Anti-Synthetase Syndrome: A Rare and Challenging Diagnosis for Interstitial Lung Disease (ILD): A Case Report

Abstract

Background: ASA syndrome is a rare and complex immune-mediated entity with heterogeneously diverse presentations diagnosed by the presence of antibodies directed against aminoacyl tRNA synthetase (most common anti-JO-1). The clinical manifestations range from myositis, interstitial lung disease (NSIP PATTERN), non-erosive arthritis, unexplained recurrent fever, Raynaud’s phenomenon, mechanics hands involving mainly skin, respiratory and musculoskeletal systems. Case Report: We report a case of 37-year-old female who had multiple admissions for polyarthritis which was symmetrical, progressive, involving large and small joints associated with morning stiffness, relieved by medication temporarily, with subsequent development of respiratory symptoms like cough, fever off and on, shortness of breath, exertional dyspnea, along with cracks on fingers of hands (mechanics hands). Symptoms worsened gradually with time, affecting her daily routine activities like combing hair, changing clothes, exertional dyspnea on taking few steps, debilitating her quality of life. Extensive and detailed workup was done, Anti-Jo-1 antibodies and Anti-SSA/Ro were positive, CPK level raised, serum aldolase raised, HRCT chest showed interstitial lung disease most likely NSIP, ANA negative, ACE levels normal, Bronchial biopsy showed benign respiratory mucosa, no evidence of malignancy was found. Patient was diagnosed with anti-synthetase antibody syndrome and started on pulse therapy with methylprednisolone. Conclusion: This article explores the clinical manifestations, diagnostic criteria, treatment, progression and prognosis of the disease course.