Idiopathic Inflammatory Myopathies: A Case of a Woman with Antisynthetase Syndrome

Abstract

Antisynthetase Syndrome (ASS) is a rare disorder characterized by myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against aminoacyl transfer RNA synthetases (anti-ARS), especially anti-Jo-1. Patients with progressive interstitial lung disease have a poorer prognosis and lung involvement is the leading cause of morbidity and mortality. Presence of "Mechanic's hands" with hyperkeratosis on the palmar side of the fingers and fissuring at their tips and radial margins, is an important diagnostic manifestation of this syndrome. Early diagnosis followed by immunosuppressive therapy can significantly improve the health status of these patients and increase their quality of life. This article reviews some of the most important pathophysiologic, clinical and diagnostic features of Inflammatory Idiopathic Miopathies and presents the case of a woman with myalgia, malaise, cough, chest pain and skin involvement who was diagnosed with Antisynthetase Syndrome and received systemic treatment.