-up. […] Nurses play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication. To promote their wellness, increase in patient safety measures and teaching is needed. The clinical responsibilities of a nurse in treating a patient with EGPA are to monitor their eosinophil levels, monitor their vital signs in case of fever or asthma, administer medications every day to help with management of their disease and establish the relationship by communication to help alleviate fear in patient and family.
  • #31
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Eosinophilic granulomatosis with polyangiitis formerly known as ChurgStrauss syndrome (CSS) is a multisystemic rare autoimmune disorder which results in restriction of blood flow to the vital organs, particularly the respiratory tract. […] Better knowledge about the disease condition and its treatment will assist nurses to educate the patients regarding the illness and prevention of further complication by adapting a healthy lifestyle such as regular exercise, Vitamin D supplement, diet control, smoking cessation and regular follow-up. […] Nurses play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication. To promote their wellness, increase in patient safety measures and teaching is needed. The clinical responsibilities of a nurse in treating a patient with EGPA are to monitor their eosinophil levels, monitor their vital signs in case of fever or asthma, administer medications every day to help with management of their disease and establish the relationship by communication to help alleviate fear in patient and family.
  • #32 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Once an induction regimen has been completed, maintenance treatment with rituximab, azathioprine 2-3 mg/kg/day, or methotrexate 0.3 mg/kg/week is recommended for the next 18-24 months. […] Patients with EGPA must be followed up very closely at a rheumatology clinic or another specialty clinic. These patients usually need long-term immunosuppressive medications. […] Follow-up consists of monitoring for signs and symptoms of active disease and performing appropriate imaging or functional studies (eg, pulmonary function tests, electromyographyelectroneurography, echocardiography), along with routine laboratory tests.
  • #33 Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Treatment & Management: Approach Considerations, Medical Care, Consultations
    https://emedicine.medscape.com/article/333492-treatment
    Once an induction regimen has been completed, maintenance treatment with rituximab, azathioprine 2-3 mg/kg/day, or methotrexate 0.3 mg/kg/week is recommended for the next 18-24 months. […] Patients with EGPA must be followed up very closely at a rheumatology clinic or another specialty clinic. These patients usually need long-term immunosuppressive medications. […] Follow-up consists of monitoring for signs and symptoms of active disease and performing appropriate imaging or functional studies (eg, pulmonary function tests, electromyographyelectroneurography, echocardiography), along with routine laboratory tests.
  • #34
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Nurses should be aware of the side effects of long-term glucocorticoid use and assess for these complications of patients with EGPA. […] Having regular follow-up, exercise and continuation of medications can minimise their complications and improve their span and quality of life. Nurses play a very important role in supporting and teaching the patients to adopt healthy lifestyle to remain free of pain and complications.
  • #35 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    The mainstay of treatment of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), is combination of steroids and immunosuppressants agents. Medical management of cardiovascular, cardiac, renal, and gastrointestinal complications of EGPA, falls under the purview of subspecialty consultation. […] Patients hospitalized for treatment of EGPA, should be assessed, as should any hospitalized patient, for the risk of deep vein thrombosis, pulmonary embolus and, if cardiac dysfunction has been noted, cardiogenic embolus. […] The recommended initial medications for treatment of severe manifestations of eosinophilic granulomatosis with polyangiitis (EGPA), including patients with EGPA-related peripheral neuritis, are corticosteroids, which are administered at high doses.
  • #36
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Eosinophilic granulomatosis with polyangiitis formerly known as ChurgStrauss syndrome (CSS) is a multisystemic rare autoimmune disorder which results in restriction of blood flow to the vital organs, particularly the respiratory tract. […] Better knowledge about the disease condition and its treatment will assist nurses to educate the patients regarding the illness and prevention of further complication by adapting a healthy lifestyle such as regular exercise, Vitamin D supplement, diet control, smoking cessation and regular follow-up. […] Nurses play a major role in caring and educating patients with EGPA because of its rarity, lifetime medication and life-threatening complication. To promote their wellness, increase in patient safety measures and teaching is needed. The clinical responsibilities of a nurse in treating a patient with EGPA are to monitor their eosinophil levels, monitor their vital signs in case of fever or asthma, administer medications every day to help with management of their disease and establish the relationship by communication to help alleviate fear in patient and family.
  • #37 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms. […] Prednisone, which reduces inflammation, is the most commonly prescribed drug for Churg-Strauss syndrome. Your doctor might prescribe a high dose of corticosteroids or a boost in your current dose of corticosteroids to get your symptoms under control quickly. […] High doses of corticosteroids can cause serious side effects, so your doctor will decrease the dose gradually until you’re taking the smallest amount that will keep your disease under control. Even lower doses taken for extended periods can cause side effects. […] Side effects of corticosteroids include bone loss, high blood sugar, weight gain, cataracts and hard-to-treat infections.
