Choroba behçeta
Leczenie

Choroba Behçeta to przewlekłe, zapalne zapalenie naczyń o zróżnicowanym obrazie klinicznym, wymagające indywidualizacji terapii w zależności od zajętych narządów i nasilenia objawów. Leczenie miejscowe, obejmujące kortykosteroidy, pimekrolimus, sukralfat oraz płukanki antyseptyczne, jest stosowane w łagodnych postaciach z zajęciem błon śluzowych i skóry. W przypadku nieskuteczności terapii miejscowej, kolchicyna (lek pierwszego wyboru) oraz apremilast (zatwierdzony przez FDA w 2019 r. do leczenia owrzodzeń jamy ustnej) stanowią kolejne opcje terapeutyczne. W cięższych postaciach stosuje się ogólnoustrojowe kortykosteroidy (np. prednizon) oraz leki immunosupresyjne, takie jak azatiopryna, cyklosporyna i cyklofosfamid, a także inhibitory TNF-α (infliksymab, adalimumab), które wykazują wysoką skuteczność w leczeniu opornych objawów, zwłaszcza ocznych i neurologicznych. Terapia powinna być prowadzona multidyscyplinarnie, z uwzględnieniem specyfiki zajętych narządów, np. agresywne leczenie zapalenia błony naczyniowej oka z zastosowaniem azatiopryny i inhibitorów TNF-α.

Leczenie choroby Behçeta

Choroba Behçeta to rzadkie, przewlekłe zaburzenie zapalne naczyń krwionośnych, które może dotyczyć różnych narządów i układów organizmu. Nie istnieje obecnie lekarstwo, które mogłoby całkowicie wyleczyć chorobę Behçeta, jednak dostępne są skuteczne metody leczenia, które mogą kontrolować objawy i zapobiegać poważnym powikłaniom12. Głównym celem leczenia jest szybkie tłumienie zaostrzeń zapalnych, zapobieganie nawrotom oraz uniknięcie nieodwracalnych uszkodzeń narządów34.

Podejście terapeutyczne w chorobie Behçeta zależy od zajętych narządów, nasilenia objawów, częstości nawrotów, wieku i płci pacjenta5. Terapia powinna być zindywidualizowana i prowadzona w sposób multidyscyplinarny, w zależności od zajętych narządów6. U niektórych pacjentów z łagodną postacią choroby leki mogą być potrzebne jedynie podczas zaostrzeń, natomiast w okresach remisji nie jest wymagane leczenie farmakologiczne7.

Leczenie zmian śluzówkowo-skórnych

W przypadku łagodnej postaci choroby Behçeta z zajęciem błon śluzowych i skóry stosuje się leczenie miejscowe8:

  • Kremy, żele i maści kortykosteroidowe – aplikowane na owrzodzenia skórne i okolice narządów płciowych9
  • Płukanki do jamy ustnej – zawierające kortykosteroidy lub inne substancje zmniejszające ból owrzodzeń jamy ustnej10
  • Krople do oczu – zawierające kortykosteroidy lub inne leki przeciwzapalne, pomocne w przypadku łagodnego zapalenia i podrażnienia oczu11
  • Pimekrolimus – alternatywa dla miejscowych steroidów12
  • Sukralfat – stosowany miejscowo jako substancja ochronna13
  • Płyny antyseptyczne do płukania jamy ustnej (np. chlorheksydyna)14

Jeśli leczenie miejscowe nie przynosi oczekiwanych efektów, często stosuje się doustnie kolchicynę (Colcrys, Mitigare), która jest skuteczna w leczeniu nawracających owrzodzeń jamy ustnej i narządów płciowych, a także może łagodzić objawy zapalenia stawów1516. Kolchicyna jest zwykle stosowana jako lek pierwszego wyboru w leczeniu objawów śluzówkowo-skórnych i stawowych17.

W 2019 roku FDA zatwierdziło apremilast (Otezla) do leczenia owrzodzeń jamy ustnej w przebiegu choroby Behçeta18. Jest to pierwszy i jedyny lek zatwierdzony przez FDA do leczenia owrzodzeń jamy ustnej związanych z chorobą Behçeta19. Działania niepożądane apremilastu mogą obejmować utratę masy ciała i depresję20.

Leczenie ciężkiej postaci choroby

W przypadku cięższej postaci choroby Behçeta może być konieczne stosowanie leków ogólnoustrojowych w celu zmniejszenia uszkodzeń powodowanych przez chorobę zarówno w okresach zaostrzeń, jak i między nimi21:

  • Kortykosteroidy ogólnoustrojowe – stosowane do kontrolowania zapalenia. Prednizon (Rayos) łagodzi stan zapalny wywołany chorobą Behçeta. Kortykosteroidy są często przepisywane wraz z innym lekiem immunosupresyjnym. Działania niepożądane kortykosteroidów obejmują przyrost masy ciała, zgagę, nadciśnienie tętnicze i osteoporozę22.
  • Leki immunosupresyjne – zapobiegające atakowaniu zdrowych tkanek przez układ odpornościowy, takie jak azatiopryna (Azasan, Imuran), cyklosporyna (Gengraf, Neoral) i cyklofosfamid (Cytoxan). Leki te mogą zwiększać ryzyko infekcji i wpływać na wątrobę i nerki oraz powodować małopłytkowość i nadciśnienie23.
  • Leki modulujące odpowiedź immunologicznąinterferon alfa-2b (Intron A) może być stosowany samodzielnie lub z innymi lekami w celu kontrolowania owrzodzeń skórnych, bólu stawów i podrażnienia oczu24.
  • Inhibitory TNF-alfa – leki blokujące czynnik martwicy nowotworów, takie jak infliksymab (Remicade) i adalimumab (Humira), są stosowane w leczeniu cięższych objawów choroby Behçeta, szczególnie u osób z objawami opornymi na standardowe leczenie. Działania niepożądane mogą obejmować bóle głowy, wysypkę skórną i zwiększone ryzyko zakażeń25.

