Progressive systemic sclerosis

This code represents systemic sclerosis, also known as scleroderma, a chronic autoimmune connective tissue disease characterized by widespread fibrosis and vascular abnormalities affecting the skin and internal organs. It is a progressive condition, meaning it tends to worsen over time, impacting various systems such as the gastrointestinal tract, lungs, heart, and kidneys.

Assign this code for patients diagnosed with diffuse cutaneous systemic sclerosis, indicating significant skin involvement and a higher risk of internal organ complications. Documentation should clearly state "systemic sclerosis" or "scleroderma" with evidence of widespread or progressive disease. This code is appropriate when the disease is not limited to a specific organ or localized area.

• Differentiate from localized scleroderma (morphea) which has different ICD-10-CM codes.
• Be sure documentation specifies "systemic" or "diffuse" to support this code.
• Code any associated organ manifestations (e.g., interstitial lung disease, renal crisis) as secondary diagnoses.