Clinical features of antisynthetase syndrome: A Tunisian cohort

Abstract

Introduction

Antisynthetase syndrome (AS) is an autoimmune idiopathic inflammatory myopathy (IIM) identified by the positivity of anti t-RNA synthetases. Clinical manifestations include inflammatory myopathy, interstitial lung disease (ILD), mechanic’s hands (hyperkeratosis lesions of the fingers), arthritis or Raynaud’s phenomenon.

Objective

The objective of our study was to describe the clinical, immunological, and therapeutic characteristics of AS and to highlight its particularities compared to other IIM.

Methods

Retrospective data from the internal medicine department of Mongi Slim hospital in Tunis were collected over a period of 16years. We compared demographic, clinical, biological and morphological findings, treatments, and outcomes of patients with AS with a group of patients with IIM other than AS.

Results

Thirty patients with AS and twenty patients with other IIMs were included. Patients with AS had significantly more amyopathic forms (p = 0.034) and ILD (p < 0.001). Cyclophosphamide was more frequently used in the treatment of severe ILD than in that of other IIMs (p = 0.023).

Conclusion

AS is a rare entity with a heterogenous clinical spectrum. It is associated with increased morbidity and mortality especially if the lung involvement is severe.