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, blood vessels, and internal organs such as the lungs, heart, kidneys, and gastrointestinal tract. It can manifest as limited cutaneous systemic sclerosis (lcSSc) or diffuse cutaneous systemic sclerosis (dcSSc), with varying degrees of organ involvement and prognosis.

Use this code for patients diagnosed with systemic sclerosis, regardless of the specific subtype (limited or diffuse) or the presence of specific organ involvement. Documentation should clearly state a diagnosis of "systemic sclerosis" or "scleroderma." This code is appropriate when the condition is the primary reason for the encounter or a significant co-morbidity impacting care.

• Do not use this code for localized scleroderma (morphea) or linear scleroderma; these are coded elsewhere.
• Sequence this code as the primary diagnosis when systemic sclerosis is the focus of treatment or evaluation.
• Code all manifestations and complications of systemic sclerosis, such as interstitial lung disease, pulmonary hypertension, or renal crisis, in addition to this code.