Leczenie zaangażowania poszczególnych narządów

Podejście terapeutyczne różni się w zależności od zajętych narządów i układów26:

Zajęcie oczne

Zapalenie błony naczyniowej oka wymaga szybkiego i agresywnego leczenia. Azatiopryna jest powszechnie akceptowana jako lek pierwszego wyboru27. W przypadku ciężkiej choroby oczu (znaczny spadek ostrości wzroku, zapalenie naczyń siatkówki lub zajęcie plamki żółtej) można stosować cyklosporynę lub infliksymab w połączeniu z azatiopryną i kortykosteroidami28.

Panel ekspertów zalecił rozważenie infliksymabu i adalimumabu jako leków immunomodulujących pierwszego wyboru w leczeniu objawów ocznych29. Interferon alfa, stosowany samodzielnie lub w połączeniu z kortykosteroidami, może być drugą opcją w ciężkiej ocznej postaci choroby Behçeta30.

Zajęcie przewodu pokarmowego

W przypadku zmian w przewodzie pokarmowym, alternatywy leczenia oparte na opinii ekspertów są następujące31:

  • Pochodne kwasu 5-aminosalicylowego (np. sulfasalazyna lub mesalazyna)
  • Ogólnoustrojowe kortykosteroidy
  • Azatiopryna
  • Antagoniści czynnika martwicy nowotworów (TNF-α)
  • Talidomid

Leczenie łagodniejszych przypadków powinno być początkowo prowadzone za pomocą pochodnych kwasu 5-aminosalicylowego, natomiast azatioprynę należy rozważyć w przypadkach nieodpowiadających na leczenie lub cięższych32.

Zajęcie stawów

Zapalenie stawów może reagować na prednizon, miejscowe wstrzyknięcia kortykosteroidów, niesteroidowe leki przeciwzapalne (NLPZ) i kolchicynę. Interferon-alfa, azatiopryna i inhibitory TNF-α mogą być stosowane w rzadkich przypadkach pacjentów z opornymi, przedłużającymi się i upośledzającymi atakami33.

Zajęcie ośrodkowego układu nerwowego

Choroba ośrodkowego układu nerwowego (OUN) jest zwykle leczona ogólnoustrojowymi kortykosteroidami, interferonem-alfa, azatiopryną, cyklofosfamidem, metotreksatem i antagonistami TNF-α34. W przypadkach parenchymalnego zajęcia OUN stosuje się zazwyczaj azatioprynę oraz codzienne dożylne podawanie pulsów kortykosteroidów (do 7 dni)35.

Zajęcie naczyniowe

Leczenie zakrzepowych incydentów naczyniowych obejmuje ogólnoustrojową antykoagulację, oprócz kortykosteroidów, azatiopryny, cyklofosfamidu lub cyklosporyny36. Tętniaki tętnicy płucnej leczy się cyklofosfamidem i kortykosteroidami37.

Inhibitory TNF-α coraz częściej są stosowane i stały się standardowym leczeniem choroby Behçeta, która nie jest odpowiednio kontrolowana przez standardowe schematy immunosupresyjne38. Infliksymab został najbardziej szczegółowo zbadany, ale adalimumab okazał się skuteczny w przypadkach opornych zarówno na konwencjonalną terapię, jak i infliksymab39.

W wieloośrodkowym badaniu dotyczącym stosowania antagonistów TNF-α (głównie infliksymabu i adalimumabu) w leczeniu ciężkiej i/lub opornej na leczenie choroby Behçeta u 124 pacjentów, wskaźniki odpowiedzi klinicznej w przypadku konkretnego zajęcia narządów były następujące40:

  • Narząd wzroku – 96,3%
  • Błony śluzowe i skóra – 88%
  • Stawy – 70%
  • Przewód pokarmowy – 77,8%
  • OUN – 92,3%
  • Układ sercowo-naczyniowy – 66,7%

Nowe kierunki w leczeniu choroby Behçeta

Oprócz wyżej wymienionych metod leczenia, badane są również inne terapie, dla których istnieją dane z opisów przypadków lub małych badań pilotażowych41:

  • Rytuksymab
  • Alemtuzumab
  • Golimumab

W otwartym wieloośrodkowym badaniu porównano skuteczność infliksymabu i adalimumabu jako leczenia pierwszego rzutu u 177 pacjentów z opornym zapaleniem błony naczyniowej oka w przebiegu choroby Behçeta. Po roku terapii obie grupy leczenia wykazały poprawę. Jednak pacjenci otrzymujący adalimumab mieli znacznie lepsze wyniki w niektórych parametrach, w tym poprawę zapalenia w komorze przedniej, poprawę zapalenia ciała szklistego i najlepszą skorygowaną ostrość wzroku42.

Apremilast, doustny inhibitor fosfodiesterazy-4, początkowo zatwierdzony do leczenia łuszczycy i łuszczycowego zapalenia stawów, został zatwierdzony do leczenia owrzodzeń jamy ustnej związanych z chorobą Behçeta w 2019 roku43. W badaniu fazy III pacjenci przyjmujący apremilast mieli znacznie mniej owrzodzeń jamy ustnej niż osoby otrzymujące placebo. Jednak 79% osób przyjmujących apremilast doświadczyło działań niepożądanych, takich jak biegunka, nudności, ból głowy i zakażenie górnych dróg oddechowych44.

Rola inhibitorów interleukiny-17 w chorobie Behçeta nie jest znana. Dwa małe retrospektywne badania sugerują poprawę objawów śluzówkowo-skórnych i stawowych po zastosowaniu sekukinumabu45. Jednakże paradoksalna choroba Behçeta była kojarzona z inhibitorami interleukiny-1746.

