Takayasu arteritis
Leczenie

Takayasu arteritis to przewlekłe zapalenie dużych naczyń, głównie aorty i jej odgałęzień, wymagające kompleksowego leczenia immunosupresyjnego. Podstawą terapii są glikokortykosteroidy, najczęściej prednizon w dawce początkowej 0,5-1 mg/kg mc./dobę (maksymalnie 60-70 mg/dobę), z późniejszym stopniowym zmniejszaniem dawki do około 10 mg/dobę w ciągu roku, dostosowanym indywidualnie do pacjenta. W celu ograniczenia działań niepożądanych GKS i kontroli choroby zaleca się włączenie leków immunosupresyjnych, takich jak metotreksat, azatiopryna, mykofenolan mofetylu czy leflunomid. W przypadkach opornych na leczenie konwencjonalne stosuje się leki biologiczne, przede wszystkim inhibitory TNF (infliksymab, adalimumab, etanercept) oraz tocilizumab, które wykazują wysoką skuteczność kliniczną i radiologiczną, umożliwiając redukcję dawki steroidów u większości pacjentów.

Leczenie Takayasu arteritis

Takayasu arteritis to rzadka postać zapalenia naczyń, która charakteryzuje się przewlekłym zapaleniem dużych naczyń krwionośnych, głównie aorty i jej głównych odgałęzień. Chociaż nie istnieje skuteczna metoda wyleczenia tego schorzenia, dostępne są różnorodne opcje terapeutyczne mające na celu kontrolę zapalenia, zapobieganie dalszemu uszkodzeniu naczyń oraz łagodzenie objawów. Leczenie Takayasu arteritis może być trudne, ponieważ choroba może pozostawać aktywna nawet gdy objawy ustępują. Ponadto w momencie diagnozy mogło już dojść do nieodwracalnych uszkodzeń naczyń.123

Leczenie farmakologiczne

Podstawą leczenia Takayasu arteritis są leki przeciwzapalne i immunosupresyjne, które mają na celu kontrolę procesu zapalnego i zapobieganie dalszemu uszkodzeniu naczyń krwionośnych.45

Glikokortykosteroidy

Glikokortykosteroidy stanowią podstawę leczenia pierwszego rzutu w aktywnej fazie choroby. Najczęściej stosowanym lekiem jest prednizon (Rayos, Prednisone Intensol) w dawce początkowej 0,5-1 mg/kg masy ciała dziennie (maksymalnie 60-70 mg/dobę). Efekty działania kortykosteroidów pojawiają się już w ciągu kilku godzin od pierwszej dawki.678

Dawka początkowa jest zwykle utrzymywana przez okres około 1 miesiąca, a następnie stopniowo zmniejszana w miarę ustępowania objawów klinicznych i normalizacji parametrów zapalnych. Zgodnie z wytycznymi European League Against Rheumatism (EULAR), zaleca się, aby u pacjentów, którzy osiągnęli dawkę 15-20 mg prednizonu po 2-3 miesiącach leczenia, dawka była powoli zmniejszana, dążąc do osiągnięcia 10 mg/dobę po upływie roku. Jednak dawka 10 mg/dobę w długoterminowej remisji może być zbyt wysoka w porównaniu z zaleceniami w innych chorobach zapalnych, takich jak reumatoidalne zapalenie stawów (zwykle 5 mg/dobę) i powinna być indywidualnie dostosowywana do każdego pacjenta w zależności od ryzyka powikłań związanych z GKS.91011

U około połowy pacjentów możliwe jest stopniowe odstawienie leków bez nawrotu choroby, jednak u pozostałych dochodzi do nawrotu objawów lub zaostrzenia choroby po zmniejszeniu dawki lub odstawieniu glikokortykosteroidów.1213

Leki immunosupresyjne

Ze względu na przewlekły i nawracający charakter choroby oraz konieczność unikania działań niepożądanych związanych z długotrwałym stosowaniem glikokortykosteroidów, często zaleca się dołączenie leków immunosupresyjnych. Eksperci sugerują systematyczne włączanie tych leków już w pierwszej linii leczenia w celu zmniejszenia dawki steroidów.141516

Do najczęściej stosowanych konwencjonalnych leków immunosupresyjnych należą:

  • Metotreksat (Trexall, Rheumatrex) – najczęściej stosowany lek immunosupresyjny w terapii Takayasu arteritis1718
  • Azatiopryna (Imuran, Azasan) – alternatywa dla metotreksatu1920
  • Mykofenolan mofetylu (CellCept, Myfortic) – wykazano skuteczność w kontrolowaniu aktywności choroby i hamowaniu progresji uszkodzeń naczyniowych2122
  • Leflunomid (Arava) – stosowany jako alternatywa dla innych leków immunosupresyjnych2324
  • Cyklofosfamid – stosowany głównie w ciężkich przypadkach, obecnie rzadziej stosowany ze względu na dostępność leków biologicznych2526
  • Takrolimus i cyklosporyna – inhibitory kalcyneuryny, które mogą być skuteczne w przypadkach opornych na standardowe leczenie2728
Leki biologiczne

W przypadku pacjentów opornych na standardowe leczenie lub wymagających długotrwałego stosowania wysokich dawek glikokortykosteroidów, stosowane są leki biologiczne. Najlepiej przebadanymi i najczęściej stosowanymi lekami z tej grupy są:293031

Metaanaliza przeprowadzona przez Misra i wsp. obejmująca 19 badań obserwacyjnych dotyczących inhibitorów TNF w Takayasu arteritis wykazała, że ponad 80% leczonych pacjentów uzyskało co najmniej częściową odpowiedź kliniczną, stabilizację angiograficzną, poprawę w badaniu PET-CT i normalizację markerów zapalnych.3233

  • Inhibitory interleukiny-6:
    • Tocilizumab (Actemra) – humanizowane przeciwciało monoklonalne blokujące receptor IL-6, które wykazało wysoką skuteczność w badaniach klinicznych

Systematyczny przegląd terapii tocilizumabem w Takayasu arteritis, który obejmował 105 pacjentów (76 z nich z chorobą oporną na leczenie), wykazał, że 85,7% pacjentów miało początkową odpowiedź kliniczną w ciągu 3 miesięcy, a 65,2% poprawę radiologiczną; 90,4% było w stanie zmniejszyć dawkę kortykosteroidów.343536

W badaniu TOCITAKA u pacjentów z nowo rozpoznanym Takayasu arteritis, leczonych tocilizumabem w połączeniu z glikokortykosteroidami, uzyskano 85% wskaźnik remisji i 54% przypadków odstawienia steroidów po 6 miesiącach terapii.37

Inne leki biologiczne badane w leczeniu Takayasu arteritis to:3839

  • Inhibitory JAK (tofacytynib, upadacytynib) – wpływają na szlak sygnałowy JAK/STAT, który odgrywa kluczową rolę w patogenezie choroby
  • Rytuksymab – przeciwciało monoklonalne przeciwko CD20 na limfocytach B
  • Ustekinumab – przeciwciało monoklonalne przeciwko IL-12/IL-23
  • Abatacept – wpływa na kostymulację limfocytów T
  • Sekukinumab – przeciwciało przeciwko IL-17
Leki wspomagające

W leczeniu Takayasu arteritis stosowane są również leki wspomagające, które mają na celu zapobieganie powikłaniom i kontrolę czynników ryzyka sercowo-naczyniowego:404142

  • Leki przeciwpłytkowe (aspiryna w małej dawce) – zmniejszają ryzyko zakrzepicy i niedokrwienia tkanki mózgowej
  • Leki przeciwnadciśnieniowe – konieczne u pacjentów z nadciśnieniem tętniczym, które często towarzyszy Takayasu arteritis
  • Statyny – obniżają poziom cholesterolu i zmniejszają ryzyko chorób serca; mogą również korzystnie wpływać na funkcję tętnic nawet przy prawidłowym poziomie cholesterolu
  • Suplementy wapnia i witaminy D – zapobiegają osteoporozie u pacjentów długotrwale leczonych glikokortykosteroidami

Leczenie interwencyjne i chirurgiczne

U pacjentów z ciężkim zwężeniem lub niedrożnością tętnic, które prowadzą do niedokrwienia narządów, lub z tętniakami zagrażającymi pęknięciem, konieczne może być leczenie interwencyjne lub chirurgiczne.434445

Interwencje naczyniowe są wskazane w następujących przypadkach:464748

  • Niedomykalność zastawki aortalnej
  • Zwężenie tętnicy wieńcowej powodujące objawową chorobę wieńcową lub niedokrwienną kardiomiopatię
  • Rozwarstwienie powiększonego tętniaka aorty
  • Ciężkie nadciśnienie tętnicze wtórne do zwężenia tętnicy nerkowej, oporne na leczenie farmakologiczne
  • Niedokrwienie kończyn zaburzające codzienne czynności
  • Niedokrwienie mózgu
  • Koarktacja aorty
  • Niemożność dokładnego pomiaru ciśnienia krwi (w żadnej kończynie)

Zabiegi chirurgiczne i endowaskularne powinny być wykonywane w okresie remisji choroby, gdy proces zapalny jest pod kontrolą. Do najczęściej stosowanych metod należą:495051

  • Operacja pomostowania naczyniowego (bypass) – polega na wszczepieniu przeszczepu naczyniowego (najlepiej autologicznego) omijającego zwężony lub niedrożny odcinek tętnicy. Zabieg ten ma najlepsze długoterminowe wskaźniki drożności i jest preferowany w przypadku nieodwracalnego zwężenia tętnic lub znacznej niedrożności przepływu krwi.
  • Angioplastyka przezskórna – zabieg polegający na wprowadzeniu cewnika z balonem do zwężonej tętnicy i rozszerzeniu jej przez napompowanie balonu. W niektórych przypadkach może być uzupełniona implantacją stentu (metalowej siateczki utrzymującej drożność naczynia).
  • Operacja zastawki aortalnej – naprawa lub wymiana zastawki aortalnej może być konieczna w przypadku znacznej niedomykalności.

