Abstract

Purpose of Review This review discusses the epidemiology, evaluation and treatment of overlap inflammatory myopathy syndromes. Recent findings Evaluation of overlap myopathy in systemic sclerosis (SSc) has shown that the disease is often heterogeneous with variable presentation, histopathology, and response to therapy. These differences have shown to be important in management, specifically with the response to immunosuppression. Inflammation and necrosis are features suggestive of a positive response, whereas fibrosis and neurogenic atrophy are also common and may be less responsive to therapy. Summary Inflammatory myopathy associated with overlap connective tissue disease is often associated with greater morbidity, and there are few studies to guide treatment. Treatment is often tailored to the patient based on severity of muscular and concomitant extramuscular disease. While further research is needed to better understand treatment of overlap syndromes, the absence of consensus criteria remains a limiting factor.

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