Objaw choroby Behçeta Leczenie pierwszego wyboru Leczenie alternatywne
Owrzodzenia jamy ustnej i narządów płciowych Miejscowe kortykosteroidy, kolchicyna Apremilast, azatiopryna, inhibitory TNF-α
Zajęcie oczne Azatiopryna, kortykosteroidy miejscowe i ogólne Cyklosporyna, infliksymab, adalimumab, interferon-α
Zajęcie stawów NLPZ, kolchicyna Prednizon, azatiopryna, inhibitory TNF-α
Zajęcie OUN Kortykosteroidy ogólne, cyklofosfamid Azatiopryna, metotreksat, inhibitory TNF-α
Zajęcie naczyniowe Kortykosteroidy, immunosupresja, antykoagulacja Inhibitory TNF-α, cyklofosfamid
Zajęcie przewodu pokarmowego Pochodne kwasu 5-aminosalicylowego Azatiopryna, inhibitory TNF-α

Zalecenia dotyczące stylu życia

Oprócz leczenia farmakologicznego, ważne są następujące aspekty stylu życia4748:

  • Odpoczynek podczas zaostrzeń choroby
  • Umiarkowana aktywność fizyczna (np. pływanie, spacery) w okresach remisji
  • Modyfikacje stylu życia mające na celu zmniejszenie stresu
  • Sprawdzenie i ewentualne skorygowanie niedoboru witaminy D, która ma właściwości immunomodulujące
  • Regularne wizyty kontrolne w celu monitorowania aktywności choroby i skuteczności leczenia

Rokowanie

Dla większości pacjentów z chorobą Behçeta oczekiwana długość życia jest normalna49. W przypadku osób z ciężkim zapaleniem oczu, postępująca utrata wzroku była wcześniej poważnym powikłaniem, jednak dzięki coraz lepszym metodom leczenia, utrata wzroku jest obecnie rzadka50. Osoby z wyłącznie zajęciem skóry, owrzodzeniami i stawami mają bardzo dobre rokowanie51.

Choroba Behçeta zazwyczaj zaczyna się od objawów śluzówkowo-skórnych, które mają ograniczony wpływ na funkcje życiowych narządów i ogólną jakość życia52. Z czasem u wielu pacjentów objawy stają się łagodniejsze, a aktywność choroby zmniejsza się53.

Leczenie musi być kontynuowane przez dłuższy czas, a zmniejszenie (lub zaprzestanie) podawania leków immunosupresyjnych lub immunomodulujących można rozważyć, z wyjątkiem wyjątkowych przypadków, tylko po co najmniej 2 latach remisji w ramach ciężkiej postaci choroby Behçeta (uszkodzenie okulistyczne, trawienne, neurologiczne, sercowo-naczyniowe i płucne)54.

Podsumowując, choroba Behçeta, choć jest schorzeniem przewlekłym, może być skutecznie kontrolowana za pomocą odpowiedniego leczenia. Kluczową rolę odgrywa wczesna diagnoza i rozpoczęcie terapii, aby zapobiec nieodwracalnym uszkodzeniom narządów. Podejście multidyscyplinarne, uwzględniające współpracę reumatologów, dermatologów, okulistów i innych specjalistów, jest niezbędne do optymalnego leczenia tej złożonej choroby.55

