Alveolar proteinosis

This code represents a rare pulmonary disorder characterized by the accumulation of lipoproteinaceous material within the alveoli, impairing gas exchange. This material is primarily composed of surfactant proteins and lipids, leading to progressive respiratory insufficiency.

Use this code when documentation confirms a diagnosis of alveolar proteinosis, typically identified through lung biopsy or bronchoalveolar lavage. It applies to both primary (idiopathic) and secondary forms of the disease. Supporting documentation should include diagnostic test results and clinical findings consistent with the condition.

• Differentiate from other interstitial lung diseases; ensure documentation specifically states "alveolar proteinosis."
• Consider additional codes for any associated underlying conditions (e.g., hematologic malignancies, infections) if secondary alveolar proteinosis is diagnosed.
• Ensure the medical record clearly distinguishes between congenital and acquired forms if specified, though this code covers both.