ICD-10 Code J84.01 – Alveolar Proteinosis (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code J84.01 – Alveolar proteinosis
What it is
J84.01 identifies alveolar proteinosis, a rare lung disorder where proteinaceous material accumulates in the alveoli and interferes with normal gas exchange. Use it for the documented diagnosis of pulmonary alveolar proteinosis.
Clinical signs
Clinical features may include progressive shortness of breath, cough, and reduced exercise tolerance. Imaging and bronchoalveolar findings are often used to support the diagnosis; clinical features vary, refer to documentation.
When to use this code
Use J84.01 when the provider documents alveolar proteinosis or pulmonary alveolar proteinosis as the confirmed diagnosis. It is appropriate for encounters focused on evaluation, follow-up, or management of this specific interstitial lung disease. Code only when the condition is clearly stated in the record.
Do not use for
Do not use this code for nonspecific dyspnea, cough, or abnormal chest imaging without a documented diagnosis. Check documentation if the record instead describes another interstitial, infectious, or occupational lung disorder.
Coding tip
Confirm the exact wording in the assessment; if the note says “alveolar proteinosis” or “pulmonary alveolar proteinosis,” J84.01 is the specific code.