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #2 Behcet’s Disease Recovery & Support | NYU Langone Health
    https://nyulangone.org/conditions/behcets-disease/support
    There is no cure for Behcets disease, but treatments can keep symptoms under control. Therapies are most effective when started early and monitored regularly. […] The goal of treatment with medication is to get you into remission. That can take several months to years. […] Some people, however, need long-term medication to control the symptoms of Behets disease. […] Its important for people with severe symptoms to continue seeing a rheumatologist after treatment is completed. […] If Behets disease does flare up, the experts at NYU Langone can immediately treat you and help alleviate your symptoms.
  • #3 Treatment of Behçet syndrome – UpToDate
    https://www.uptodate.com/contents/treatment-of-behcet-syndrome/print
    Treatment of Behçet syndrome will be reviewed here. […] The goal of treatment is to promptly suppress inflammatory exacerbations and recurrences to prevent irreversible organ damage.
  • #4 Treatment of Behçet’s Disease: An Algorithmic Multidisciplinary Approach
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8115406/
    Behet’s disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. […] Although the number of effective drugs used in the disease’s treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. […] The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. […] For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs.
  • #5 Treatment of Behçet’s Disease: An Algorithmic Multidisciplinary Approach
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8115406/
    In this review, we propose a symptom-based, algorithmic treatment approach. […] Treatment varies depending on involved organ/s, the severity and duration of involvement, the frequency of attacks, gender and patient’s age. At present, no specific recommendations based on gender or age exist for all the manifestations of BD; however, age and early disease can influence the treatment of ocular involvement. […] Nevertheless, the primary goal of treatment should be rapidly to suppress and prevent new inflammatory attacks to avoid irreversible organ damage, especially in the early, active stages of BD. […] In acute and severe attacks of mucocutaneous lesions (e.g., major OU, GU, and/or EN-like lesions), corticosteroids (prednisolone, initial dose 40-60 mg daily for 24 weeks, tapered over the ensuing 4-6 weeks) can be used as an effective treatment.
  • #6 Treatment of Behçet’s Disease: An Algorithmic Multidisciplinary Approach
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8115406/
    Behet’s disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. […] Although the number of effective drugs used in the disease’s treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. […] The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. […] For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs.
  • #7 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #8 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #9 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #10 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #11 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    There’s no cure for Behcet disease. If you have a mild form, your healthcare professional might suggest medicines to manage the pain and swelling of flares. You might not need medicine between flares. For some people, symptoms get better over time. […] For worse symptoms, you might take medicines to manage Behcet disease throughout your body. Plus you might take medicines for flares. […] Medicines to manage symptoms during flares might include: Skin creams, gels and ointments. These products, called topical medicines, are corticosteroid medicines that you put on skin and genital sores. Mouth rinses. Special mouthwashes that have corticosteroids and other agents might reduce the pain of mouth sores. Eye drops. For mild swelling and irritation, eye drops that have corticosteroids or other medicines might help.
  • #12 Behçet disease: Background, Treatment and More
    https://dermnetnz.org/topics/behcet-disease
    Behet disease treatment should be tailored to each individual patient based on clinical manifestations and extent of disease an MDT approach is required. Behet disease typically has a relapsing-remitting course, and treatments should aim to suppress any inflammatory exacerbations and prevent irreversible organ damage, particularly in the initial phase of disease. […] Ocular, vascular, and neurological manifestations are often more serious and require more aggressive treatment. […] Topical treatments include: corticosteroids applied as creams, gels, or mouth rinses, pimecrolimus as an alternative to topical steroids, sucralfate, antibiotics such as tetracyclines, antiseptics chlorhexidine mouthwash, hydroxypropyl cellulose, diclofenac, lidocaine. […] Systemic treatments for mucosal disease include: colchicine, glucocorticoids, apremilast, azathioprine, TNF-alpha inhibitors, ciclosporin, interferon alpha-21 and interferon alpha-2b, thalidomide, methotrexate, roflumilast, secukinumab, ustekinumab.
  • #13 Behçet disease: Background, Treatment and More
    https://dermnetnz.org/topics/behcet-disease
    Behet disease treatment should be tailored to each individual patient based on clinical manifestations and extent of disease an MDT approach is required. Behet disease typically has a relapsing-remitting course, and treatments should aim to suppress any inflammatory exacerbations and prevent irreversible organ damage, particularly in the initial phase of disease. […] Ocular, vascular, and neurological manifestations are often more serious and require more aggressive treatment. […] Topical treatments include: corticosteroids applied as creams, gels, or mouth rinses, pimecrolimus as an alternative to topical steroids, sucralfate, antibiotics such as tetracyclines, antiseptics chlorhexidine mouthwash, hydroxypropyl cellulose, diclofenac, lidocaine. […] Systemic treatments for mucosal disease include: colchicine, glucocorticoids, apremilast, azathioprine, TNF-alpha inhibitors, ciclosporin, interferon alpha-21 and interferon alpha-2b, thalidomide, methotrexate, roflumilast, secukinumab, ustekinumab.
  • #14 Behçet disease: Background, Treatment and More
    https://dermnetnz.org/topics/behcet-disease
    Behet disease treatment should be tailored to each individual patient based on clinical manifestations and extent of disease an MDT approach is required. Behet disease typically has a relapsing-remitting course, and treatments should aim to suppress any inflammatory exacerbations and prevent irreversible organ damage, particularly in the initial phase of disease. […] Ocular, vascular, and neurological manifestations are often more serious and require more aggressive treatment. […] Topical treatments include: corticosteroids applied as creams, gels, or mouth rinses, pimecrolimus as an alternative to topical steroids, sucralfate, antibiotics such as tetracyclines, antiseptics chlorhexidine mouthwash, hydroxypropyl cellulose, diclofenac, lidocaine. […] Systemic treatments for mucosal disease include: colchicine, glucocorticoids, apremilast, azathioprine, TNF-alpha inhibitors, ciclosporin, interferon alpha-21 and interferon alpha-2b, thalidomide, methotrexate, roflumilast, secukinumab, ustekinumab.
  • #15 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    If topical medicines don’t help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling. […] More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression. […] If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe: Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active. Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
  • #16 French recommendations for the management of Behçet’s disease | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01620-4
    The use of anticoagulants in treating vascular involvement is disputed but remains recommended; inflammation of the vascular walls is the main cause of Behets disease-related thrombosis. […] In its serious and/or complicated forms, Behets disease is categorized as a life-long disorder, with waiver of co-pay (care at 100%). […] Therapeutic education is indispensable to optimize the care and compliance of the patient with the therapeutic project. […] Colchicine is the first-line treatment of mucocutaneous and articular manifestations. […] Reduction (or cessation) of immunosuppressant or immunomodulating treatment can be considered, except in exceptional cases, only after at least 2 years of remission within the framework of severe Behets disease (ophthalmological, digestive, neurological, cardiovascular, and pulmonary damage).
