Behçet Disease

Arrange urgent ophthalmologic examination for red eye, new floaters, blurred vision, reduced acuity, or photophobia in a patient with suspected or established Behçet disease. Eye involvement includes anterior or posterior uveitis, vitreous inflammatory cells on slit-lamp examination, and retinal vasculitis; ocular disease is a poor-prognosis phenotype and may occur in approximately 50% of patients. BMJ+2BMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical NeurologyBMJ2018 update of the EULAR recommendations for the management ...NEJMBehçet's Syndrome

Escalate immediately for focal neurologic deficits, altered cognition, severe persistent headache, or symptoms suggesting cerebral venous thrombosis. Neurologic Behçet disease requires a diagnostic evaluation that distinguishes inflammatory parenchymal disease from vascular cerebral venous disease because both alter acute management and prognosis. BMJ+1BMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical NeurologyBMJ2018 update of the EULAR recommendations for the management ...

Evaluate hemoptysis, pleuritic chest symptoms, unexplained venous thrombosis, limb ischemia, or pulsatile masses as possible vascular Behçet disease. The vasculitic process can involve arteries or veins of any size, including pulmonary arteries, and pulmonary arterial aneurysm rupture is a recognized catastrophic complication. AHA Journals+1AHA Journals2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM ...NatureDifferential diagnosis and management of Behçet syndrome | Nature Reviews Rheumatology

Assess overt gastrointestinal bleeding, acute abdominal pain, obstruction, or suspected perforation urgently. Gastrointestinal Behçet disease can produce ulceration, bleeding, and perforation; confirm inflammatory digestive involvement with endoscopy and/or imaging while excluding infectious and NSAID-related ulceration. AHA Journals+1AHA Journals2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM ...PubMedFrench recommendations for the management of Behçet’s disease

Establish the diagnosis from the longitudinal pattern of recurrent oral and genital aphthosis, skin lesions, ocular inflammation, and compatible vascular, neurologic, gastrointestinal, or articular disease. There is no diagnostic test for Behçet disease; manifestations may occur in different combinations and may emerge sequentially over time. Nature+1NatureDifferential diagnosis and management of Behçet syndrome | Nature Reviews RheumatologyBMJBehcet syndrome - Symptoms, diagnosis and treatment | BMJ Best Practice US

Use the 2014 International Criteria for Behçet's Disease as a structured adjunct: oral aphthosis, genital aphthosis, and ocular lesions each contribute 2 points; skin lesions contribute 1 point. Neurologic and vascular manifestations are also included in the criteria, and a pathergy test can add diagnostic support. BMJBMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

If performing pathergy testing, have the reaction read by a clinician 24 to 48 hours after testing. A positive result supports but does not independently establish the diagnosis; its diagnostic performance varies across populations. BMJ+1BMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical NeurologyScienceDirectHLA-B51 - an overview

Document the phenotype at every baseline assessment: mucocutaneous activity, eye disease, arthritis, vascular events, neurologic involvement, and gastrointestinal symptoms. This is essential because management is individualized according to the organ involved and its severity, and ocular, vascular, neurologic, and gastrointestinal involvement carry a poorer prognosis than isolated mucocutaneous disease. BMJBMJ2018 update of the EULAR recommendations for the management ...

For oral and genital ulcers, use topical corticosteroids for active lesions. For prevention of recurrent mucocutaneous disease, try colchicine first, particularly when genital ulcers or erythema nodosum-like lesions are the dominant manifestations. BMJBMJ2018 update of the EULAR recommendations for the management ...

