Recovery from severe dysphagia in systemic sclerosis – myositis overlap: a case report.

Keith J Chinniah, Girish M Mody
Department of Rheumatology, School of Clinical Medicine, College of Health Sciences,
University of KwaZulu-Natal and Inkosi Albert Luthuli Central Hospital, Durban, South Africa

Abstract
Background: Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. Clinical case:A 56-year old African Black woman initially presented with systemic sclerosis (SSC) – myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes. Six years later she had a myositis flare with severe dysphagia.

Her myositis improved after high doses of corticosteroids, azathioprine and two doses of intravenous immunoglobulin (IVIG). As her dysphagia persisted, she was fed via a percutaneous endoscopic gastrostomy (PEG) tube and given a course of rituximab. Her dysphagia gradually resolved and the PEG tube was removed within two months. She received another dose of rituximab six months later and continued low dose prednisone and azathioprine. Her muscle power improved, weight returned to normal
and she remained well 20 months after hospital discharge.

Conclusion: Our patient with SSC-myositis overlap and severe dysphagia requiring PEG feeding, improved with high dose corticosteroids, azathioprine, two courses of IVIG and rituximab, and remained in remission 20 months after hospital discharge.

Keywords:Severe dysphagia, systemic sclerosis, myositis overlap.