Early respiratory involvement leading to respiratory failure in a case of Systemic sclerosis - Polymyositis overlap

Abstract

Polymyositis is an autoimmune myopathy with a preponderance to involve proximal limb muscles in a symmetrical manner. Type two respiratory failure and severe bulbar weakness is not a common mode of initial presentation of polymyositis – systemic sclerosis overlap syndrome. We present a case of a middle age female , who presented with dysphagia, nasal regurgitation, aspiration of food and proximal muscle weakness, worsening over 6 months duration. The disease was rapidly progressing and culminated in severe respiratory muscle weakness and respiratory arrest necessitating invasive ventilation. The diagnosis of sero- negative polymyositis and systemic sclerosis overlap syndrome was made and she achieved a remarkable recovery with intensive care and meticulous immunosuppressive therapy. Physicians should be aware of the rapid respiratory muscle involvement in systemic sclerosis and Polymyositis overlap since timely initiation of treatment is life saving.