Idiopathic pulmonary hemosiderosis

This code represents a rare, chronic lung disorder characterized by recurrent episodes of diffuse alveolar hemorrhage, leading to iron deposition (hemosiderin) in the lungs. It is idiopathic, meaning the cause is unknown, and primarily affects children and young adults. Patients often present with anemia, cough, dyspnea, and hemoptysis.

Assign this code when documentation clearly states a diagnosis of idiopathic pulmonary hemosiderosis. This diagnosis is typically supported by clinical findings, imaging studies showing diffuse infiltrates, and bronchoalveolar lavage or lung biopsy revealing hemosiderin-laden macrophages without an identifiable underlying cause.

• Ensure the documentation explicitly states "idiopathic" to differentiate from secondary pulmonary hemosiderosis.
• Do not code with R04.81 (acute idiopathic pulmonary hemorrhage in infants [AIPHI]) as per the Excludes1 note.
• Consider coding associated conditions such as iron deficiency anemia (D50.0) or respiratory failure (J96.-) as secondary diagnoses.