A characterisation of patients with Antisynthetase Syndrome>

Abstract

Introduction: Antisynthetase syndrome describes a clinically heterogeneous form of idiopathic inflammatory myopathy which is characterised by interstitial lung disease (ILD) and antisynthetase antibodies, in the context of myositis, Raynaud phenomenon, mechanics hands and arthralgia. It has an estimated prevalence of 1/25,000-1/33,000 worldwide and typically affects women twice as commonly as men.1 The ILD clinic in Portsmouth serves as a tertiary referral centre for hospitals across Hampshire and currently looks after nine patients with a confirmed diagnosis of Antisythetase Syndrome. Method: We reviewed all patients with a confirmed diagnosis and characterised their condition. Results: The population consists of four women and five men with an average age at diagnosis of 55 (range 38-67). Autoantibodies to Jo-1 were positive in all patients whilst anti-Ro-52 antibodies were positive in 2 patients. Lung involvement characterised on HRCT was described as organising pneumonia in 8 patients and non-specific interstitial pneumonia (NSIP) in 1 patient. The following systemic symptoms were described; rash (54%) mechanic hands, (44%), Raynaud’s phenomenon (33%), myalgia (33%) and sicca symptoms (22%). The mean FVC pre-treatment was 2.44L and mean TLCO 4.02. Following treatment with a variety of immunosuppressive treatments, the mean FVC was 2.68L and mean TLCO 4.89. There was an average of 12% improvement in FVC and 25% improvement in TLCO, compared with baseline readings. Conclusion: Early, aggressive treatment with immunosuppression is important to provide patients with the best chance of improvement in lung function and outcome. 1 www.orpha.net Accessed 14/2/19