W niedawnej metaanalizie porównującej wyniki angioplastyki balonowej i stentowania nie stwierdzono istotnych różnic w częstości występowania restenozy i innych powikłań ogółem, ale ryzyko restenozy po stentowaniu było znacznie wyższe niż po angioplastyce balonowej w przypadku zwężenia tętnic nerkowych.5253

Podejście wielodyscyplinarne i monitorowanie

Leczenie Takayasu arteritis wymaga podejścia wielodyscyplinarnego obejmującego reumatologów, kardiologów i chirurgów naczyniowych. Ze względu na rzadkość występowania tej choroby, zaleca się, aby pacjenci byli leczeni w ośrodkach i przez lekarzy z doświadczeniem w leczeniu tego schorzenia.545556

Choroba ma charakter przewlekły i wymaga długoterminowego leczenia u około 80% pacjentów. Leczenie zapobiega nasileniu zwężenia naczyń, ale zwykle nie poprawia objawów wynikających z trwałych uszkodzeń, które już wystąpiły.5758

Regularne monitorowanie aktywności choroby obejmuje:5960

  • Badania kliniczne co 1-3 miesiące w pierwszym roku po rozpoznaniu i co 3-6 miesięcy później
  • Oznaczanie markerów zapalnych (OB, CRP) – należy jednak pamiętać, że poziomy markerów zapalnych mogą nie odzwierciedlać wiarygodnie aktywności choroby, zwłaszcza w późnym stadium choroby
  • Sekwencyjne obrazowanie aorty w celu wykrycia progresji choroby
  • Ocena funkcji narządów zagrożonych niedokrwieniem

Podejście funkcjonalne i zalecenia żywieniowe

Poza konwencjonalnym leczeniem farmakologicznym i chirurgicznym, pacjenci z Takayasu arteritis mogą odnieść korzyść z holistycznego podejścia do zarządzania chorobą, które obejmuje:616263

  • Dieta przeciwzapalna – bogata w kwasy omega-3 (ryby), owoce i warzywa, z ograniczeniem żywności przetworzonej
  • Regularna aktywność fizyczna – dostosowana do możliwości pacjenta i stopnia zaawansowania choroby
  • Techniki zarządzania stresem – jak medytacja i joga, które mogą pomóc w kontroli stresu, który może nasilać objawy choroby
  • Utrzymanie prawidłowej masy ciała – wspiera ogólny dobrostan i zmniejsza ryzyko powikłań
  • Edukacja pacjenta – zwiększa świadomość choroby i umożliwia aktywny udział w procesie leczenia

Wytyczne i rekomendacje dotyczące leczenia Takayasu arteritis

Wytyczne dotyczące postępowania w Takayasu arteritis zostały opracowane przez kilka towarzystw naukowych, w tym:646566

  1. European League Against Rheumatism (EULAR) – zaktualizowane w 2018 roku
  2. American College of Rheumatology (ACR)/Vasculitis Foundation – opublikowane w 2021 roku
  3. Japanese Circulation Society (JCS) – wytyczne z 2017 roku

Główne zalecenia obejmują:676869

  • Glikokortykosteroidy jako podstawa leczenia indukującego remisję
  • Dodanie konwencjonalnych leków modyfikujących przebieg choroby (DMARD) do glikokortykosteroidów u wszystkich pacjentów z Takayasu arteritis
  • Stosowanie leków biologicznych (tocilizumab lub inhibitory TNF) u pacjentów z chorobą oporną na leczenie lub przy wysokim ryzyku wczesnych nawrotów
  • Unikanie interwencji naczyniowych w aktywnej fazie choroby, jeśli to możliwe

Wytyczne ACR z 2021 roku zalecają dodanie inhibitora TNF zamiast tocilizumabu u pacjentów opornych na leczenie, podczas gdy wytyczne EULAR sugerują, że tocilizumab lub inhibitory TNF mogą być równoważnie rozważane.7071

Rokowanie i przebieg długoterminowy

Takayasu arteritis jest chorobą przewlekłą, która wymaga długoterminowego leczenia i monitorowania. Rokowanie jest zróżnicowane i zależy od wielu czynników, w tym od ciężkości choroby, czasu rozpoczęcia leczenia i odpowiedzi na terapię.7273

  • Około 80% pacjentów z Takayasu arteritis może skutecznie kontrolować swoją chorobę przy odpowiednim leczeniu
  • U niektórych pacjentów choroba wchodzi w remisję, co pozwala na zmniejszenie lub nawet odstawienie leków
  • U około 25% pacjentów nie udaje się całkowicie opanować choroby bez ciągłego stosowania leków
  • Wczesne rozpoznanie i leczenie są kluczowe dla poprawy rokowania

Długoterminowe powikłania wynikają głównie z niedrożności tętnic i związanych z tym uszkodzeń, w tym niedokrwienia kończyn oraz powikłań sercowych i neurologicznych.7475

Częstość nawrotów po odstawieniu leków jest dość wysoka – badania sugerują, że nawet po skutecznym leczeniu tocilizumabem, wskaźnik nawrotów wynosi około 45% po przerwaniu terapii.76

Podsumowanie

Leczenie Takayasu arteritis wymaga kompleksowego podejścia obejmującego leczenie farmakologiczne, interwencje naczyniowe w wybranych przypadkach oraz modyfikację stylu życia. Podstawą terapii są glikokortykosteroidy w połączeniu z lekami immunosupresyjnymi, które mają na celu kontrolę zapalenia i zapobieganie dalszemu uszkodzeniu naczyń. W przypadkach opornych na leczenie konwencjonalne, leki biologiczne, takie jak inhibitory TNF i tocilizumab, oferują obiecujące wyniki.

Ze względu na przewlekły charakter choroby, kluczowe znaczenie ma regularne monitorowanie aktywności choroby oraz współpraca wielodyscyplinarnego zespołu medycznego. Wczesne rozpoznanie i rozpoczęcie leczenia, standardowe podejście terapeutyczne oraz właściwe postępowanie w przypadku powikłań są istotne dla poprawy rokowania i jakości życia pacjentów z Takayasu arteritis.7778

