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Philip Murray, Mohammed Mubin, Henry Knott, Bharat Markandey, Nikita Joji, Rahul Malhotra, Alastair Denniston; Evidence-Based Analysis for the Medical Treatment of Behçet’s Disease. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5373.
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Behçet’s disease (BD) is a multisystem, remitting-relapsing, potentially blinding disease of unknown aetiology. The mainstay of treatment is systemic corticosteroid and immunosuppression. We wished to quantify the available levels of evidence for the treatment of BD with regards to the different systems involved.
We performed a Medline, EMBASE and CENTRAL literature search between 1975 - 2010 for papers on the treatment of BD. Inclusion criteria were: written in English, meta analyses, systematic reviews of RCTs, RCTs, cohort studies, case control studies, case series involving more than 20 patients, and expert opinion. Assessment of eligible studies included system involved, number of patients in study, level of evidence according to SIGN (Scottish Intercollegiate Group Network) criteria, and therapy used.
From an initial scope of 2892 papers, 93 papers fulfilled the inclusion criteria. Only 25% were graded as SIGN 1 (meta-analyses, systematic reviews of RCTs, and RCTs). Treatments included corticosteroids, immunosuppressants and biologics. Many RCTs were poorly designed with small patient numbers and short follow up times. Just under 50% of the 93 studies included patients with ocular disease. Patients with orogenital ulceration and skin lesions comprised 31% and 23% of the 93 studies, respectively. Patients with musculoskeletal and vascular manifestations were each mentioned in about 15% of studies, with a paucity of studies on CNS disease.
BD has potentially sight and life threatening complications but the quality of current evidence for therapy is poor. A myriad of different treatments are being employed for numerous systemic manifestations.
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