  • #17 French recommendations for the management of Behçet’s disease | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01620-4
    Behets disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. […] Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. […] The drug treatments for Behets disease essentially depend on the clinical manifestations and target the abatement of inflammation. […] Colchicine is the first-choice treatment of mucocutaneous and articular involvement. The most serious, ocular, vascular, or neurological involvements require immunomodulating treatment, most often associating systemic corticosteroid therapy with immunosuppressants and biotherapy (anti-TNF), depending on the indication.
  • #18 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    If topical medicines don’t help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling. […] More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression. […] If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe: Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active. Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
  • #19 Treatment of Behcet’s Disease
    https://www.behcets.com/treatment
    Apremilast (Otezla), an oral selective phosphodiesterase 4 (PDE4) inhibitor, is the first and only approved treatment by the US Food and Drug Administration (FDA) for oral ulcers associated with Behcet’s Disease. […] Treatment of Behcet’s Disease typically focuses on reducing discomfort and preventing serious complications. […] Patients can control symptoms of Behcet’s Disease with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications. […] Topical medication is applied directly to the ulcers and skin lesions in order to relieve pain and discomfort. […] TNF inhibitors (anti tumor necrosis factor drugs) are a class of drugs used in the United States for more than 15 years and used worldwide to treat inflammatory conditions.
  • #20 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    If topical medicines don’t help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling. […] More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression. […] If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe: Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active. Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
  • #21 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    If topical medicines don’t help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling. […] More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression. […] If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe: Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active. Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
  • #22 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    If topical medicines don’t help, you might take a medicine by mouth called colchicine (Colcrys, Mitigare, others). This is for mouth and genital sores that come back. Colchicine also might ease joint swelling. […] More recently, the U.S. Food and Drug Administration approved apremilast (Otezla) for the treatment of mouth sores caused by Behcet disease. Side effects may include weight loss and depression. […] If you have more-serious Behcet disease, you may need treatments to lessen damage from the disease between flares. Your healthcare professional might prescribe: Corticosteroids to manage swelling and irritation, called inflammation. Corticosteroids, such as prednisone (Rayos), ease the inflammation caused by Behcet disease. Healthcare professionals often prescribe corticosteroids with another medicine to make the immune system less active. Side effects of corticosteroids include weight gain, heartburn, high blood pressure and bone thinning, called osteoporosis.
  • #23 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    Medicines that make the immune system less active. Medicines that keep the immune system from attacking healthy tissues include azathioprine (Azasan, Imuran), cyclosporine (Gengraf, Neoral, others) and cyclophosphamide (Cytoxan). These medicines can increase the risk of infection. They also can affect the liver and kidneys and cause low blood counts and high blood pressure. […] Medicines that change how the immune system responds. Interferon alfa-2b (Intron A) may be used alone or with other medicines to help manage skin sores, joint pain and eye irritation in people with Behcet disease. Side effects include flu-like symptoms, such as muscle pain and fatigue. […] Medicines that block a substance called tumor necrosis factor can treat some of the symptoms of Behcet disease. People who have worse symptoms or symptoms that resist treatment might take one of these medicines. Medicines include infliximab (Remicade) and adalimumab (Humira). Side effects might include headache, skin rash and a higher risk of infections.
  • #24 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    Medicines that make the immune system less active. Medicines that keep the immune system from attacking healthy tissues include azathioprine (Azasan, Imuran), cyclosporine (Gengraf, Neoral, others) and cyclophosphamide (Cytoxan). These medicines can increase the risk of infection. They also can affect the liver and kidneys and cause low blood counts and high blood pressure. […] Medicines that change how the immune system responds. Interferon alfa-2b (Intron A) may be used alone or with other medicines to help manage skin sores, joint pain and eye irritation in people with Behcet disease. Side effects include flu-like symptoms, such as muscle pain and fatigue. […] Medicines that block a substance called tumor necrosis factor can treat some of the symptoms of Behcet disease. People who have worse symptoms or symptoms that resist treatment might take one of these medicines. Medicines include infliximab (Remicade) and adalimumab (Humira). Side effects might include headache, skin rash and a higher risk of infections.
  • #25 Behcet disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/behcet-disease/diagnosis-treatment/drc-20351331
    Medicines that make the immune system less active. Medicines that keep the immune system from attacking healthy tissues include azathioprine (Azasan, Imuran), cyclosporine (Gengraf, Neoral, others) and cyclophosphamide (Cytoxan). These medicines can increase the risk of infection. They also can affect the liver and kidneys and cause low blood counts and high blood pressure. […] Medicines that change how the immune system responds. Interferon alfa-2b (Intron A) may be used alone or with other medicines to help manage skin sores, joint pain and eye irritation in people with Behcet disease. Side effects include flu-like symptoms, such as muscle pain and fatigue. […] Medicines that block a substance called tumor necrosis factor can treat some of the symptoms of Behcet disease. People who have worse symptoms or symptoms that resist treatment might take one of these medicines. Medicines include infliximab (Remicade) and adalimumab (Humira). Side effects might include headache, skin rash and a higher risk of infections.
  • #26 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    The goals of therapy in Behet disease are to suppress inflammation and reduce the frequency and severity of recurrences. To be effective, treatment must be started early. The sites and extent of involvement and the severity of disease determine the choice of medication. […] The treatment approach depends on the individual patient, severity of disease, and major organ involvement. The European League Against Rheumatism (EULAR) recommendations for the management of Behet disease, which were developed in 2008 and updated in 2018, aid in the management of different aspects of Behet disease. […] As disease activity subsides, taper medications to the lowest dose that effectively controls disease activity. Inpatient care is based on individual organ-system involvement. Individualize the transfer situation for each patient based on the specifics of organ-system involvement.