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Materiały źródłowe

  • #1 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340
    Treatment of Takayasu’s arteritis focuses on controlling inflammation with medications and preventing further damage to your blood vessels. […] Takayasu’s arteritis can be difficult to treat because the disease may remain active even if your symptoms improve. It’s also possible that irreversible damage has already occurred by the time you’re diagnosed. […] On the other hand, if you don’t have signs and symptoms or serious complications, you may not need treatment or you may be able to taper and stop treatment if your doctor recommends it. […] Talk with your doctor about the drug or drug combinations that are options for you and their possible side effects. Your doctor may prescribe: […] Corticosteroids to control inflammation. The first line of treatment is usually a corticosteroid, such as prednisone (Prednisone Intensol, Rayos). Even if you start feeling better, you may need to continue taking the drug long term. After a few months, your doctor may gradually begin to lower the dose until you reach the lowest dose you need to control inflammation. Eventually your doctor may tell you to stop taking the medication completely. Possible side effects of corticosteroids include weight gain, increased risk of infection and bone thinning. To help prevent bone loss, your doctor may recommend a calcium supplement and vitamin D.
  • #2 Takayasu’s Arteritis: Causes, Symptoms and Treatment
    https://my.clevelandclinic.org/health/diseases/7097-takayasus-arteritis
    Takayasus arteritis is a rare type of vasculitis, or blood vessel inflammation. […] Although there is no cure for it, you can treat it with medication and/or surgery. […] Medicines can help control inflammation, but some people may need surgery to create a way around a blocked artery. […] Corticosteroids like prednisone (Rayos or Sterapred) or prednisolone (Flo-Pred or Orapred) are the most common treatments for TAK. […] Usually, providers also prescribe immunosuppressive medications such as Methotrexate (Rheumatrex or Trexall), Azathioprine (Imuran or Azasan), Mycophenolate (Cellcept or Myfortic), Leflunomide, Cyclophosphamide. […] Taking immunosuppressive drugs in addition to prednisone can help 50% of people who previously relapsed get rid of their symptoms and gradually stop taking prednisone.
  • #3 Treatment of Takayasu arteritis – UpToDate
    https://www.uptodate.com/contents/treatment-of-takayasu-arteritis
    Treatment of Takayasu arteritis […] The treatment of TAK will be reviewed here. […] The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a „steroid-sparing” benefit and longer-term disease control. […] Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. […] It is recommended that patients with TAK be evaluated and managed by centers and physicians with experience treating this rare disease.
  • #4 Takayasu’s arteritis – Symptoms & causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/symptoms-causes/syc-20351335
    If you don’t have symptoms, you may not need treatment. But most people with the disease need medications to control inflammation in the arteries and to prevent complications. […] Early detection of Takayasu’s arteritis is key to getting effective treatment. […] If you’ve already been diagnosed with Takayasu’s arteritis, keep in mind that your symptoms may come and go even with effective treatment.
  • #5 Takayasu’s Arteritis | UVA Health
    https://uvahealth.com/services/aortic-artery-disease/takayasus-arteritis
    Takayasu arteritis is a chronic condition. That means we can treat it, but it wont go away completely. The swelling will come and go, for better or worse. […] Treating Takayasu arteritis involves controlling the swelling with medicine. We treat Takayasu arteritis with: Corticosteroids (to reduce swelling), Immunosuppressants (to help the corticosteroids reduce swelling), Biologics (when other medicines dont work). […] If you have blockages in your blood vessels, you might also need surgery.
  • #6 Takayasu Arteritis Treatment & Management: Approach Considerations, Surgical Therapy, Follow-Up
    https://emedicine.medscape.com/article/332378-treatment
    Medical management of Takayasu arteritis depends on the disease activity and the complications that develop. Some patients have only mild forms of the disease; others deteriorate considerably. The two most important aspects of treatment are controlling the inflammatory process and controlling hypertension. […] Corticosteroids are the mainstay of therapy for active Takayasu arteritis. However, some patients may also require cytotoxic agents, to achieve remission and taper of long-term corticosteroid treatment. […] Oral corticosteroids are started at 1 mg/kg daily or divided twice daily and tapered over weeks to months as symptoms subside. […] Long-term, low-dose corticosteroid therapy may be required. Osteoporosis prevention when patients are started on corticosteroids should be seriously considered.
  • #7 French recommendations for the management of Takayasu’s arteritis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01922-1
    The aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and care approach for a patient with Takayasus arteritis. […] Oral corticosteroid therapy is the standard treatment for Takayasus arteritis. It is introduced at the initial dose of 0.5 to 1 mg/kg/day of oral prednisone in an adult. […] Experts suggest putting immunosuppressants (i.e.methotrexate) systematically on as the first line for the purpose of reducing the use of steroids. The immunosuppressants for which there is the most data are methotrexate. Biotherapies have also been shown to be effective in these situations, including anti-TNF-alpha (infliximab, adalimumab, etanercept) and anti-IL6 (tocilizumab). […] The treatment of the conventional approach is prednisone at a dose of 0.5 to 1 mg/kg/d (maximum dosage of 70 mg/day) in the inflammatory phase.
  • #8 Takayasu’s Arteritis: Symptoms, Causes, Diagnosis & Treatment
    https://www.webmd.com/heart-disease/takayasus-arteritis
    There is no cure for Takayasu’s arteritis, but treatment can help manage symptoms of the condition. Treatment focuses on using medications to lower inflammation as well as lower blood pressure, preventing further damage to the affected arteries and tissues. […] Although statistics vary, one study found that about 80% of people with Takayasu’s arteritis can manage their condition with proper treatment. Some even go into remission. This doesn’t mean theyre cured, but the disease isnt active, and they might be able to reduce or even stop their medications. […] Corticosteroids, also called systemic glucocorticoids or just steroids, are usually the first treatment for Takayasu’s arteritis. Steroids work within hours after the first dose is given. The most common drug in this class is prednisone. While this drug is often highly effective, it may be only partially effective for some people.
  • #9 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    Glucocorticoids are the mainstay of treatment for remission induction in TAK. The initial dose of prednisolone is 1 mg/kg/day (maximum 60 mg/day). The initial high dose should be maintained for a month and tapered gradually. According to the 2018 update of the EULAR recommendations for the management of LVV it was recommended that in patients who have reached 15-20 mg daily GC dose after 2-3 months, GCs should be decreased slowly targeting 10 mg/day at the end of one year. However, 10 mg/day doses of GCs in long-term remission are possibly too high compared with the recommendations in other disorders such as rheumatoid arthritis (usually 5 mg/day) and should be individually assessed in each patient according to the risk of GC-associated complications. Recent ACR guideline conditionally recommend tapering off glucocorticoids over long-term treatment with low-dose glucocorticoids for remission maintenance in TAK patients achieved remission while receiving GCs for 6-12 months. Both EULAR and ACR recommend the use of non-biologic disease-modifying agents in addition to glucocorticoids in all patients with TAK.
  • #10 Takayasu Arteritis Treatment & Management: Approach Considerations, Surgical Therapy, Follow-Up
    https://emedicine.medscape.com/article/332378-treatment
    Medical management of Takayasu arteritis depends on the disease activity and the complications that develop. Some patients have only mild forms of the disease; others deteriorate considerably. The two most important aspects of treatment are controlling the inflammatory process and controlling hypertension. […] Corticosteroids are the mainstay of therapy for active Takayasu arteritis. However, some patients may also require cytotoxic agents, to achieve remission and taper of long-term corticosteroid treatment. […] Oral corticosteroids are started at 1 mg/kg daily or divided twice daily and tapered over weeks to months as symptoms subside. […] Long-term, low-dose corticosteroid therapy may be required. Osteoporosis prevention when patients are started on corticosteroids should be seriously considered.
  • #11 French recommendations for the management of Takayasu’s arteritis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01922-1
    The initial dose is usually maintained for 24 weeks. The reduction phase can begin as soon as the clinical signs and biological inflammatory syndrome have significantly improved. […] Most experts recommended the use of methotrexate as the first line for the purpose of reducing the use of steroids. […] The use of cyclophosphamide reported in older publications is no longer routinely used with the advent of biotherapies. […] The value of biotherapies has been evaluated primarily in retrospective open studies in patients resistant to conventional immunosuppressants or corticosteroid-dependent. […] Several retrospective open series have shown the effectiveness of tocilizumab, an anti-IL6R, in terms of clinical-biological remission and a steroid-sparing effect. […] The main issue is knowing to whom and when to prescribe the adjuvant treatment.
  • #12 Takayasu’s Arteritis: Symptoms, Causes, Diagnosis & Treatment
    https://www.webmd.com/heart-disease/takayasus-arteritis
    If prednisone works and your disease is under control, your doctor will likely slowly lower the dosage of prednisone (a steroid) to sustain improvement, thereby trying to lessen treatment side effects. […] In some people, it is possible to gradually stop medication without a relapse, but about half of all patients will have repeated symptoms or worsening of the illness. […] Nonglucocorticoid immunosuppressive agents (drugs) are medications that suppress the immune system but aren’t steroids. These drugs fall into one of two classifications: nonbiologics (made purely with synthetic chemicals) and biologics (grown from living cells). […] If corticosteroids don’t work on their own or if the symptoms return after tapering, your doctor may recommend these nonbiologic drugs to suppress your immune system and the inflammation: Azathioprine (Imuran), Cyclophosphamide, Leflunomide (Arava), Methotrexate, Mycophenolate mofetil.
  • #13 Takayasu’s Arteritis: Causes, Symptoms and Treatment
    https://my.clevelandclinic.org/health/diseases/7097-takayasus-arteritis
    Some people may also take tumor-necrosis factor (TNF) inhibitors to fight inflammation. […] In some instances, narrowing of arteries to your kidney may cause hypertension (high blood pressure). Your provider can stretch narrow vessel openings with a balloon (angioplasty) or do a bypass operation to restore normal flow to your kidney. […] Some people with Takayasus arteritis may need an aortic valve replacement. […] Overall, about 25% of people who have Takayasus arteritis won’t be able to manage their disease completely without continued use of medication. […] Medicines for treating Takayasus arteritis can have serious side effects, but other medicines can help with these side effects. […] Corticosteroids work within hours after you receive your first dose. […] Although there is no cure for Takayasus arteritis, it is a treatable disease. Most people who have it improve with treatment. […] Takayasus arteritis is a chronic disease that progresses slowly. You’ll need long-term treatment, most likely with medicine.
  • #14 French recommendations for the management of Takayasu’s arteritis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01922-1
    The aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and care approach for a patient with Takayasus arteritis. […] Oral corticosteroid therapy is the standard treatment for Takayasus arteritis. It is introduced at the initial dose of 0.5 to 1 mg/kg/day of oral prednisone in an adult. […] Experts suggest putting immunosuppressants (i.e.methotrexate) systematically on as the first line for the purpose of reducing the use of steroids. The immunosuppressants for which there is the most data are methotrexate. Biotherapies have also been shown to be effective in these situations, including anti-TNF-alpha (infliximab, adalimumab, etanercept) and anti-IL6 (tocilizumab). […] The treatment of the conventional approach is prednisone at a dose of 0.5 to 1 mg/kg/d (maximum dosage of 70 mg/day) in the inflammatory phase.
