Purpose of review: This review discusses the most updated literature of myopathy in scleroderma and other connective tissue diseases.
Recent findings: In the past year, studies have demonstrated that myopathy in scleroderma is associated with poor outcomes such as disability and mortality. In addition, muscle histopathology in scleroderma continues to reveal that it is a heterogeneous entity, and that necrosis and acute neurogenic atrophy may be a more prevalent histopathologic feature in muscle biopsies than previously reported. In other connective tissue diseases such as SLE, the onset of overlap myositis typically does not occur simultaneously as it does in scleroderma or rheumatoid arthritis.
Summary: Myopathy in scleroderma is heterogeneous and given that it is associated with poor outcomes, it is imperative that optimal diagnostic strategies and therapies including a classification criterion be developed. In other connective tissue diseases, such as rheumatoid arthritis and systemic lupus erythematosus, myopathy is even more poorly defined and requires more robust studies to clarify both the clinical features and muscle histopathology in this group.