Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study

Abstract

Background and objective: To describe the clinical characteristics including the bronchoalveolar lavage fluid (BALF) characteristics of patients with antisynthetase syndrome (AS) associated interstitial lung disease (ILD) in a tertiary ILD outpatient clinic, their medical therapy and outcome. Methods: Retrospective cohort study of patients with AS-ILD. All available data of clinical characteristics, pulmonary function tests, laboratory parameters, BALF analysis, histology, high-resolution computed tomography (HRCT) and treatment were collected from the patient files. Results and conclusions: Twelve patients with AS-ILD were identified. Mean age at diagnosis was 55 years (range 45–69), 67% were female. Mean follow-up time was 7 years. The anti-aminoacyl tRNA-synthetase antibodies presented were anti-Jo1 (n = 6), anti-PL7 (n = 3), anti-PL12 (n = 2) and anti-EJ (n = 1). HRCT patterns were mainly non-specific interstitial pneumonia (75%). Four patients had BALF-eosinophilia (two of four anti-Jo1 patients) and two anti-PL12 positive patients had BALF-neutrophilia. Two AS-ILD patients improved on rituximab (RTX) as induction treatment and three out of four patients were stabilised on RTX as maintenance treatment. Two patient obtained remission by cyclophosphamide. Four patients were stabilised on azathioprine alone or in combination with oral corticosteroids. Our cohort of AS-ILD patients showed clinical characteristics in accordance with previous reports at baseline and was comparable to other cohorts. Most patients can be stabilised with immunosuppressive treatment.

Keywords

• antisynthetase syndrome
• bronchoalveolar lavage fluid
• interstitial lung diseases
• polymyositis
• treatments