  • #15 Treatment of Takayasu arteritis – UpToDate
    https://www.uptodate.com/contents/treatment-of-takayasu-arteritis
    Treatment of Takayasu arteritis […] The treatment of TAK will be reviewed here. […] The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a „steroid-sparing” benefit and longer-term disease control. […] Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. […] It is recommended that patients with TAK be evaluated and managed by centers and physicians with experience treating this rare disease.
  • #16 Novel Therapies in Takayasu Arteritis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8790042/
    Given the high frequency of GC adverse effects and the high rate of relapse during tapering, the upfront use of immunosuppressives in addition to GC seems to be the most preferable management strategy in TAK patients. […] EULAR recommendations in 2018 suggested choosing as first line agent a csDMARD among methotrexate, azathioprine, mycophenolate mofetil or leflunomide. […] However, new therapeutic approaches are needed for patients with refractory disease or contraindications to conventional therapies. […] Tumor Necrosis Factor- (TNF-) and Interleukin 6 (IL-6) seem to be the most promising therapeutic targets, but other pathways have been studied, and will be discussed in the next sections. […] A recent meta-analysis by Misra et al. analyzed 19 observational studies on TNFi in TAK, showing that more than 80% of treated patients attained at least partial clinical response, angiographic stabilization, improvement in PET-CT and normalization of inflammatory markers.
  • #17 Takayasu’s Arteritis: Causes, Symptoms and Treatment
    https://my.clevelandclinic.org/health/diseases/7097-takayasus-arteritis
    Takayasus arteritis is a rare type of vasculitis, or blood vessel inflammation. […] Although there is no cure for it, you can treat it with medication and/or surgery. […] Medicines can help control inflammation, but some people may need surgery to create a way around a blocked artery. […] Corticosteroids like prednisone (Rayos or Sterapred) or prednisolone (Flo-Pred or Orapred) are the most common treatments for TAK. […] Usually, providers also prescribe immunosuppressive medications such as Methotrexate (Rheumatrex or Trexall), Azathioprine (Imuran or Azasan), Mycophenolate (Cellcept or Myfortic), Leflunomide, Cyclophosphamide. […] Taking immunosuppressive drugs in addition to prednisone can help 50% of people who previously relapsed get rid of their symptoms and gradually stop taking prednisone.
  • #18 French recommendations for the management of Takayasu’s arteritis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01922-1
    The initial dose is usually maintained for 24 weeks. The reduction phase can begin as soon as the clinical signs and biological inflammatory syndrome have significantly improved. […] Most experts recommended the use of methotrexate as the first line for the purpose of reducing the use of steroids. […] The use of cyclophosphamide reported in older publications is no longer routinely used with the advent of biotherapies. […] The value of biotherapies has been evaluated primarily in retrospective open studies in patients resistant to conventional immunosuppressants or corticosteroid-dependent. […] Several retrospective open series have shown the effectiveness of tocilizumab, an anti-IL6R, in terms of clinical-biological remission and a steroid-sparing effect. […] The main issue is knowing to whom and when to prescribe the adjuvant treatment.
  • #19 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340
    Other drugs that suppress the immune system. If your condition doesn’t respond well to corticosteroids or you have trouble as your medication dose is lowered, your doctor may prescribe drugs such as methotrexate (Trexall, Xatmep, others), azathioprine (Azasan, Imuran) and leflunomide (Arava). Some people respond well to medications that were developed for people receiving organ transplants, such as mycophenolate mofetil (CellCept). The most common side effect is an increased risk of infection. […] Medications to regulate the immune system. If you don’t respond to standard treatments, your doctor may suggest drugs that correct abnormalities in the immune system (biologics), although more research is needed. Examples of biologics include etanercept (Enbrel), infliximab (Remicade) and tocilizumab (Actemra). The most common side effect with these drugs is an increased risk of infection.
  • #20 Takayasu Arteritis Medication: Corticosteroids, Antineoplastics, Other, Immunosuppressants, DMARDs, TNF Inhibitors, Calcium Channel Blockers, Antiplatelet Agents, Hematologic, Anticoagulants, Hematologic
    https://emedicine.medscape.com/article/332378-medication
    The goals of therapy in Takayasu arteritis are to reduce inflammation and suppress autoimmune disease. To treat the active disease, corticosteroids are used and gradually tapered. Agents such as tumor necrosis factor inhibitors and tocilizumab are used along with corticosteroids for active disease. Cytotoxic agents such as methotrexate, azathioprine, and cyclophosphamide are the main therapeutic agents when the response to steroids is unsatisfactory. […] The immunosuppressant prednisone is a first-line therapy for autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear leukocyte activity and CD4 counts. […] Cyclophosphamide or methotrexate either in combination with prednisone or alone can be used to suppress active disease in patients who are not responding to prednisone.
  • #21 The efficacy of Mycophenolate mofetil for the treatment of Chinese Takayasu’s arteritis | Scientific Reports
    https://www.nature.com/articles/srep38687
    To investigate the therapeutic effect of mycophenolate mofetil(MMF) on Chinese Takayasus arteritis(TAK) patients. MMF combined with glucocorticoid was the primary treatment regimen. The effectiveness of MMF was defined as: (1) ESR20mm/hr; (2) CRP10mg/L or hs-CRP3mg/L; (3) stable or improved in vascular image studies; (4) clinical assessment is stable, improved or in remission; (5) the dosage of glucocorticoid could be tapered to less than 15mg/day. MMF alone combined with corticosteroid was effective in 12(40.0%) patients. The overall effective rate of therapy including MMF in treating TAK is 80%. […] Mycophenolate mofetil (MMF) has been reported in a few studies as a useful alternative for TAK treatment. […] In this study, we have demonstrated that MMF may be effective in controlling disease activity and vessel damage progression, either combined with low-dosage GCs or combined with MTX or AZA, with the total effective rate up to 80.0%. […] Therefore, we may conclude that MMF may be an effective alternative for disease control and tapering of steroid in the treatment of TAK. It offers a good choice for active TAK patients.
  • #22 Takayasu’s Arteritis: Causes, Symptoms and Treatment
    https://my.clevelandclinic.org/health/diseases/7097-takayasus-arteritis
    Takayasus arteritis is a rare type of vasculitis, or blood vessel inflammation. […] Although there is no cure for it, you can treat it with medication and/or surgery. […] Medicines can help control inflammation, but some people may need surgery to create a way around a blocked artery. […] Corticosteroids like prednisone (Rayos or Sterapred) or prednisolone (Flo-Pred or Orapred) are the most common treatments for TAK. […] Usually, providers also prescribe immunosuppressive medications such as Methotrexate (Rheumatrex or Trexall), Azathioprine (Imuran or Azasan), Mycophenolate (Cellcept or Myfortic), Leflunomide, Cyclophosphamide. […] Taking immunosuppressive drugs in addition to prednisone can help 50% of people who previously relapsed get rid of their symptoms and gradually stop taking prednisone.
  • #23 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    There are very low quality data coming from observational studies and case series showing the efficacy and safety of metotrexate (MTX), azathiopurine (AZA), mycophenolate mofetil (MMF), leflunomide (LEF), cyclophosphamide (CYC) in TAK treatment. Two open prospective series from China reported better outcomes with LEF than with CYC. Tacrolimus and cyclosporine, which are calcineurin inhibitors widely used in transplant patients, were reported to be effective in very few cases with TAK. Tofacitinib compared with MTX and LEF in an open prospective series in TAK was found to be superior to LEF preventing relapse and decreasing the GC dose. There are only 2 double-blind RCTs on TAK treatment. Abatacept and tocilizumab (TCZ) failed in these studies when compared with placebo. Long-term results of TCZ RCT study reported angiographic stabilization in patients. A recent open RCT reported similar clinical responses and angiographic stabilization in TAK patients treated with mycophenolate or methotrexate.
  • #24 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340
    Other drugs that suppress the immune system. If your condition doesn’t respond well to corticosteroids or you have trouble as your medication dose is lowered, your doctor may prescribe drugs such as methotrexate (Trexall, Xatmep, others), azathioprine (Azasan, Imuran) and leflunomide (Arava). Some people respond well to medications that were developed for people receiving organ transplants, such as mycophenolate mofetil (CellCept). The most common side effect is an increased risk of infection. […] Medications to regulate the immune system. If you don’t respond to standard treatments, your doctor may suggest drugs that correct abnormalities in the immune system (biologics), although more research is needed. Examples of biologics include etanercept (Enbrel), infliximab (Remicade) and tocilizumab (Actemra). The most common side effect with these drugs is an increased risk of infection.
  • #25 French recommendations for the management of Takayasu’s arteritis | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-021-01922-1
    The initial dose is usually maintained for 24 weeks. The reduction phase can begin as soon as the clinical signs and biological inflammatory syndrome have significantly improved. […] Most experts recommended the use of methotrexate as the first line for the purpose of reducing the use of steroids. […] The use of cyclophosphamide reported in older publications is no longer routinely used with the advent of biotherapies. […] The value of biotherapies has been evaluated primarily in retrospective open studies in patients resistant to conventional immunosuppressants or corticosteroid-dependent. […] Several retrospective open series have shown the effectiveness of tocilizumab, an anti-IL6R, in terms of clinical-biological remission and a steroid-sparing effect. […] The main issue is knowing to whom and when to prescribe the adjuvant treatment.
  • #26 Takayasu Arteritis Medication: Corticosteroids, Antineoplastics, Other, Immunosuppressants, DMARDs, TNF Inhibitors, Calcium Channel Blockers, Antiplatelet Agents, Hematologic, Anticoagulants, Hematologic
    https://emedicine.medscape.com/article/332378-medication
    The goals of therapy in Takayasu arteritis are to reduce inflammation and suppress autoimmune disease. To treat the active disease, corticosteroids are used and gradually tapered. Agents such as tumor necrosis factor inhibitors and tocilizumab are used along with corticosteroids for active disease. Cytotoxic agents such as methotrexate, azathioprine, and cyclophosphamide are the main therapeutic agents when the response to steroids is unsatisfactory. […] The immunosuppressant prednisone is a first-line therapy for autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear leukocyte activity and CD4 counts. […] Cyclophosphamide or methotrexate either in combination with prednisone or alone can be used to suppress active disease in patients who are not responding to prednisone.
  • #27 Successful Treatment of Refractory Takayasu Arteritis with Tacrolimus | The Journal of Rheumatology
    https://www.jrheum.org/content/39/7/1487
    Takayasu arteritis (TA) is a systemic vasculitis that affects large-size vessels such as the aorta and/or its main branches. […] Although high-dose corticosteroid (CS) therapy is effective in TA, CS alone does not provide sustained remission in about half of patients. […] CS-resistant patients with TA have been treated with immunosuppressants, including methotrexate (MTX) and azathioprine. […] Anti-tumor necrosis factor (anti-TNF) therapy and anti-interleukin 6 receptor antibody are also promising treatments for TA. […] We describe a patient with TA who was successfully treated with tacrolimus, a calcineurin inhibitor, after failed trial of conventional CS, MTX, and infliximab (IFX). […] This case suggests that tacrolimus is another potential alternative treatment option for refractory TA.
