Systemic sclerosis with myopathy

This code signifies a chronic, autoimmune connective tissue disease characterized by widespread fibrosis and vascular abnormalities, specifically when it also involves muscle inflammation and weakness. It describes the systemic form of scleroderma that has progressed to affect the musculature.

Apply this code when documentation clearly indicates a diagnosis of systemic sclerosis (scleroderma) co-occurring with myopathy. This typically involves findings such as muscle weakness, elevated muscle enzymes, and/or characteristic muscle biopsy results in a patient with systemic sclerosis.

• Ensure documentation specifies "systemic" sclerosis, not localized scleroderma (morphea), as the latter has different coding.
• Verify that myopathy is explicitly documented as a manifestation of the systemic sclerosis, not an unrelated muscle disorder.
• Consider sequencing this code after the primary manifestation or complication if the myopathy is a secondary condition.