  • #27 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    For oral and genital ulcerations, topical corticosteroids or sucralfate solution are first-line therapy for mild isolated ulcerations. […] Colchicine has also been used to prevent mucocutaneous relapse. […] For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have demonstrated benefit. […] Apremilast is a second-line treatment for oral ulcers associated with Behet disease. […] A study that compared TNF- inhibitors and apremilast for the treatment of refractory oral ulcers showed that both are equally effective. […] For ocular disease, azathioprine is widely accepted as the initial agent. For severe eye disease (significant drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine or infliximab may be used in combination with azathioprine and corticosteroids. […] An expert panel has recommended considering infliximab and adalimumab as first-line immunomodulatory agents for the treatment of ocular manifestations.
  • #28 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    For oral and genital ulcerations, topical corticosteroids or sucralfate solution are first-line therapy for mild isolated ulcerations. […] Colchicine has also been used to prevent mucocutaneous relapse. […] For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have demonstrated benefit. […] Apremilast is a second-line treatment for oral ulcers associated with Behet disease. […] A study that compared TNF- inhibitors and apremilast for the treatment of refractory oral ulcers showed that both are equally effective. […] For ocular disease, azathioprine is widely accepted as the initial agent. For severe eye disease (significant drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine or infliximab may be used in combination with azathioprine and corticosteroids. […] An expert panel has recommended considering infliximab and adalimumab as first-line immunomodulatory agents for the treatment of ocular manifestations.
  • #29 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    For oral and genital ulcerations, topical corticosteroids or sucralfate solution are first-line therapy for mild isolated ulcerations. […] Colchicine has also been used to prevent mucocutaneous relapse. […] For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have demonstrated benefit. […] Apremilast is a second-line treatment for oral ulcers associated with Behet disease. […] A study that compared TNF- inhibitors and apremilast for the treatment of refractory oral ulcers showed that both are equally effective. […] For ocular disease, azathioprine is widely accepted as the initial agent. For severe eye disease (significant drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine or infliximab may be used in combination with azathioprine and corticosteroids. […] An expert panel has recommended considering infliximab and adalimumab as first-line immunomodulatory agents for the treatment of ocular manifestations.
  • #30 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Interferon-alfa, alone or in combination with corticosteroids, can be a second choice in severe ocular Behet disease. It is used primarily in Europe. A retrospective study of a large case series from Germany reported long-lasting remission and improved visual prognosis in patients with severe ocular Behet disease treated with alpha interferon. […] For gastrointestinal lesions, treatment alternatives based on expert opinion are as follows: Minosalicylate (5-ASA) derivatives (eg, sulfasalazine or mesalamine), systemic corticosteroids, azathioprine, tumor necrosis factor (TNF-) antagonists, thalidomide. […] Arthritis may respond to prednisone, local corticosteroid injections, nonsteroidal anti-inflammatory drugs (NSAIDs), and colchicine. Interferon-alfa, azathioprine, and TNF- blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.
  • #31 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Interferon-alfa, alone or in combination with corticosteroids, can be a second choice in severe ocular Behet disease. It is used primarily in Europe. A retrospective study of a large case series from Germany reported long-lasting remission and improved visual prognosis in patients with severe ocular Behet disease treated with alpha interferon. […] For gastrointestinal lesions, treatment alternatives based on expert opinion are as follows: Minosalicylate (5-ASA) derivatives (eg, sulfasalazine or mesalamine), systemic corticosteroids, azathioprine, tumor necrosis factor (TNF-) antagonists, thalidomide. […] Arthritis may respond to prednisone, local corticosteroid injections, nonsteroidal anti-inflammatory drugs (NSAIDs), and colchicine. Interferon-alfa, azathioprine, and TNF- blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.
  • #32 Treatment of Behçet’s Disease: An Algorithmic Multidisciplinary Approach
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8115406/
    In patients unresponsive to colchicine monotherapy, the addition of benzathine penicillin may be beneficial. […] In patients with severe clinical presentation or poor prognostic factors, anti-TNF- agents or cyclophosphamide can be considered as a first-line therapy. […] In patients with refractory venous thrombosis, anti-TNF-alpha agents, alone or in combination with traditional DMARDs, or interferon-alpha, can be considered. […] The treatment of vascular BD manifestations differs according to the involved district, and to the specific type of event. […] The pharmacological treatment of gastrointestinal involvement varies according to its severity. While milder cases should be initially treated with 5-amino salicylate derivatives (e.g., sulfasalazine, mesalamine), azathioprine should be considered in unresponsive or more severe cases. […] Because of the high incidence of vital organ involvement, regular follow-up and appropriate management of BD is mandatory.
  • #33 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Interferon-alfa, alone or in combination with corticosteroids, can be a second choice in severe ocular Behet disease. It is used primarily in Europe. A retrospective study of a large case series from Germany reported long-lasting remission and improved visual prognosis in patients with severe ocular Behet disease treated with alpha interferon. […] For gastrointestinal lesions, treatment alternatives based on expert opinion are as follows: Minosalicylate (5-ASA) derivatives (eg, sulfasalazine or mesalamine), systemic corticosteroids, azathioprine, tumor necrosis factor (TNF-) antagonists, thalidomide. […] Arthritis may respond to prednisone, local corticosteroid injections, nonsteroidal anti-inflammatory drugs (NSAIDs), and colchicine. Interferon-alfa, azathioprine, and TNF- blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.
  • #34 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, methotrexate, and TNF- antagonists. […] Treatment of major-vessel disease with thrombotic events is with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids. […] TNF- antagonists are increasingly used and have become standard treatment of Behet disease that is inadequately controlled by standard immunosuppressive regimens. […] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. […] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behet disease.
  • #35 Advances in the Treatment of Behcet’s Disease
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8136102/
    Azathioprine or cyclosporine A are traditionally first-line treatment options for the treatment of ocular involvement since their efficacy for protecting visual acuity and preventing uveitis relapses were confirmed in RCTs. […] TNF inhibitors seem to be effective treatment options in all types of refractory vascular involvement of BD. […] According to EULAR 2018 recommendations, GCs and ISs such as azathioprine, cyclophosphamide, or cyclosporine A are recommended for the management of acute deep venous thrombosis (DVT) in BD. […] According to EULAR 2018 recommendations, azathioprine and daily IV GC pulses (up to 7 day) are usually prescribed as first-line treatment of an acute attack of parenchymal involvement. […] Treatment approaches for the management of gastrointestinal disease in BD are based on retrospective observational studies or extrapolations from studies on inflammatory bowel disease since there are no controlled trials.