  • #28 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    There are very low quality data coming from observational studies and case series showing the efficacy and safety of metotrexate (MTX), azathiopurine (AZA), mycophenolate mofetil (MMF), leflunomide (LEF), cyclophosphamide (CYC) in TAK treatment. Two open prospective series from China reported better outcomes with LEF than with CYC. Tacrolimus and cyclosporine, which are calcineurin inhibitors widely used in transplant patients, were reported to be effective in very few cases with TAK. Tofacitinib compared with MTX and LEF in an open prospective series in TAK was found to be superior to LEF preventing relapse and decreasing the GC dose. There are only 2 double-blind RCTs on TAK treatment. Abatacept and tocilizumab (TCZ) failed in these studies when compared with placebo. Long-term results of TCZ RCT study reported angiographic stabilization in patients. A recent open RCT reported similar clinical responses and angiographic stabilization in TAK patients treated with mycophenolate or methotrexate.
  • #29 Novel Therapies in Takayasu Arteritis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8790042/
    Given the high frequency of GC adverse effects and the high rate of relapse during tapering, the upfront use of immunosuppressives in addition to GC seems to be the most preferable management strategy in TAK patients. […] EULAR recommendations in 2018 suggested choosing as first line agent a csDMARD among methotrexate, azathioprine, mycophenolate mofetil or leflunomide. […] However, new therapeutic approaches are needed for patients with refractory disease or contraindications to conventional therapies. […] Tumor Necrosis Factor- (TNF-) and Interleukin 6 (IL-6) seem to be the most promising therapeutic targets, but other pathways have been studied, and will be discussed in the next sections. […] A recent meta-analysis by Misra et al. analyzed 19 observational studies on TNFi in TAK, showing that more than 80% of treated patients attained at least partial clinical response, angiographic stabilization, improvement in PET-CT and normalization of inflammatory markers.
  • #30 Takayasu Arteritis Treatment & Management: Approach Considerations, Surgical Therapy, Follow-Up
    https://emedicine.medscape.com/article/332378-treatment
    Evidence supports interleukin-6 (IL-6) as a major component in the inflammatory process of large-vessel vasculitis, and case reports and observational studies have shown that the humanized monoclonal antibody tocilizumab, which blocks the soluble IL-6 receptor, can produce clinical responses and have a steroid-sparing effect in patients with refractory Takayasu arteritis, including patients refractory to tumor necrosis factor (TNF) inhibitors. […] A systematic review of tocilizumab therapy for Takayasu arteritis that included 105 patients, 76 of them with refractory disease, found that 85.7% of patients had an initial clinical response within 3 months and 65.2% had radiologic improvement; 90.4% were able to reduce their corticosteroid dose. […] The 2021 American College of Rheumatology/Vasculitis Foundation (ACR/VF) guideline conditionally recommends tumor necrosis factor (TNF) inhibitors for use in combination with glucocorticoids in patients with active Takayasu arteritis.
  • #31 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    Several case series and observational studies reported the efficacy and safety of TNF inhibitors (TNFi) and TCZ in TAK. Two large retrospective comparison studies found similar clinical response rates and radiologic progression between TNFi or TCZ. A recent meta-analysis also confirmed similar clinical response, angiographic stabilization, and adverse events with TNFi or TCZ. In a head-to-head retrospective comparison, the drug survival rate of TNF was significantly higher than that of TCZ. A retrospective, longitudinal follow-up cohort from Norway reported less angiographic progression at 2 years in patients with TAK receiving TNFi than in those receiving conventional immunosuppressive. According to EULAR recommendations, TCZ or TNFi can be equally considered in refractory patients. However, recent ACR guidelines recommend adding a TNFi over TCZ in refractory patients. A very recent open prospective study compared secukinumab and TNFi in patients with refractory TAK secukinumab and TNFi were found to be comparable regarding response rates at 3 and 6 months.
  • #32 Novel Therapies in Takayasu Arteritis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8790042/
    Given the high frequency of GC adverse effects and the high rate of relapse during tapering, the upfront use of immunosuppressives in addition to GC seems to be the most preferable management strategy in TAK patients. […] EULAR recommendations in 2018 suggested choosing as first line agent a csDMARD among methotrexate, azathioprine, mycophenolate mofetil or leflunomide. […] However, new therapeutic approaches are needed for patients with refractory disease or contraindications to conventional therapies. […] Tumor Necrosis Factor- (TNF-) and Interleukin 6 (IL-6) seem to be the most promising therapeutic targets, but other pathways have been studied, and will be discussed in the next sections. […] A recent meta-analysis by Misra et al. analyzed 19 observational studies on TNFi in TAK, showing that more than 80% of treated patients attained at least partial clinical response, angiographic stabilization, improvement in PET-CT and normalization of inflammatory markers.
  • #33 Congress of Clinical Rheumatology Annual Meeting
    https://www.healio.com/news/rheumatology/20230909/we-should-be-thinking-cure-for-giant-cell-takayasu-arteritis-as-trial-data-grow
    We are most confident in the use of methotrexate, TNF inhibitors and tocilizumab, Merkel said. […] Although TNF inhibitors are not recommended for GCA, the situation is different for Takayasus arteritis. […] The key study of tocilizumab in Takayasu arteritis was conducted in 2017, Merkel said, stressing that more data are necessary. […] Despite the dearth of data, Merkel stated he is confident and optimistic that more options for managing patients with Takayasu arteritis will be available in the coming years, for one important reason. […] As researchers continue to build the evidence base for treatments of these two diseases, Merkel offered an ambitious objective.
  • #34 Takayasu Arteritis Treatment & Management: Approach Considerations, Surgical Therapy, Follow-Up
    https://emedicine.medscape.com/article/332378-treatment
    Evidence supports interleukin-6 (IL-6) as a major component in the inflammatory process of large-vessel vasculitis, and case reports and observational studies have shown that the humanized monoclonal antibody tocilizumab, which blocks the soluble IL-6 receptor, can produce clinical responses and have a steroid-sparing effect in patients with refractory Takayasu arteritis, including patients refractory to tumor necrosis factor (TNF) inhibitors. […] A systematic review of tocilizumab therapy for Takayasu arteritis that included 105 patients, 76 of them with refractory disease, found that 85.7% of patients had an initial clinical response within 3 months and 65.2% had radiologic improvement; 90.4% were able to reduce their corticosteroid dose. […] The 2021 American College of Rheumatology/Vasculitis Foundation (ACR/VF) guideline conditionally recommends tumor necrosis factor (TNF) inhibitors for use in combination with glucocorticoids in patients with active Takayasu arteritis.
  • #35 Current Immunosuppressive Treatment for Takayasu Arteritis
    https://www.jstage.jst.go.jp/article/circj/advpub/0/advpub_CJ-23-0780/_html/-char/en
    TCZ is a recombinant humanized anti-IL-6 receptor monoclonal antibody, which case reports and case series since 2008 have reported as effective in refractory TAK. […] In a large retrospective multicenter study of 46 TAK patients, TCZ reduced the median National Institutes of Health disease activity score and GC dose, and event-free survival was significantly better with TCZ than DMARDs. […] It was on the basis of this evidence that TCZ was a Class I treatment recommendation in the 2017 JCS guidelines. […] A more recent systematic review/meta-analysis reported equivalent efficacy for TCZ and TNFi. […] In a recent observational study of 120 TAK patients treated with TCZ, 83.0% of relapse-free patients were able to reduce their GC intake to 10 mg/day prednisolone dose equivalent or less.
  • #36 Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labeled trial | Arthritis Research & Therapy | Full Text
    https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-020-02311-y
    To assess long-term efficacy of tocilizumab in treatment-naive patients with Takayasu arteritis (TAK). […] The primary endpoint was the number of patients who discontinued steroids after 7 infusions of tocilizumab. […] A significant decrease of disease activity was observed after 6 months of tocilizumab therapy: decrease of median NIH scale (3 [3, 4] at baseline, versus 1 [02] after 6 months; p0.001), ITAS-2010 score (5 [27] versus 3 [08]; p=0.002), and ITAS-A score (7 [410] versus 4 [115]; p=0.0001). […] Tocilizumab seems an effective steroid sparing therapy in TAK, but maintenance therapy is necessary. […] Tocilizumab addition to steroids in treatment-nave Takayasu patients is highly effective, but maintenance therapy is necessary. […] This is the first prospective trial aiming to evaluate the benefit of a combination of biological-targeted therapy with steroids, to allow steroid discontinuation among treatment-nave patients with TAK.
  • #37 Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labeled trial | Arthritis Research & Therapy | Full Text
    https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-020-02311-y
    Tocilizumab combined to steroids in treatment-nave patients with TAK is highly effective, with 85% remission rate and 54% of steroid discontinuation after 6 months of therapy, and despite the high remission rates obtained using tocilizumab, relapse rates were of 45% after discontinuation. […] Our study is the first to show that a high response rate can be achieved by adding tocilizumab to conventional steroids and that 36% of patients with TAK will remain without treatment during at least 18 months. […] Further studies among treatment-nave patients with TAK are necessary to determine the best maintenance therapy strategy.
  • #38 Novel Therapies in Takayasu Arteritis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8790042/
    Tocilizumab is a humanized monoclonal antibody blocker of IL-6 signaling and has been approved for the treatment of Giant Cells Arteritis. […] The above-mentioned meta-analysis performed by Misra et al. included 22 observational studies on tocilizumab in TAK patients. […] JAK-Inhibitors are a more recent family of drugs, classified as targeted synthetic DMARDs (tsDMARDs). […] There is increasing evidence about the possible role of B-cells in the pathogenesis of TAK. […] As discussed above, new therapeutic approaches with bDMARDs and tsDMARDs have showed promising results, with high efficacy and acceptable safety profile. […] In 2018, based on these new insights, EULAR recommendations advised the use of bDMARDs in TAK. […] The most recent ACR guidelines, published in 2021, suggest a similar but different approach. […] In conclusion, biological therapies can provide additional benefits to TAK patients, and they are gradually becoming part of the clinical practice.
  • #39 Current Immunosuppressive Treatment for Takayasu Arteritis
    https://www.jstage.jst.go.jp/article/circj/advpub/0/advpub_CJ-23-0780/_html/-char/en
    Several studies have reported various benefits of TNFi in TAK patients, including safety, clinical and radiological improvements, and reduction in GC dose. […] The JAK/signal transducer and activator of transcription (JAK/STAT) signaling pathway is critical in TAK pathogenesis. […] Several clinical trials have demonstrated their efficacy and safety in TAK. […] Ustekinumab is a monoclonal antibody against p40 that is used in Japan for patients with psoriasis vulgaris, Crohns disease, or ulcerative colitis. […] Although a few case studies have reported its effectiveness in patients with refractory TAK, further evidence is required. […] Rituximab therapy for Takayasu arteritis: A seven patients experience and a review of the literature. […] In the 2017 JCS guidelines, use of TCZ in TAK patients was a Class I recommendation and TNF-inhibitor use was a Class IIa recommendation; JAK inhibitors, ustekinumab, abatacept, secukinumab, and rituximab were not described. […] Nonetheless, these drugs may be effective and might be recommended in the future provided that supporting evidence emerges.
  • #40 Takayasu Arteritis Treatment & Management: Approach Considerations, Surgical Therapy, Follow-Up
    https://emedicine.medscape.com/article/332378-treatment
