Management of major organ involvement of Behçet’s syndrome: a systematic review for update of the EULAR recommendations

Özgüler, Yeşim; Leccese, Pietro; Christensen, Robin; Esatoğlu, Sinem Nihal; Bang, Dongsik; Bodaghi, Bahram; Çelık, Aykut Ferhat; Fortune, Farida; Gaudric, Julien; Gül, Ahmet; Kötter, Ina; Mahr, Alfred; Moots, Robert J.; Richter, Jutta; Saadoun, David; Salvarani, Carlo; Scuderi, Francesco; Sfikakis, Petros P.; Síva, Aksel; Stanford, Miles; Tuğal-Tutkun, İlknur; West, Roy; Yurdakul, Sebahattin; Olivieri, Ignazio; Yazıcı, Hasan; Hatemi, Gülen

doi:10.1093/rheumatology/key242

Cited by 105 publications

(67 citation statements)

References 104 publications

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“…Immunosuppressants demonstrated a decrease in recurrence of deep vein thrombosis while aminosalicylate drugs and azathioprine were found beneficial in gastrointestinal involvement. Immunosuppressants and glucocorticoids improved outcomes in nervous system involvement, and, relevant to this case, cyclophosphamide and high-dose glucocorticoids demonstrated decreased mortality in patients with PAP 9. Some cases of BD with pulmonary artery involvement require urgent intervention due to rapid enlargement or rupture of the PAP 10…”

Section: Discussionmentioning

confidence: 66%

Large pulmonary artery pseudoaneurysm in a patient with Behçet’s disease treated with an Amplatzer vascular plug

Gutierrez¹,

Cabrera

2019

BMJ Case Rep

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Section: Discussionmentioning

confidence: 66%

Large pulmonary artery pseudoaneurysm in a patient with Behçet’s disease treated with an Amplatzer vascular plug

Gutierrez¹,

Cabrera

2019

BMJ Case Rep

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“…In case of aneurysms of the aorta or in peripheral arteries, medical treatment with cyclophosphamide and corticosteroids is recommended as first choice, before considering operative repair [12]. The recurrence rate of DVT is significantly decreased when these patients are treated with immunosuppressives while anticoagulants did not reduce recurrence of DVT as shown in a meta-analysis of casecontrol studies [13]. If a patient also has extensive acute thrombosis, starting anticoagulation may be considered like in seven of our cases.…”

Section: Discussionmentioning

confidence: 75%

“…The use of anticoagulants and anti- fibrinolytic agents in BD is not currently recommended by EULAR [12]. As biologic treatments, mostly TNFinhibitors have become more important in the treatment of patients with BD, and further studies are needed to determine the place of biologics in HSS [13].…”

Section: Discussionmentioning

confidence: 99%

Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

et al. 2019

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To describe the pattern of pulmonary artery vasculitis and the characteristic computed tomographic pulmonary angiography (CTPA) signs in patients with Hughes-Stovin syndrome (HSS). In a retrospective study, the medical records of eight HSS patients (six men), seen between February 2008 and January 2018, were reviewed regarding history, disease characteristics, laboratory investigations, imaging, and treatments. The mean (SD) age was 37.375 ± 8.65 years (range 30-55) and mean (SD) follow-up 30 ± 41.60 months (range 9-132). In all patients, routine laboratory investigations and complete coagulation profile were done. In all, CTPA studies were performed as well as and Doppler ultrasound for suspected deep vein thrombosis (DVT). Four patients had a history of thrombophlebitis, and DVT was observed in all, in two cases bilateral. Arterial thromboses involving popliteal, tibial, common iliac, and femoral arteries were observed in one patient. All patients had mild to moderate hemoptysis, and one had massive hemoptysis. None of the patients had a history of recurrent mouth and/or genital ulcers, uveitis, or arthritis. In all patients, CTPA identified bilateral pulmonary artery aneurysms (PAAs) with adherent in situ thrombosis and mural enhancement in all patients. Lobar PA branches were involved in all patients, segmental in six and main PA in five patients. Proper immunomodulators were initiated early, with favorable outcome; none was treated with TNF-α antagonists. HSS is a systemic vasculitis that may affect virtually all major veins and arteries in patients with normal coagulation profile. PAAs, adherent in situ thrombosis, and mural wall enhancement are characteristic CTPA signs. Early treatment with immunomodulators is essential. Key Points • Hughes Stevin syndrome (HSS) is a systemic vasculitis that may affect virtually all major veins and arteries in patients. It has a normal coagulation profile. • Computed tomography (CT) pulmonary angiography is considered to be the most important diagnostic tool to assess the degree and the extent of the characteristic pulmonary artery aneurysms, and in situ thrombosis, and mural wall enhancement. • It is likely that HSS syndrome is often not recognized and misdiagnosed as deep venous thrombosis (DVT) with pulmonary thromboembolism. • Early treatment with combined immunomodulators is essential to ensure favorable outcome.

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“…It has been reported that due to its antiangiogenic activity, it also leads to reperfusion of occluded vessels and regression of neovascularization. 30 , 90 , 91 , 92 , 93 , 94 , 95 , 96 , 97 , 98 There is no standard usage. Some recommend starting at a high dose and then tapering after a response is achieved, while others prefer to start with a low dose and increase the dose according to the response achieved.…”

Section: Introductionmentioning

confidence: 99%

“…The main adverse effects include the influenza-like symptoms experienced by nearly all patients, especially at the beginning of treatment, as well as alopecia, elevated liver enzymes, thyroiditis, autoantibody formation, weight loss, and depression. 21 , 97 The most important advantage of IFN-a-2a is that it can provide long-lasting remission that persists even after treatment is discontinued, and that the same effectiveness can be attained if treatment must be reinitiated. 95 , 96 …”

Section: Introductionmentioning

confidence: 99%

Behçet’s Uveitis: Current Diagnostic and Therapeutic Approach

Özdal¹

2020

tjo

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Behçet’s disease is a chronic, multisystem inflammatory disorder characterized by relapsing inflammation. Although its etiopathogenesis has not yet been clarified, both the adaptive and innate immune systems, genetic predisposition, and environmental factors have all been implicated. It is more frequent and more severe in males in the third and fourth decades of life. The eye is the most frequently involved organ in the course of the disease. Ocular involvement (Behçet’s uveitis) is characterized by bilateral recurrent non-granulomatous panuveitis and occlusive retinal vasculitis. Recurrent inflammatory episodes in the posterior segment may lead to permanent vision loss due to irreversible retinal damage and complications such as macular scarring, macular atrophy, and optic atrophy. Early and aggressive immunomodulatory treatment and the use of biologic agents when needed are crucial for preventing recurrences and improving visual prognosis.

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Management of major organ involvement of Behçet’s syndrome: a systematic review for update of the EULAR recommendations

Abstract: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

Cited by 105 publications

References 104 publications

Large pulmonary artery pseudoaneurysm in a patient with Behçet’s disease treated with an Amplatzer vascular plug

Large pulmonary artery pseudoaneurysm in a patient with Behçet’s disease treated with an Amplatzer vascular plug

Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

Behçet’s Uveitis: Current Diagnostic and Therapeutic Approach

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