French recommendations list colchicine 1 to 2 mg/day for mucocutaneous disease and refractory oral ulcers can be treated with apremilast 30 mg twice daily. In a phase 2 trial, apremilast reduced oral-ulcer incidence and severity; use it for oral-ulcer-predominant disease after weighing the absence of demonstrated benefit for organ-threatening disease in that trial population. PubMed+1PubMedFrench recommendations for the management of Behçet’s diseaseNEJMTrial of Apremilast for Oral Ulcers in Behçet's Syndrome

For mucocutaneous disease not controlled with colchicine or apremilast, phenotype-directed alternatives include azathioprine 2 mg/kg/day, anti-TNF therapy, thalidomide 50 to 100 mg/day, or ustekinumab in the French recommendations. Select escalation with rheumatology input and avoid treating oral ulcers alone as a proxy for control of ocular, vascular, neurologic, or gastrointestinal disease. PubMed+1PubMedFrench recommendations for the management of Behçet’s diseaseBMJ2018 update of the EULAR recommendations for the management ...

For acute arthritis, consider intra-articular corticosteroid injection, NSAIDs, or short-term oral corticosteroids. Colchicine at 1 to 2 mg/day is a listed option for articular disease; recurrent or refractory arthritis may prompt azathioprine 2 mg/kg/day, methotrexate 0.3 mg/kg/week, or anti-TNF therapy. PubMedPubMedFrench recommendations for the management of Behçet’s disease

For ocular, vascular, neurologic, or gastrointestinal disease, use coordinated multidisciplinary care because treatment intensity must reflect the affected organ and severity. EULAR emphasizes individualized treatment according to age, sex, organ involvement, disease severity, and patient preferences. BMJBMJ2018 update of the EULAR recommendations for the management ...

For confirmed digestive involvement, French recommendations use systemic corticosteroids at 0.5 mg/kg/day with azathioprine 2 mg/kg/day or 5-aminosalicylic acid. In severe disease, anti-TNF-alpha therapy is indicated; confirm disease by endoscopy and/or imaging and exclude infectious or NSAID-induced ulceration before initiating this pathway. PubMedPubMedFrench recommendations for the management of Behçet’s disease

Ocular disease merits early ophthalmology-rheumatology co-management because retinal vasculitis and posterior uveitis are vision-threatening. Evidence-based management recommendations identify immunosuppressive treatment and biologic therapy, including anti-TNF approaches, as central options for severe or refractory Behçet uveitis, while treatment selection depends on the ocular phenotype and prior response. BMJ+1BMJ2018 update of the EULAR recommendations for the management ...BMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

Vascular disease should prompt imaging of the symptomatic territory and assessment for arterial aneurysm, particularly with thoracic symptoms or hemoptysis. Behçet disease uniquely can involve both arterial and venous beds; vascular treatment is manifestation-specific and systemic corticosteroids are described as typical therapy for vascular involvement. AHA JournalsAHA Journals2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM ...

For neurologic Behçet disease, involve neurology and rheumatology early. Controlled treatment trials for neurologic complications remain lacking, so treatment decisions depend on whether presentation is parenchymal inflammatory disease or cerebral venous thrombosis and on clinical severity. BMJBMJNeurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome | Practical Neurology

At follow-up, ask specifically about visual change, neurologic symptoms, thrombosis symptoms, hemoptysis, gastrointestinal bleeding, abdominal pain, and new genital ulcers or skin lesions. Clinical manifestations can arise in different combinations and sequences, so isolated mucocutaneous disease does not exclude subsequent systemic involvement. BMJ+1BMJBehcet syndrome - Symptoms, diagnosis and treatment | BMJ Best Practice USWolters KluwerBehçet's disease unraveled

Use a lower threshold for structured surveillance and rapid reassessment in men, younger patients, and those with genetic links to endemic regions because severe manifestations and mortality are generally greatest in men, patients younger than 35 years, and those with links to endemic areas. NatureNatureDifferential diagnosis and management of Behçet syndrome | Nature Reviews Rheumatology

Base treatment de-escalation on sustained control of the highest-risk organ manifestation rather than improvement in oral ulcers alone. EULAR notes that manifestations may ameliorate over time, but ocular, vascular, neurologic, and gastrointestinal involvement remain poor-prognosis features requiring individualized management. BMJBMJ2018 update of the EULAR recommendations for the management ...