Idiopathic pulmonary hemosiderosis

This code represents idiopathic pulmonary hemosiderosis, a rare chronic lung disease characterized by recurrent diffuse alveolar hemorrhage without an identifiable underlying cause. It leads to iron deposition (hemosiderin) in the lungs, causing progressive pulmonary fibrosis and often iron deficiency anemia.

Use this code for patients diagnosed with idiopathic pulmonary hemosiderosis, typically confirmed by lung biopsy showing hemosiderin-laden macrophages and exclusion of other causes of pulmonary hemorrhage. Documentation should clearly state "idiopathic" or "primary" to differentiate from secondary forms.

• Ensure documentation explicitly states "idiopathic" to support this specific code.
• Code associated iron deficiency anemia (e.g., 280.9) as a secondary diagnosis.
• Do not confuse with other causes of pulmonary hemorrhage, such as Goodpasture's syndrome or vasculitis, which have distinct codes.