Pulmonary alveolar proteinosis

This code represents a rare lung disorder characterized by the abnormal accumulation of lipoproteinaceous material within the alveoli, impairing gas exchange. This material is primarily composed of surfactant components, leading to progressive respiratory symptoms.

Assign this code when documentation confirms a diagnosis of pulmonary alveolar proteinosis, whether primary (idiopathic) or secondary to other conditions. Supporting documentation typically includes lung biopsy results, bronchoalveolar lavage findings, and characteristic imaging studies.

• Differentiate between primary (idiopathic) and secondary forms, as secondary forms may require an additional code for the underlying cause.
• Do not confuse with other interstitial lung diseases or pneumonia, as the pathology is distinct.
• Ensure documentation specifies the diagnosis clearly, as symptoms can overlap with other respiratory conditions.