    Cytotoxic agents are used for patients whose disease is steroid resistant or relapsing. These agents are usually continued for at least 1 year after remission and are then tapered to discontinuation. […] Bypass graft surgery is the procedure with the best long-term patency rate. […] Strict management of traditional cardiovascular risk factors such as dyslipidemia, hypertension, and lifestyle factors that increase the risk of cardiovascular disease is mandatory to minimize secondary cardiovascular complications. […] Hypertension is treated with antihypertensive agents, and aggressive therapy is necessary to prevent complications.
  • #41 Takayasu Arteritis or Pulseless Disease – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/takayasu-arteritis
    It is crucial to ensure no further narrowing of blood vessels occurs. Therefore risk factors such as high cholesterol and high blood pressure need to be controlled. Drugs to lower blood pressure are often recommended. Statins are commonly prescribed to lower cholesterol and reduce the risk of heart disease. In TA statins and certain blood pressure drugs may also have a beneficial effect on arterial function, even when cholesterol levels are normal. Low-dose aspirin may be prescribed in some cases to reduce the risk of a blood clot developing. If high doses of steroids are required drugs to prevent osteoporosis (weakening of the bones) may also be prescribed. […] Surgical intervention should not be undertaken lightly and is only indicated for the most severe cases of TA. For optimal results surgery should be carried out when the disease is in remission and decisions will involve a team of specialist doctors including the rheumatologist, vascular surgeons and radiologists. Procedures that may be carried out include:
  • #42 Takayasu Arteritis
    https://rheumatology.org/patients/takayasu-arteritis
    TAK needs treatment to prevent damage to arteries. Yet, the narrowing that has already occurred often does not improve. Steroids are an important part of treatment for TAK in addition to other medications that will allow the steroids to be tapered or discontinued. These medicines include methotrexate, azathioprine, drugs that block tumor necrosis factor (such as adalimumab or infliximab), and other biologics like tocilizumab. Low-dose aspirin may be necessary for some patients with narrowing blood vessels to the brain. Surgery may be necessary to open narrowed arteries. Therapy for TAK also includes treating high blood pressure and high cholesterol if these problems are present. […] TAK is a chronic disease and will need long-term treatment. Some patients have no symptoms or only mild symptoms, but others are disabled or need surgery. Because TAK can cause heart problems, high blood pressure and stroke, patients with TAK should talk to their doctor about ways to lower the risk of these serious problems.
  • #43 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340
    If your arteries become severely narrowed or blocked, you may need surgery to open or bypass these arteries to allow an uninterrupted flow of blood. Often this helps to improve certain symptoms, such as high blood pressure and chest pain. In some cases, though, the narrowing or blockage may happen again, requiring a second procedure. […] Surgical options are best performed when inflammation of the arteries has been reduced. They include: […] Bypass surgery. In this procedure, an artery or a vein is removed from a different part of your body and attached to the blocked artery, providing a bypass for blood to flow through. Bypass surgery is usually performed when the narrowing of the arteries is irreversible or when there is significant obstruction to blood flow. […] Blood vessel widening (percutaneous angioplasty). This procedure may be indicated if the arteries are severely blocked. During percutaneous angioplasty, a tiny balloon is threaded through a blood vessel and into the affected artery. Once in place, the balloon is expanded to widen the blocked area, then it’s deflated and removed. […] Aortic valve surgery. Surgical repair or replacement of the aortic valve may be needed if the valve is leaking significantly.
  • #44 Takayasu’s Arteritis: Causes, Symptoms and Treatment
    https://my.clevelandclinic.org/health/diseases/7097-takayasus-arteritis
    Some people may also take tumor-necrosis factor (TNF) inhibitors to fight inflammation. […] In some instances, narrowing of arteries to your kidney may cause hypertension (high blood pressure). Your provider can stretch narrow vessel openings with a balloon (angioplasty) or do a bypass operation to restore normal flow to your kidney. […] Some people with Takayasus arteritis may need an aortic valve replacement. […] Overall, about 25% of people who have Takayasus arteritis won’t be able to manage their disease completely without continued use of medication. […] Medicines for treating Takayasus arteritis can have serious side effects, but other medicines can help with these side effects. […] Corticosteroids work within hours after you receive your first dose. […] Although there is no cure for Takayasus arteritis, it is a treatable disease. Most people who have it improve with treatment. […] Takayasus arteritis is a chronic disease that progresses slowly. You’ll need long-term treatment, most likely with medicine.
  • #45 Treatment of Takayasu arteritis – UpToDate
    https://www.uptodate.com/contents/treatment-of-takayasu-arteritis
    Treatment of Takayasu arteritis […] The treatment of TAK will be reviewed here. […] The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a „steroid-sparing” benefit and longer-term disease control. […] Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. […] It is recommended that patients with TAK be evaluated and managed by centers and physicians with experience treating this rare disease.
  • #46 Takayasu Arteritis – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/vasculitis/takayasu-arteritis
    Vascular intervention, usually a bypass procedure, may be needed to reestablish blood flow to ischemic tissues if pharmacotherapy is ineffective. Indications include the following: Aortic insufficiency, Coronary artery stenosis causing symptomatic coronary artery disease or ischemic cardiomyopathy, Dissection of enlarged aortic aneurysm, Severe hypertension secondary to renal artery stenosis that is refractory to medical management, Limb ischemia that interferes with daily activities, Brain ischemia, Coarctation of the aorta, Inability to measure blood pressure accurately (in any limb). […] Bypass grafting preferably with an autologous graft has the best patency rates. […] For aortic regurgitation, valvular surgery with aortic root replacement may be necessary.
  • #47 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    There are conflicting results with rituximab therapy in refractory TAK. Therefore, this limited experience of rituximab do not support a role for rituximab as the first or second line biologic therapy in TAK patients. There are case reports showing the efficacy of ustekinumab and anakinra in refractory TAK patients. A phase 3 multicenter randomized control trial comparing upadacitinib vs placebo in TAK is currently active. […] Except in emergency conditions, open or endovascular vascular interventions should be considered as the last option in case of medical treatment failure to prevent ischemic arterial symptoms or injury in TAK. As a general rule, such interventions should be avoided during the active phase of the disease and should be attempted only after suppression of vascular inflammation by appropriate IS treatment. According to data from case series, the main indications for surgery are as follows: refractory hypertension related to renal artery stenosis, aortic disease including coarctation and ascending aortic dilatation aortic valve regurgitation, ischemic heart disease, supra-aortic disease with cerebral ischemicemia, mesenteric ischemicemia, severe limb-threatening claudication, and aneurysm repair. In a recent meta-analysis comparing balloon angioplasty and stenting outcomes, there were no significant differences in the incidence of restenosis and other complications overall, but restenosis risk in stenting was significantly higher than that in balloon angioplasty in renal stenosis.
  • #48 Takayasu Disease and Stroke
    https://practicalneurology.com/articles/2020-jan/takayasu-disease-and-stroke
    Surgical interventions are often performed during the late stages of TA. Options include both open and endovascular approaches. Both are complex because of the inflammatory nature of the disease and the length and location of the arterial pathology. There are no trials directly comparing open surgery versus endovascular treatment. In general, the indications for revascularization include severe hypertension from renal or aortic stenosis, cerebral or limb ischemia, or an aneurysm of the aorta or another major artery. Another reason for surgery is the presence of occlusive vascular lesions that persist despite medical treatment. In general, elective operations should be done during a remission to promote improved recovery. Emergency operations are necessary for aneurysm formation, aortic dissection, or critical ischemia including stroke. The choice of surgical procedure depends on the anatomic location and timing of the operation. Emergency surgery during an active disease phase carries a high risk of complications.
  • #49 Takayasu’s arteritis – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/diagnosis-treatment/drc-20351340
    If your arteries become severely narrowed or blocked, you may need surgery to open or bypass these arteries to allow an uninterrupted flow of blood. Often this helps to improve certain symptoms, such as high blood pressure and chest pain. In some cases, though, the narrowing or blockage may happen again, requiring a second procedure. […] Surgical options are best performed when inflammation of the arteries has been reduced. They include: […] Bypass surgery. In this procedure, an artery or a vein is removed from a different part of your body and attached to the blocked artery, providing a bypass for blood to flow through. Bypass surgery is usually performed when the narrowing of the arteries is irreversible or when there is significant obstruction to blood flow. […] Blood vessel widening (percutaneous angioplasty). This procedure may be indicated if the arteries are severely blocked. During percutaneous angioplasty, a tiny balloon is threaded through a blood vessel and into the affected artery. Once in place, the balloon is expanded to widen the blocked area, then it’s deflated and removed. […] Aortic valve surgery. Surgical repair or replacement of the aortic valve may be needed if the valve is leaking significantly.
  • #50 Takayasu’s arteritis | Altru Health System
    https://www.altru.org/health-library/conditions/takayasus-arteritis
    If your arteries become severely narrowed or blocked, you may need surgery to open or bypass these arteries to allow an uninterrupted flow of blood. Often this helps to improve certain symptoms, such as high blood pressure and chest pain. In some cases, though, the narrowing or blockage may happen again, requiring a second procedure. […] Also, if you develop large aneurysms, surgery may be needed to prevent them from rupturing. […] Surgical options are best performed when inflammation of the arteries has been reduced. They include: […] Bypass surgery. In this procedure, an artery or a vein is removed from a different part of your body and attached to the blocked artery, providing a bypass for blood to flow through. Bypass surgery is usually performed when the narrowing of the arteries is irreversible or when there is significant obstruction to blood flow.
  • #51
    https://link.springer.com/article/10.1007/s40674-024-00216-4
    A significant proportion of patients with Takayasu arteritis (TAK) need vascular surgical interventions to correct enlarging aneurysms and severe stenotic or occluded arteries with tissue ischemia. […] Vascular interventions are a crucial part of TAK management and comprise different modalities of open surgery graft bypass and endovascular interventions. Vascular interventions are indicated for hemodynamically significant stenosis or occlusions leading to ischemia and to repair dissecting or increasing aneurysms in TAK patients. Ideally, TAK patients should undergo vascular interventions during the remission state of the disease and still under therapy with glucocorticoids and immunosuppressive agents. […] Vascular interventions are an important part of TAK management and should be indicated whenever medical therapy fails to treat vascular lesions with significant ischemia. The rate of complications is lower in patients with stable disease and still under therapy.
