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2025 ICD-10-CM code M34.81

Systemic sclerosis with lung involvement.

Code first the underlying disease. Ensure documentation clearly indicates both the systemic sclerosis and the specific lung involvement.

Medical necessity must be established for all services related to the systemic sclerosis and lung involvement. Documentation must support the need for diagnostic testing, treatment, and other interventions.

Diagnosis involves patient history, physical exam, imaging (X-rays, CT scans), blood tests (autoantibody tests, ESR, CBC), urinalysis, and potentially skin biopsy. Treatment is symptomatic and may include corticosteroids, immunosuppressants, NSAIDs, and physical therapy.

IMPORTANT:If other interstitial pulmonary diseases are present, J84.89 should also be coded.For secondary pulmonary arterial hypertension, use I27.21.

In simple words: This code is used when someone has scleroderma, a condition where the body makes too much scar tissue, and that scar tissue is affecting their lungs.

This code designates systemic sclerosis, also known as scleroderma, with associated lung complications. Systemic sclerosis is an autoimmune disease characterized by the abnormal growth of scar-like tissue, replacing healthy tissue.This specific code (M34.81) identifies cases where the disease affects the lungs.

Example 1: A patient with known limited cutaneous systemic sclerosis presents with increasing shortness of breath and a dry cough.Pulmonary function tests and imaging confirm interstitial lung disease.M34.81 and J84.89 would be used., A patient with diffuse systemic sclerosis develops pulmonary hypertension.M34.81 and I27.21 are applicable., A patient presents with Raynaud's phenomenon, skin thickening, and dyspnea.Further workup confirms systemic sclerosis and pulmonary fibrosis. M34.81 is used to specify the lung involvement.

Documentation should support the diagnosis of systemic sclerosis and specify the lung involvement. This may include clinical findings, imaging results, pulmonary function tests, and other relevant diagnostic data.

** M34.81 is a more specific code than M34.9 (Systemic sclerosis, unspecified) and should be used when lung involvement is documented.The choice between M34.0 (Progressive systemic sclerosis) or M34.1 (CREST syndrome) with M34.81 depends on the specific type of systemic sclerosis present along with lung involvement.

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