  • #38 Churg-Strauss syndrome | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/churg-strauss-syndrome
    For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: […] Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. […] Exercise can help you maintain a healthy weight, which is important when you’re taking corticosteroid medications that can cause weight gain. […] Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition.
  • #39 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: […] Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. […] Exercise can help you maintain a healthy weight, which is important when you’re taking corticosteroid medications that can cause weight gain. […] Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains.
  • #40 Churg-Strauss syndrome | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/churg-strauss-syndrome
    For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: […] Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. […] Exercise can help you maintain a healthy weight, which is important when you’re taking corticosteroid medications that can cause weight gain. […] Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition.
  • #41 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    Administration of antacids or histamine-blocking agents to reduce the risk for gastrointestinal hemorrhage should be continued for as long as patients are administered oral corticosteroids. Long-term corticosteroid administration entails risk for electrolyte disturbances, infections, and fractures because of diminished bone mass. These risks must be reassessed continually during the course of therapy. […] Many patients with EGPA manifest a favorable response to this monotherapeutic approach within a few days; however, in many cases, persistence of asthma prevents oral prednisone from being tapered to doses lower than 10-15 mg/d. […] Corticosteroid treatment, whether oral or intravenous, has been combined with plasma exchange or plasmapheresis for cases that were difficult to treat. […] Some patients have demonstrated marked clinical improvement, accompanied by declining circulating pANCA titers, after treatment with intravenous immunoglobulin (IVIg).
  • #42 Churg-Strauss Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/1178795-treatment
    Administration of antacids or histamine-blocking agents to reduce the risk for gastrointestinal hemorrhage should be continued for as long as patients are administered oral corticosteroids. Long-term corticosteroid administration entails risk for electrolyte disturbances, infections, and fractures because of diminished bone mass. These risks must be reassessed continually during the course of therapy. […] Many patients with EGPA manifest a favorable response to this monotherapeutic approach within a few days; however, in many cases, persistence of asthma prevents oral prednisone from being tapered to doses lower than 10-15 mg/d. […] Corticosteroid treatment, whether oral or intravenous, has been combined with plasma exchange or plasmapheresis for cases that were difficult to treat. […] Some patients have demonstrated marked clinical improvement, accompanied by declining circulating pANCA titers, after treatment with intravenous immunoglobulin (IVIg).
  • #43 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    Knowing about the disease can help prepare you to deal with complications or recurrences. […] Family and friends can help tremendously. You might also want to talk to a counselor or medical social worker who’s familiar with the disease. Or you may find it helpful to talk to other people with Churg-Strauss syndrome. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. You might also see a pulmonologist since Churg-Strauss affects your respiratory tract.
  • #44 Churg-Strauss syndrome – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/churg-strauss-syndrome/diagnosis-treatment/drc-20353765
    Knowing about the disease can help prepare you to deal with complications or recurrences. […] Family and friends can help tremendously. You might also want to talk to a counselor or medical social worker who’s familiar with the disease. Or you may find it helpful to talk to other people with Churg-Strauss syndrome. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition. […] You may be referred to a doctor who specializes in disorders that cause blood vessel inflammation (vasculitis), such as a rheumatologist or immunologist. You might also see a pulmonologist since Churg-Strauss affects your respiratory tract.
  • #45 CE Activity | Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome) | NPs
    https://www.statpearls.com/nursepractitioner/ce/activity/94228
    Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, belongs to a group of diseases characterized by necrotizing vasculitis affecting small- and medium-sized systemic blood vessels, also known as antineutrophil cytoplasmic autoantibody (ANCA)associated vasculitis (AAV). EGPA is distinguished from other conditions in this group by its association with asthma, rhinosinusitis, and peripheral eosinophilia. […] The management of EGPA also differs from other AAVs, requiring tailored treatment strategies. This activity reviews the presentation, evaluation, and management of EGPA, highlighting the crucial role of an interprofessional healthcare team in optimizing patient care, particularly given the variability in symptoms and treatment responses. […] Collaborate with an interprofessional healthcare team to optimize the management of eosinophilic granulomatosis with polyangiitis, ensuring a comprehensive approach to patient care. […] Outline the pharmacologic therapy as it applies to Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome).
  • #46 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ
    https://www.hkmj.org/abstracts/v21n6/565.htm
    With treatment, most of the symptoms in any of the three phases can be relieved. We would like to raise the awareness of such an entity in treating patients with neuropathy. Close collaboration with rheumatologists in treating such a complex illness, including prompt diagnosis by performing nerve biopsy by an orthopaedic surgeon and prompt medical treatment by a rheumatologist, is the key to successful management.