  • #52 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    There are conflicting results with rituximab therapy in refractory TAK. Therefore, this limited experience of rituximab do not support a role for rituximab as the first or second line biologic therapy in TAK patients. There are case reports showing the efficacy of ustekinumab and anakinra in refractory TAK patients. A phase 3 multicenter randomized control trial comparing upadacitinib vs placebo in TAK is currently active. […] Except in emergency conditions, open or endovascular vascular interventions should be considered as the last option in case of medical treatment failure to prevent ischemic arterial symptoms or injury in TAK. As a general rule, such interventions should be avoided during the active phase of the disease and should be attempted only after suppression of vascular inflammation by appropriate IS treatment. According to data from case series, the main indications for surgery are as follows: refractory hypertension related to renal artery stenosis, aortic disease including coarctation and ascending aortic dilatation aortic valve regurgitation, ischemic heart disease, supra-aortic disease with cerebral ischemicemia, mesenteric ischemicemia, severe limb-threatening claudication, and aneurysm repair. In a recent meta-analysis comparing balloon angioplasty and stenting outcomes, there were no significant differences in the incidence of restenosis and other complications overall, but restenosis risk in stenting was significantly higher than that in balloon angioplasty in renal stenosis.
  • #53 Outcomes of different treatments on Takayasu’s arteritis – Xiao – Journal of Thoracic Disease
    https://jtd.amegroups.org/article/view/8936/html
    Cutting balloon angioplasty can be considered as a safe, effective, and less-invasive alternative for non-diffuse SAA lesions, especially in young TA patients. However, bypass surgery has better primary patency rate than endovascular treatment. […] Surgical bypass is considered as the efficient therapy for TA patients with symptomatic, irreversible SAA lesions; however, the early and late complications after bypass surgery, such as postoperative bleeding, cerebrovascular accidents, anastomotic aneurysm were the primary causes of mortality. […] The primary patency between the cutting balloon angioplasty and conventional balloon angioplasty was not significantly altered. […] The Cutting balloon was designed to alleviate vascular trauma and thereby reduce neointimal hyperplasia to restore stenotic arteries and improve long-term patency.
  • #54 Treatment of Takayasu arteritis – UpToDate
    https://www.uptodate.com/contents/treatment-of-takayasu-arteritis
    Treatment of Takayasu arteritis […] The treatment of TAK will be reviewed here. […] The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a „steroid-sparing” benefit and longer-term disease control. […] Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. […] It is recommended that patients with TAK be evaluated and managed by centers and physicians with experience treating this rare disease.
  • #55 Treatment of Takayasu arteritis – UpToDate
    https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print
    Treatment of Takayasu arteritis […] The treatment of TAK will be reviewed here. […] The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a „steroid-sparing” benefit and longer-term disease control. […] Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. However, both endovascular intervention and surgical procedures should generally be avoided when the disease is active. […] It is recommended that patients with TAK be evaluated and managed by centers and physicians with experience treating this rare disease.
  • #56 Functional Medicine: Takayasu’s Arteritis Pathophysiology & Care | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/functional-medicine-takayasus-arteritis-pathophysiology-care/
    Managing Takayasus arteritis requires a multidisciplinary approach. Patients often need care from rheumatologists, cardiologists, and vascular surgeons for corticosteroid therapy and blood pressure management. […] Consultation with specialists is vital for effective management. Patients should seek care at competence centers experienced with vasculitis cases and blood pressure. […] Functional medicine focuses on root causes. It looks beyond symptoms. For Takayasus arteritis, it examines the immune system and inflammation in patients. […] Dietary Interventions: Diet plays a crucial role. Anti-inflammatory foods can reduce symptoms. Omega-3 fatty acids, found in fish, are beneficial. Fruits and vegetables also help. Avoiding processed foods is important. […] Stress impacts health significantly. High stress levels can worsen Takayasus arteritis. Techniques like meditation and yoga help manage stress.
  • #57 Takayasu’s Arteritis Symptoms and Treatment – Brigham and Women’s Hospital
    https://www.brighamandwomens.org/medicine/rheumatology-inflammation-immunity/services/takayasus-arteritis?TRILIBIS_EMULATOR_UA=…%3Fcmp%3Dhrgn%3Fcmp%3Dhrgn%2C…%3Fcmp%3Dhrgn%3Fcmp%3Dhrgn%2Caqkljlpwmmk%3Fcmp%3Dhrgn%3Fcmp%3Dhrgn
    TAK is highly treatable but requires strong immune-suppressive medications. The disease is chronic and therefore requires long-term treatment in about 80% of patients. Treatment prevents narrowing from worsening but does not usually improve symptoms that are due to permanent damage that has already occurred. […] Corticosteroids are usually used at high doses at the beginning of treatment and when the disease relapses. It is often continued at very low doses to maintain remission. […] Immunosuppressive drugs such as methotrexate and azathioprine may be used to reduce the need for corticosteroids long-term. […] Biologic immunosuppressive drugs such as infliximab, adalimumab, and tocilizumab are often used if methotrexate and azathioprine are ineffective. […] Some patients require procedures such as surgery or angioplasty and stent placement in order to reduce symptoms that are due to permanent damage.
  • #58 Takayasu Arteritis – Vasculitis Foundation
    https://vasculitisfoundation.org/education/vasculitis-types/takayasu-arteritis/
    Takayasu arteritis (TAK) is usually treated with glucocorticoids such as prednisone and other medications that suppress the immune system, to control inflammation. […] The goals of treatment include controlling inflammation of the blood vessels and preventing further damage. For some patients, the disease may go into remission and medication can be tapered or stopped. But for others, the disease may need to be controlled with medicine on a long-term basis. In severe cases, surgery may be required to bypass blocked vessels. […] Initial treatment typically consists of glucocorticoids such as prednisone, starting at a high dose and then tapered down to minimize side effects. Other medications that suppress the immune system may be prescribed to achieve remission and reduce the need for prednisone. They include methotrexate, azathioprine, and mycophenolate mofetil.
  • #59 Functional Medicine: Takayasu’s Arteritis Pathophysiology & Care | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/functional-medicine-takayasus-arteritis-pathophysiology-care/
    A personalized plan is essential for managing Takayasus arteritis effectively in patients. […] Patients with Takayasus arteritis can benefit from specific dietary changes. Consuming anti-inflammatory foods helps reduce inflammation. […] Stress management is vital for individuals with Takayasus arteritis. Chronic stress can worsen symptoms and trigger relapses. […] Regular physical activity is essential but should be approached with caution by patients. […] Frequent monitoring of patients health status is crucial for managing Takayasus arteritis effectively. Regular check-ups help patients track disease progression and the effectiveness of treatments. […] Maintaining a healthy weight supports patients overall well-being and reduces the risk of complications. […] Educating patients about their condition empowers them to take an active role in their care.
  • #60 Takayasu Disease and Stroke
    https://practicalneurology.com/articles/2020-jan/takayasu-disease-and-stroke
    No data are available to guide long-term follow up. As a rule of thumb, clinic visits should be scheduled every 1 to 3 months in the first year after diagnosis and every 3 to 6 months thereafter. Visits should include clinical assessments and ESR and CRP measurements with the proviso that inflammatory markers levels may not reliably indicate TA disease activity, especially late in the disease course when neurologic symptoms occur. A major relapse is defined as new ischemic symptoms or progressive inflammatory changes. Minor worsening includes isolated symptoms consistent with polymyalgia rheumatica (ie, muscle pain and tenderness) and may or may not be associated with increased ESR or CRP levels. Although an increase in inflammatory markers may prompt new vascular imaging, the changes should not, on their own, result in new treatment unless new ischemic symptoms are also present. Regardless of the medical regimen, sequential imaging of the aorta for evidence of disease progression is required.
  • #61 Functional Medicine: Takayasu’s Arteritis Pathophysiology & Care | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/functional-medicine-takayasus-arteritis-pathophysiology-care/
    Functional medicine offers a fresh perspective on treating Takayasus arteritis, a rare inflammatory disease affecting the arteries with vascular inflammation and arterial lesions. Unlike conventional methods, functional medicine digs deep to find the root causes of this condition, focusing on pathogenesis, biological treatments, and therapeutic approaches. Its not just about managing symptoms but understanding pathogenesis, diagnosis, and prevention in the first place. […] Holistic Approach: Functional medicine offers a comprehensive approach to managing Takayasus arteritis by addressing underlying causes, pathogenesis, arterial lesions, and promoting overall well-being. […] Personalized Care: Tailor treatments to individual needs, focusing on diet, lifestyle changes, corticosteroid therapy, and stress management to complement conventional therapies.
  • #62 Functional Medicine: Takayasu’s Arteritis Pathophysiology & Care | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/functional-medicine-takayasus-arteritis-pathophysiology-care/
    Integrative Therapies: Combining conventional treatments with novel therapies, corticosteroid therapy, and functional medicine can improve patient quality of life and reduce symptoms. […] Self-Care Importance: Encourage patients to adopt self-care practices such as regular exercise, balanced nutrition, and mindfulness to support treatment, prevent flare-ups, and manage disease activity during corticosteroid therapy. […] Therapeutic management of Takayasus arteritis in patients often involves corticosteroid therapy and disease-modifying anti-rheumatic drugs (DMARDs). These drugs are the cornerstone of medical therapy. They help reduce inflammation and prevent further damage to blood vessels in tak patients through corticosteroid therapy, reducing risk and pressure. […] Inducing remission is a primary goal in managing Takayasus arteritis in patients with corticosteroid therapy and monitoring blood pressure.
  • #63 Functional Medicine: Takayasu’s Arteritis Pathophysiology & Care | Maggie Yu MD, IFMCP
    https://drmaggieyu.com/blog/functional-medicine-takayasus-arteritis-pathophysiology-care/
    Managing Takayasus arteritis requires a multidisciplinary approach. Patients often need care from rheumatologists, cardiologists, and vascular surgeons for corticosteroid therapy and blood pressure management. […] Consultation with specialists is vital for effective management. Patients should seek care at competence centers experienced with vasculitis cases and blood pressure. […] Functional medicine focuses on root causes. It looks beyond symptoms. For Takayasus arteritis, it examines the immune system and inflammation in patients. […] Dietary Interventions: Diet plays a crucial role. Anti-inflammatory foods can reduce symptoms. Omega-3 fatty acids, found in fish, are beneficial. Fruits and vegetables also help. Avoiding processed foods is important. […] Stress impacts health significantly. High stress levels can worsen Takayasus arteritis. Techniques like meditation and yoga help manage stress.
  • #64 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    Glucocorticoids are the mainstay of treatment for remission induction in TAK. The initial dose of prednisolone is 1 mg/kg/day (maximum 60 mg/day). The initial high dose should be maintained for a month and tapered gradually. According to the 2018 update of the EULAR recommendations for the management of LVV it was recommended that in patients who have reached 15-20 mg daily GC dose after 2-3 months, GCs should be decreased slowly targeting 10 mg/day at the end of one year. However, 10 mg/day doses of GCs in long-term remission are possibly too high compared with the recommendations in other disorders such as rheumatoid arthritis (usually 5 mg/day) and should be individually assessed in each patient according to the risk of GC-associated complications. Recent ACR guideline conditionally recommend tapering off glucocorticoids over long-term treatment with low-dose glucocorticoids for remission maintenance in TAK patients achieved remission while receiving GCs for 6-12 months. Both EULAR and ACR recommend the use of non-biologic disease-modifying agents in addition to glucocorticoids in all patients with TAK.