  • #36 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, methotrexate, and TNF- antagonists. […] Treatment of major-vessel disease with thrombotic events is with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids. […] TNF- antagonists are increasingly used and have become standard treatment of Behet disease that is inadequately controlled by standard immunosuppressive regimens. […] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. […] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behet disease.
  • #37 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, methotrexate, and TNF- antagonists. […] Treatment of major-vessel disease with thrombotic events is with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids. […] TNF- antagonists are increasingly used and have become standard treatment of Behet disease that is inadequately controlled by standard immunosuppressive regimens. […] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. […] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behet disease.
  • #38 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, methotrexate, and TNF- antagonists. […] Treatment of major-vessel disease with thrombotic events is with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids. […] TNF- antagonists are increasingly used and have become standard treatment of Behet disease that is inadequately controlled by standard immunosuppressive regimens. […] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. […] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behet disease.
  • #39 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Central nervous system (CNS) disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide, methotrexate, and TNF- antagonists. […] Treatment of major-vessel disease with thrombotic events is with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids. […] TNF- antagonists are increasingly used and have become standard treatment of Behet disease that is inadequately controlled by standard immunosuppressive regimens. […] Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab. […] Etanercept is the only TNF inhibitor with data from a short-term randomized controlled study demonstrating efficacy in suppressing most of the mucocutaneous manifestations of Behet disease.
  • #40 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    In a multicenter study of the use of TNF- antagonists (mainly infliximab and adalimumab) for treatment of severe and/or refractory Behet’s disease in 124 patients, clinical response rates with particular organ involvement were as follows: Ocular – 96.3%, Mucocutaneous – 88%, Joint – 70%, GI – 77.8%, CNS – 92.3%, Cardiovascular – 66.7%. […] Infliximab was reported to successfully treat a case of Behet disease in pregnancy. […] Other therapies with data from case reports or small pilot studies include the following, among others: Rituximab, Alemtuzumab, Golimumab. […] An open-label multicenter study compared the efficacy of infliximab versus adalimumab as a firstline treatment in 177 patients with refractory uveitis due to Behet disease. After 1 year of therapy, both treatment groups showed improvement. However, the patients receiving adalimumab had significantly better outcomes in some parameters, including improvement in anterior chamber inflammation, improvement in vitritis, and best-corrected visual acuity.
  • #41 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    In a multicenter study of the use of TNF- antagonists (mainly infliximab and adalimumab) for treatment of severe and/or refractory Behet’s disease in 124 patients, clinical response rates with particular organ involvement were as follows: Ocular – 96.3%, Mucocutaneous – 88%, Joint – 70%, GI – 77.8%, CNS – 92.3%, Cardiovascular – 66.7%. […] Infliximab was reported to successfully treat a case of Behet disease in pregnancy. […] Other therapies with data from case reports or small pilot studies include the following, among others: Rituximab, Alemtuzumab, Golimumab. […] An open-label multicenter study compared the efficacy of infliximab versus adalimumab as a firstline treatment in 177 patients with refractory uveitis due to Behet disease. After 1 year of therapy, both treatment groups showed improvement. However, the patients receiving adalimumab had significantly better outcomes in some parameters, including improvement in anterior chamber inflammation, improvement in vitritis, and best-corrected visual acuity.
  • #42 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    In a multicenter study of the use of TNF- antagonists (mainly infliximab and adalimumab) for treatment of severe and/or refractory Behet’s disease in 124 patients, clinical response rates with particular organ involvement were as follows: Ocular – 96.3%, Mucocutaneous – 88%, Joint – 70%, GI – 77.8%, CNS – 92.3%, Cardiovascular – 66.7%. […] Infliximab was reported to successfully treat a case of Behet disease in pregnancy. […] Other therapies with data from case reports or small pilot studies include the following, among others: Rituximab, Alemtuzumab, Golimumab. […] An open-label multicenter study compared the efficacy of infliximab versus adalimumab as a firstline treatment in 177 patients with refractory uveitis due to Behet disease. After 1 year of therapy, both treatment groups showed improvement. However, the patients receiving adalimumab had significantly better outcomes in some parameters, including improvement in anterior chamber inflammation, improvement in vitritis, and best-corrected visual acuity.
  • #43 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Apremilast, an oral phosphodiesterase-4 inhibitor initially approved for the treatment of psoriasis and psoriatic arthritis, was approved for treatment of oral ulcers associated with Behet disease in 2019. […] In a phase III trial, patients taking apremilast had significantly fewer oral ulcers than individuals receiving a placebo. However, 79% of those taking apremilast experienced adverse events, which included diarrhea, nausea, headache, and upper respiratory tract infection. […] The role of antiinterleukin-17 in Behet disease is not known. Two small retrospective studies suggest improvement in mucocutaneous and articular symptoms with secukinumab. […] However, paradoxical Behet disease has been associated with interleukin-17 inhibitors.
  • #44 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Apremilast, an oral phosphodiesterase-4 inhibitor initially approved for the treatment of psoriasis and psoriatic arthritis, was approved for treatment of oral ulcers associated with Behet disease in 2019. […] In a phase III trial, patients taking apremilast had significantly fewer oral ulcers than individuals receiving a placebo. However, 79% of those taking apremilast experienced adverse events, which included diarrhea, nausea, headache, and upper respiratory tract infection. […] The role of antiinterleukin-17 in Behet disease is not known. Two small retrospective studies suggest improvement in mucocutaneous and articular symptoms with secukinumab. […] However, paradoxical Behet disease has been associated with interleukin-17 inhibitors.
  • #45 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Apremilast, an oral phosphodiesterase-4 inhibitor initially approved for the treatment of psoriasis and psoriatic arthritis, was approved for treatment of oral ulcers associated with Behet disease in 2019. […] In a phase III trial, patients taking apremilast had significantly fewer oral ulcers than individuals receiving a placebo. However, 79% of those taking apremilast experienced adverse events, which included diarrhea, nausea, headache, and upper respiratory tract infection. […] The role of antiinterleukin-17 in Behet disease is not known. Two small retrospective studies suggest improvement in mucocutaneous and articular symptoms with secukinumab. […] However, paradoxical Behet disease has been associated with interleukin-17 inhibitors.
  • #46 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    Apremilast, an oral phosphodiesterase-4 inhibitor initially approved for the treatment of psoriasis and psoriatic arthritis, was approved for treatment of oral ulcers associated with Behet disease in 2019. […] In a phase III trial, patients taking apremilast had significantly fewer oral ulcers than individuals receiving a placebo. However, 79% of those taking apremilast experienced adverse events, which included diarrhea, nausea, headache, and upper respiratory tract infection. […] The role of antiinterleukin-17 in Behet disease is not known. Two small retrospective studies suggest improvement in mucocutaneous and articular symptoms with secukinumab. […] However, paradoxical Behet disease has been associated with interleukin-17 inhibitors.