  • #47 Churg-Strauss syndrome (CSS; allergic granulomatous angiitis) | Healthengine Blog
    https://healthinfo.healthengine.com.au/churg-strauss-syndrome-css
    Churg-Strauss syndrome is a serious disease that can be fatal. Untreated it is extremely dangerous and threatens the organs that are affected. With aggressive treatment and monitoring it can be controlled fairly well. Total inactivation of the disease (remission) is possible as well. […] The treatment of patients with Churg-Strauss syndrome is directed toward both immediately reducing the inflammation of the blood vessels (vasculitis) and suppressing the immune system (because it is the bodys own immune system that is attacking the cells). Treatment usually includes high doses of corticosteroids medication (such as prednisone or prednisolone) to suppress the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan). […] If the patient is unresponsive to the above treatment schedule or if the disease is severe, other treatment may be used.
  • #48 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    Plasmapheresis, mepolizumab (an anti-IL-5 monoclonal antibody) and omalizumab (recombinant humanised monoclonal anti-IVIG E antibody) have been found to be useful in refractory cases. […] Complications of vasculitis depend on the specific organ system involvement. […] Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. […] However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. […] Relapses are not uncommon.
  • #49 Churg-Strauss syndrome (now called EGPA – Eosinophilic Granulomatosis with Polyangiitis)
    https://patient.info/doctor/churg-strauss-syndrome-pro
    Plasmapheresis, mepolizumab (an anti-IL-5 monoclonal antibody) and omalizumab (recombinant humanised monoclonal anti-IVIG E antibody) have been found to be useful in refractory cases. […] Complications of vasculitis depend on the specific organ system involvement. […] Cardiac and neurological complications are particularly serious and are more likely in patients with a delayed diagnosis. […] Without treatment, the five-year survival rate is about 25%. […] However, patient outcomes have dramatically improved in recent years. With appropriate and timely treatment, the survival rate at five years is now 90%. […] Relapses are not uncommon.
  • #50 Churg-Strauss Syndrome Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/churg-strauss-sydrome-4174962
    Churg-Strauss syndrome can be fatal if left untreated. Complications from chronic vasculitis can increase a person’s risk of developing serious conditions such as aneurysms, heart disease, and stroke. Diagnosing the condition early and beginning treatment can help reduce inflammation and prevent the progression to more serious symptoms. […] Treatment for Churg-Strauss depends on the phase the condition is in at the time of diagnosis, which organ systems are affected, and the individual symptoms. Most people will begin treatment with medications that suppress their immune system, called corticosteroids. One of the most commonly prescribed corticosteroid or steroid medications is prednisone. […] People with advanced disease may also need to take cytotoxic medications such as cyclophosphamide or methotrexate.
  • #51 Churg-Strauss Syndrome Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/churg-strauss-sydrome-4174962
    Churg-Strauss syndrome can be fatal if left untreated. Complications from chronic vasculitis can increase a person’s risk of developing serious conditions such as aneurysms, heart disease, and stroke. Diagnosing the condition early and beginning treatment can help reduce inflammation and prevent the progression to more serious symptoms. […] Treatment for Churg-Strauss depends on the phase the condition is in at the time of diagnosis, which organ systems are affected, and the individual symptoms. Most people will begin treatment with medications that suppress their immune system, called corticosteroids. One of the most commonly prescribed corticosteroid or steroid medications is prednisone. […] People with advanced disease may also need to take cytotoxic medications such as cyclophosphamide or methotrexate.
  • #52 Churg-Strauss Syndrome: ANCA Positivity and Long-Term Outcomes – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/news/churg-strauss-syndrome-anca-positivity-and-long-term-outcomes/
    Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, is a rare disorder that causes inflammation of the blood vessels, which can restrict blood flow to vital organs and tissue and cause permanent damage. Patients may have a variety of symptoms, such as asthma, gastrointestinal bleeding, pain, and rashes, which can significantly reduce their quality of life. […] The study findings suggest that patients with Churg-Strauss have good overall mortality rates but an unacceptably high rate of morbidity and relapse. ANCA positivity might be predictive of renal and neurological involvement and complications, whereas ENT involvement might be a good prognostic factor. […] Based on their findings, the authors suggest that the challenges of managing Churg-Strauss revolve around preventing morbidity and improving quality of life, which might be facilitated by prolonging use of low-dose corticosteroids and immunosuppressive drugs.
