A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome?

Gurveen Malhotra,Nitya Ramreddy,Tanjeev Kaur,Serafin Chua,Mira Iliescu

doi:10.1155/2016/7379829

Gurveen Malhotra, Nitya Ramreddy + Show 3 more

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https://doi.org/10.1155/2016/7379829

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Abstract

Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). We report a case of a 43-year-old male who presented with nonresolving pneumonia who was eventually diagnosed to have ADM through a skin biopsy without any muscle weakness. ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis.

Highlights

Antisynthetase (AS) syndrome is a rare disease entity with predominant interstitial lung disease associated with the presence of anti-Jo-1 antibodies
The clinical picture with the presence of Interstitial lung disease (ILD), fever, and arthritis in the setting of amyopathic dermatomyositis (ADM) suggested a diagnosis of AS syndrome and immunosuppression with cyclophosphamide was initiated
Inflammatory myopathies are a diverse group of autoimmune disorders with polymyositis, dermatomyositis, and inclusion body myositis as the major entities

Summary

A Curious Case of Acute Respiratory Failure

Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. It is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS). ADM may be associated with rapidly progressive course of interstitial lung disease (ADM-ILD) which is associated with high mortality. Differentiation between ADM-ILD and AS syndrome may be difficult in the absence of positive serology and clinical presentation may help in clinching the diagnosis

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