  • #47 Behcet’s Syndrome – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/behcets-disease
    Lifestyle modifications to reduce stress should be considered as many people report that stress exacerbates Behets. It is often worthwhile checking for and correcting vitamin D deficiency, as vitamin D has many immune-modulating effects and is often at surprisingly low levels in people from Northern Europe. […] For the vast majority of people with Behets, a normal life span is expected. For those with severe eye inflammation, progressive loss of vision was previously a major complication. However, with improving treatments, visual loss is now uncommon. People with purely skin/ulcers/joint disease have a very good prognosis. […] Treatment is based on immunosuppression with agents including steroids, azathioprine, and biologic agents.
  • #48 Behcet’s Disease Signs & Symptoms | Rush
    https://www.rush.edu/conditions/behcets-disease
    Behcet’s disease is treatable but not curable. Symptoms may disappear (go into remission) and come back (flare). Your Rush rheumatologist will work with you to develop a personalized plan to reduce your pain, and prevent flares and complications. With effective treatment, flares typically become less frequent. […] Your treatment at Rush might include the following medications: Corticosteroids to reduce pain and inflammation, Immunosuppressive medicine to control the immune system, reduce inflammation and prevent flares, Eye drops for inflammation in the eyes, Topical steroids to treat rashes and other skin symptoms. […] Getting a lot of rest when your symptoms are flaring may help. During periods of remission, moderate exercise, such as swimming or walking, can help prevent flares. […] Behcets disease affects several organ systems. Other specialists may be consulted during your diagnosis, including some of the following: Ophthalmologists to identify and treat complications from eye inflammation, Dermatologists to address mouth or skin lesions, Gynecologists to treat womens genital sores, Urologists to treat mens genital sores, Gastroenterologists to identify and manage digestive problems.
  • #49 Behcet’s Syndrome – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/behcets-disease
    Lifestyle modifications to reduce stress should be considered as many people report that stress exacerbates Behets. It is often worthwhile checking for and correcting vitamin D deficiency, as vitamin D has many immune-modulating effects and is often at surprisingly low levels in people from Northern Europe. […] For the vast majority of people with Behets, a normal life span is expected. For those with severe eye inflammation, progressive loss of vision was previously a major complication. However, with improving treatments, visual loss is now uncommon. People with purely skin/ulcers/joint disease have a very good prognosis. […] Treatment is based on immunosuppression with agents including steroids, azathioprine, and biologic agents.
  • #50 Behcet’s Syndrome – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/behcets-disease
    Lifestyle modifications to reduce stress should be considered as many people report that stress exacerbates Behets. It is often worthwhile checking for and correcting vitamin D deficiency, as vitamin D has many immune-modulating effects and is often at surprisingly low levels in people from Northern Europe. […] For the vast majority of people with Behets, a normal life span is expected. For those with severe eye inflammation, progressive loss of vision was previously a major complication. However, with improving treatments, visual loss is now uncommon. People with purely skin/ulcers/joint disease have a very good prognosis. […] Treatment is based on immunosuppression with agents including steroids, azathioprine, and biologic agents.
  • #51 Behcet’s Syndrome – Vasculitis UK
    https://www.vasculitis.org.uk/about-vasculitis/behcets-disease
    Lifestyle modifications to reduce stress should be considered as many people report that stress exacerbates Behets. It is often worthwhile checking for and correcting vitamin D deficiency, as vitamin D has many immune-modulating effects and is often at surprisingly low levels in people from Northern Europe. […] For the vast majority of people with Behets, a normal life span is expected. For those with severe eye inflammation, progressive loss of vision was previously a major complication. However, with improving treatments, visual loss is now uncommon. People with purely skin/ulcers/joint disease have a very good prognosis. […] Treatment is based on immunosuppression with agents including steroids, azathioprine, and biologic agents.
  • #52 Advances in the Treatment of Behcet’s Disease
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8136102/
    Most mild forms of major organ involvement are sufficiently controlled by conventional ISs (cISs) such as GCs, azathioprine, cyclosporine A, and mycophenolate mofetil (MMF). […] Although randomized-controlled trials (RCT) are lacking, many biologic agents targeting proinflammatory cytokines, such tumor necrosis factor alpha (TNF), IL-1, IL-6, IL-12, and IL-17 licensed for other rheumatological disorders, became part of the treatment choices in BD. […] A recent controversial issue of biologic use is the optimization of advanced therapies in BD before discontinuation. […] Recently, biosimilars are also used in BD patients. […] Our expert-opinion approach to the management of different organ manifestations in BD is summarized in Table 2. […] Behcets disease usually starts with mucocutaneous manifestations which have a limited impact on vital organ functions and general quality of life (QoL).
  • #53 Behcet Disease Treatment & Management: Approach Considerations, Medical Care, Surgical Care
    https://emedicine.medscape.com/article/329099-treatment
    The goals of therapy in Behet disease are to suppress inflammation and reduce the frequency and severity of recurrences. To be effective, treatment must be started early. The sites and extent of involvement and the severity of disease determine the choice of medication. […] The treatment approach depends on the individual patient, severity of disease, and major organ involvement. The European League Against Rheumatism (EULAR) recommendations for the management of Behet disease, which were developed in 2008 and updated in 2018, aid in the management of different aspects of Behet disease. […] As disease activity subsides, taper medications to the lowest dose that effectively controls disease activity. Inpatient care is based on individual organ-system involvement. Individualize the transfer situation for each patient based on the specifics of organ-system involvement.
  • #54 French recommendations for the management of Behçet’s disease | Orphanet Journal of Rare Diseases | Full Text
    https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-01620-4
    The use of anticoagulants in treating vascular involvement is disputed but remains recommended; inflammation of the vascular walls is the main cause of Behets disease-related thrombosis. […] In its serious and/or complicated forms, Behets disease is categorized as a life-long disorder, with waiver of co-pay (care at 100%). […] Therapeutic education is indispensable to optimize the care and compliance of the patient with the therapeutic project. […] Colchicine is the first-line treatment of mucocutaneous and articular manifestations. […] Reduction (or cessation) of immunosuppressant or immunomodulating treatment can be considered, except in exceptional cases, only after at least 2 years of remission within the framework of severe Behets disease (ophthalmological, digestive, neurological, cardiovascular, and pulmonary damage).
  • #55 Behçet’s Disease, Pathogenesis, Clinical Features, and Treatment Approaches: A Comprehensive Review
    https://www.mdpi.com/1648-9144/60/4/562
    Regular follow-ups are essential to track the course of the disease and the effectiveness of treatment, in addition to pharmacological interventions. For the best treatment of Behçet’s illness, rheumatologists, dermatologists, ophthalmologists, and other specialists must work together in multidisciplinary care.