  • #53 Churg-Strauss Syndrome: ANCA Positivity and Long-Term Outcomes – Rheumatology Advisor
    https://www.rheumatologyadvisor.com/news/churg-strauss-syndrome-anca-positivity-and-long-term-outcomes/
    Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis, is a rare disorder that causes inflammation of the blood vessels, which can restrict blood flow to vital organs and tissue and cause permanent damage. Patients may have a variety of symptoms, such as asthma, gastrointestinal bleeding, pain, and rashes, which can significantly reduce their quality of life. […] The study findings suggest that patients with Churg-Strauss have good overall mortality rates but an unacceptably high rate of morbidity and relapse. ANCA positivity might be predictive of renal and neurological involvement and complications, whereas ENT involvement might be a good prognostic factor. […] Based on their findings, the authors suggest that the challenges of managing Churg-Strauss revolve around preventing morbidity and improving quality of life, which might be facilitated by prolonging use of low-dose corticosteroids and immunosuppressive drugs.
  • #54 Churg-Strauss syndrome (CSS; allergic granulomatous angiitis) | Healthengine Blog
    https://healthinfo.healthengine.com.au/churg-strauss-syndrome-css
    Churg-Strauss syndrome is a serious disease that can be fatal. Untreated it is extremely dangerous and threatens the organs that are affected. With aggressive treatment and monitoring it can be controlled fairly well. Total inactivation of the disease (remission) is possible as well. […] The treatment of patients with Churg-Strauss syndrome is directed toward both immediately reducing the inflammation of the blood vessels (vasculitis) and suppressing the immune system (because it is the bodys own immune system that is attacking the cells). Treatment usually includes high doses of corticosteroids medication (such as prednisone or prednisolone) to suppress the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan). […] If the patient is unresponsive to the above treatment schedule or if the disease is severe, other treatment may be used.
  • #55 Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment – PubMed
    https://pubmed.ncbi.nlm.nih.gov/21674415/
    Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. […] Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. […] All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses.
  • #56 Churg-Strauss syndrome | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/churg-strauss-syndrome
    For people with mild symptoms, a corticosteroid alone may be enough. Other people may need to add another drug to help suppress their immune systems. […] Because these drugs impair your body’s ability to fight infection and can cause other serious side effects, your condition will be closely monitored while you’re taking them. […] Long-term treatment with corticosteroids can cause a number of side effects. You can minimize these problems by taking the following steps: […] Ask your doctor how much vitamin D and calcium you need in your diet, and discuss whether you should take supplements. […] Exercise can help you maintain a healthy weight, which is important when you’re taking corticosteroid medications that can cause weight gain. […] Eat foods that help keep blood sugar stable, such as fruits, vegetables and whole grains. […] If you have signs and symptoms common to Churg-Strauss syndrome, make an appointment with your doctor. Early diagnosis and treatment significantly improve the outlook of this condition.
  • #57
    https://journals.lww.com/ijcn/fulltext/2019/20010/care_of_patients_with_eosinophilic_granulomatosis.4.aspx
    Nurses should be aware of the side effects of long-term glucocorticoid use and assess for these complications of patients with EGPA. […] Having regular follow-up, exercise and continuation of medications can minimise their complications and improve their span and quality of life. Nurses play a very important role in supporting and teaching the patients to adopt healthy lifestyle to remain free of pain and complications.
  • #58 Churg-Strauss syndrome from an orthopaedic perspective | HKMJ
    https://www.hkmj.org/abstracts/v21n6/565.htm
    With treatment, most of the symptoms in any of the three phases can be relieved. We would like to raise the awareness of such an entity in treating patients with neuropathy. Close collaboration with rheumatologists in treating such a complex illness, including prompt diagnosis by performing nerve biopsy by an orthopaedic surgeon and prompt medical treatment by a rheumatologist, is the key to successful management.
  • #59 Eosinophilic granulomatosis with polyangiitis – Knowledge @ AMBOSS
    https://www.amboss.com/us/knowledge/eosinophilic-granulomatosis-with-polyangiitis/
    Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules […] Management of EGPA is based on disease severity, taking into account the presence of organ- and/or life-threatening manifestations (e.g., alveolar hemorrhage, glomerulonephritis, limb ischemia). […] Consult rheumatology and other specialties (e.g., nephrology, pulmonology) as required. […] Goal of pharmacotherapy (e.g., glucocorticoids PLUS cyclophosphamide) is induction of remission […] Prevent complications of glucocorticoid therapy. […] Monitor for adverse effects of immunosuppressants. […] Consider pneumocystis pneumonia prophylaxis.
  • #60 Churg-Strauss Syndrome Symptoms, Causes, and Treatment
    https://www.verywellhealth.com/churg-strauss-sydrome-4174962
    Diagnosis can be complicated by the need to rule out other conditions (asthma, for example) but Churg-Strauss can be successfully treated using steroid therapy. Many people achieve complete remission from their symptoms. […] Left untreated, Churg-Strauss can cause potentially fatal complications. Talk to your healthcare provider if you have concerns about the disorder or experience specific symptoms.