Guideline sources The following summarized guidelines for the evaluation and management of Beh et's syndrome (BS) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023) and the European League Against Rheumatism (EULAR 2022; 2018). 1 2 3 Guidelines 1. Diagnostic investigations Pretreatment evaluation: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 2. Medical management Management of mucocutaneous lesions: initiate topical corticosteroids for the treatment of oral and genital ulcers. B Show 4 more https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 1/4 6/29/23, 3:16 AM Beh et's syndrome Pathway Management of uveitis: Ensure close collaboration with ophthalmologists for the management of uveitis in patients with BS with the ultimate aim of inducing and maintaining remission. Initiate any of following in patients with BS and inflammatory eye disease affecting the posterior segment: azathioprine B cyclosporine A B interferon-alpha B monoclonal anti-TNF antibodies. B Show 4 more Management of venous thrombosis: initiate corticosteroids and immunosuppressive agents, such as azathioprine, cyclophosphamide or cyclosporine A, for the management of acute deep vein thrombosis in patients with BS. B Show 2 more Management of arterial aneurysms: initiate high-dose corticosteroids and cyclophosphamide for the management of pulmonary artery aneurysms. B Show 3 more Management of GI involvement: confirm gastrointestinal involvement of BS by endoscopy and/or imaging. Rule out NSAID ulcers, IBD and infections such as tuberculosis. B Show 3 more Management of CNS involvement: Initiate high-dose corticosteroids followed by slow tapering, together with immunosuppressive agents, such as azathioprine, in patients with acute attacks of parenchymal involvement. Avoid using cyclosporine A. Consider initiating monoclonal anti-TNF antibodies in patients with severe disease as first-line or in patients with refractory disease. B Initiate high-dose corticosteroids followed by tapering for the treatment of the first episode of cerebral venous thrombosis. Consider adding anticoagulants for a short duration. Screen for vascular disease at an extracranial site. B Management of arthritis: initiate colchicine as the initial treatment in patients with BS with acute arthritis. B Show 2 more 3. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 2/4 6/29/23, 3:16 AM Beh et's syndrome Pathway Clinical findings Symptoms Past medical history Abdominal pain Budd-chiari syndrome Blurry vision Cerebral venous thrombosis Diarrhea DVT Dysarthria Gastrointestinal fistula Eye pain Intestinal perforation Headache Meningitis Nausea Optic neuritis Personality or behavioral changes Superficial thrombophlebitis Ocular exam Photophobia Visual disturbances Episcleritis Scleritis Neurological exam Uveitis Ataxia Musculoskeletal exam Cranial nerve palsy Hemiparesis Arthritis ICP Polyarticular synovitis Lhermitte's phenomenon Integument exam Head and neck exam Cutaneous lesions Aphtous ulcers Erythema nodosum Oral ulcers Pyoderma gangrenosum Skin papules Genitourinary exam Skin pustules Genital ulcers Genetic testing Lab findings Hla-b51 ESR serum CRP Diagnostic procedures pathergy test References 1. Gulen Hatemi, Robin Christensen, Dongsik Bang et al. 2018 update of the EULAR recommendations for the management of Beh et's syndrome. Ann Rheum Dis. 2018 Jun;77 6 808 818. Open https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 3/4 6/29/23, 3:16 AM Beh et's syndrome Pathway 2. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 3. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 4. Mendes D, Correia M, Barbedo M et al. Behcet's disease a contemporary review. J Autoimmun. 2009 May-Jun;32 3 4 178 88. Open 5. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 6. Expert Panel on Neurological Imaging, Vikas Agarwal, Lubdha M Shah et al. ACR Appropriateness Criteria Myelopathy: 2021 Update. J Am Coll Radiol. 2021 May;18 5S S73 S82. Open https://web.pathway.md/diseases/recDqh7HlDbJG7Aa8 4/4