  • #65 Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasu’s arteritis
    https://www.degruyter.com/document/doi/10.1515/rir-2024-0001/html?lang=en
    European League Against Rheumatism (EULAR) endorsed the recommendations for the management of large vessel vasculitis in 2018 and American College of Rheumatology (ACR) developed the guideline for the management of large vessel vasculitis in 2021. […] The basic treatment for remission induction is high dose glucocorticoid combined with traditional immunosuppressive agents, while for remission maintenance, glucocorticoid should be tapered to the minimum maintenance dose or stopped, but immunosuppressive agents should be combined to maintain the disease in a persistent stable state. […] Therefore, the level of standardization of treatment should be improved. […] This guideline also takes the published international guidelines on TAK as important references. […] It also provides measures to deal with the above 4 problems and challenges in the management of TAK based on the best evidence from the literature.
  • #66 Takayasu Arteritis – Vasculitis Foundation
    https://vasculitisfoundation.org/education/vasculitis-types/takayasu-arteritis/
    Those with severe disease who don’t respond to traditional treatments may be prescribed a biologic medication. Biologic drugs are complex proteins derived from living organisms. They target certain parts of the immune system to control inflammation. Among these, tocilizumab and infliximab have been used in TAK. […] In cases where arteries have become severely narrowed or blocked or to repair aneurysms, vascular surgery may be required to improve blood flow. […] In 2021 the American College of Rheumatology (ACR) published guidelines for the management of certain vasculitides, that were also endorsed by the Vasculitis Foundation (VF).
  • #67 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    Glucocorticoids are the mainstay of treatment for remission induction in TAK. The initial dose of prednisolone is 1 mg/kg/day (maximum 60 mg/day). The initial high dose should be maintained for a month and tapered gradually. According to the 2018 update of the EULAR recommendations for the management of LVV it was recommended that in patients who have reached 15-20 mg daily GC dose after 2-3 months, GCs should be decreased slowly targeting 10 mg/day at the end of one year. However, 10 mg/day doses of GCs in long-term remission are possibly too high compared with the recommendations in other disorders such as rheumatoid arthritis (usually 5 mg/day) and should be individually assessed in each patient according to the risk of GC-associated complications. Recent ACR guideline conditionally recommend tapering off glucocorticoids over long-term treatment with low-dose glucocorticoids for remission maintenance in TAK patients achieved remission while receiving GCs for 6-12 months. Both EULAR and ACR recommend the use of non-biologic disease-modifying agents in addition to glucocorticoids in all patients with TAK.
  • #68 Current Immunosuppressive Treatment for Takayasu Arteritis
    https://www.jstage.jst.go.jp/article/circj/advpub/0/advpub_CJ-23-0780/_html/-char/en
    Current Immunosuppressive Treatment for Takayasu Arteritis […] The principal treatment has been glucocorticoids, but the recent emergence of biological disease-modifying anti-rheumatic drugs (bDMARDs), represented by tocilizumab (TCZ), has significantly changed the treatment landscape. […] In the 2017 Japanese Circulation Society (JCS) guidelines for the management of vasculitis syndrome, TCZ is a Class I treatment recommendation for TAK. […] In the 2021 American College of Rheumatology (ACR)/Vasculitis Foundation guidelines, TCZ may be considered for patients who show an inadequate response to other immunosuppressive therapies such as methotrexate, tumor necrosis factor inhibitors (TNFi), and azathioprine. […] Our aim is to help cardiologists and cardiovascular surgeons, who are occasionally involved in treating TAK patients, to better understand the disease and treatment.
  • #69
    https://link.springer.com/article/10.1007/s11926-020-00964-x
    Guidelines for the management of large vessel vasculitides have been recently updated by several scientific societies. We have evaluated the current recommendations for treatment of giant cell arteritis (GCA) and Takayasu arteritis (TA) and addressed potential future therapeutic strategies. […] While glucocorticoids (GCs) remain the gold standard for induction of remission, many patients relapse and acquire high cumulative GC exposure. Thus, GC-sparing therapies such as methotrexate are recommended for selected patients with GCA and all patients with TA. Recent high-quality evidence shows that tocilizumab is an effective GC-sparing agent in GCA. Non-biologic and biologic immunomodulators also appear to have GC-sparing properties in TA. […] Tocilizumab is now considered to be part of the standard treatment for GCA, particularly with relapsing disease, but questions on its use such as length of treatment and monitoring of disease activity remain open. High-quality evidence to guide treatment of TA is still lacking.
  • #70 UPDATE IN TAKAYASU’S ARTERITIS – Rheumatology Quarterly
    https://qrheumatol.com/articles/update-in-takayasus-arteritis/doi/qrheumatol.galenos.2023.36844
    Several case series and observational studies reported the efficacy and safety of TNF inhibitors (TNFi) and TCZ in TAK. Two large retrospective comparison studies found similar clinical response rates and radiologic progression between TNFi or TCZ. A recent meta-analysis also confirmed similar clinical response, angiographic stabilization, and adverse events with TNFi or TCZ. In a head-to-head retrospective comparison, the drug survival rate of TNF was significantly higher than that of TCZ. A retrospective, longitudinal follow-up cohort from Norway reported less angiographic progression at 2 years in patients with TAK receiving TNFi than in those receiving conventional immunosuppressive. According to EULAR recommendations, TCZ or TNFi can be equally considered in refractory patients. However, recent ACR guidelines recommend adding a TNFi over TCZ in refractory patients. A very recent open prospective study compared secukinumab and TNFi in patients with refractory TAK secukinumab and TNFi were found to be comparable regarding response rates at 3 and 6 months.
  • #71 Congress of Clinical Rheumatology Annual Meeting
    https://www.healio.com/news/rheumatology/20230909/we-should-be-thinking-cure-for-giant-cell-takayasu-arteritis-as-trial-data-grow
    Increasingly, clinicians will initiate tocilizumab (Actemra, Genentech) or methotrexate at the time of diagnosis, particularly if the patient demonstrates severe disease. […] The goal, ultimately, is to get the patient off of steroids, according to Merkel. […] Beyond tocilizumab, encouraging data have also been reported for abatacept (Orencia, Bristol Myers Squibb), which has one randomized, controlled trial demonstrating efficacy to its credit. […] Turning to Takayasu arteritis, Merkel acknowledged that there is much less data from which to draw conclusions. […] That said, a wide array of drugs have been used to varying degrees of effectiveness in the Takayasu setting, including methotrexate, TNF inhibitors, tocilizumab, cyclophosphamide, rituximab (Rituxan, Genentech), secukinumab (Cosentyx, Novartis) and tacrolimus, among others, he added.
  • #72 Takayasu Arteritis
    https://rheumatology.org/patients/takayasu-arteritis
    TAK needs treatment to prevent damage to arteries. Yet, the narrowing that has already occurred often does not improve. Steroids are an important part of treatment for TAK in addition to other medications that will allow the steroids to be tapered or discontinued. These medicines include methotrexate, azathioprine, drugs that block tumor necrosis factor (such as adalimumab or infliximab), and other biologics like tocilizumab. Low-dose aspirin may be necessary for some patients with narrowing blood vessels to the brain. Surgery may be necessary to open narrowed arteries. Therapy for TAK also includes treating high blood pressure and high cholesterol if these problems are present. […] TAK is a chronic disease and will need long-term treatment. Some patients have no symptoms or only mild symptoms, but others are disabled or need surgery. Because TAK can cause heart problems, high blood pressure and stroke, patients with TAK should talk to their doctor about ways to lower the risk of these serious problems.
  • #73 Takayasu’s Arteritis: Symptoms, Causes, Diagnosis & Treatment
    https://www.webmd.com/heart-disease/takayasus-arteritis
    There is no cure for Takayasu’s arteritis, but treatment can help manage symptoms of the condition. Treatment focuses on using medications to lower inflammation as well as lower blood pressure, preventing further damage to the affected arteries and tissues. […] Although statistics vary, one study found that about 80% of people with Takayasu’s arteritis can manage their condition with proper treatment. Some even go into remission. This doesn’t mean theyre cured, but the disease isnt active, and they might be able to reduce or even stop their medications. […] Corticosteroids, also called systemic glucocorticoids or just steroids, are usually the first treatment for Takayasu’s arteritis. Steroids work within hours after the first dose is given. The most common drug in this class is prednisone. While this drug is often highly effective, it may be only partially effective for some people.
  • #74 Takayasu’s arteritis – Symptoms, diagnosis and treatment | BMJ Best Practice
    https://bestpractice.bmj.com/topics/en-gb/1064
    Glucocorticoids are initial therapy, with additional use of corticosteroid-sparing immunosuppressants. Biological agents are often used for refractory disease. Surgery may be required for established complications. […] Long-term complications are due mainly to arterial occlusion and related damage, including limb ischaemia and cardiac and neurological manifestations.
  • #75 Takayasu arteritis – Symptoms, diagnosis and treatment | BMJ Best Practice US
    https://bestpractice.bmj.com/topics/en-us/1064
    Glucocorticoids are initial therapy, with additional use of corticosteroid-sparing immunosuppressants. Biologic agents are often used for refractory disease. Surgery may be required for established complications. […] Long-term complications are due mainly to arterial occlusion and related damage, including limb ischemia and cardiac and neurologic manifestations.
  • #76 Tocilizumab in treatment-naïve patients with Takayasu arteritis: TOCITAKA French prospective multicenter open-labeled trial | Arthritis Research & Therapy | Full Text
    https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-020-02311-y
    Tocilizumab combined to steroids in treatment-nave patients with TAK is highly effective, with 85% remission rate and 54% of steroid discontinuation after 6 months of therapy, and despite the high remission rates obtained using tocilizumab, relapse rates were of 45% after discontinuation. […] Our study is the first to show that a high response rate can be achieved by adding tocilizumab to conventional steroids and that 36% of patients with TAK will remain without treatment during at least 18 months. […] Further studies among treatment-nave patients with TAK are necessary to determine the best maintenance therapy strategy.
  • #77 Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasu’s arteritis
    https://www.degruyter.com/document/doi/10.1515/rir-2024-0001/html?lang=en
    Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasus arteritis. […] Therefore, early diagnosis, standardized treatment and manage complications are important to alter the nature course of the disease, improve the overall prognosis and quality of life of patients with TAK. […] Like the majority rheumatic diseases, early detection and early treatment of TAK can slow down or halt the disease progression, avoid vital organ ischemia caused by severe vascular stenosis, and thus substantially improve the prognosis. […] Therefore, comprehensive and timely evaluation of disease activity, disease severity and organ damage are essential to make treatment decisions. […] So multidisciplinary management of patients with TAK has been proven to be an effective approach to optimize treatment outcomes.
  • #78 Treatment of Chronic and Relapsing Takayasu’s Arteritis
    https://consultqd.clevelandclinic.org/treatment-of-chronic-and-relapsing-takayasus-arteritis
    Prevention of progression and complications is the primary goal […] Control of disease activity, halting vascular lesion progression and preventing complications are the primary goals of treatment. […] TAK is a chronic or relapsing disease in the majority of patients and continuous or multiple courses of treatment are often needed. […] While TAK may be challenging to diagnose and treat, careful monitoring with the appropriate clinical expertise and tools and thoughtful, risk-sensitive treatment can improve outcomes for patients.