Idiopathic Pulmonary Hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by recurrent diffuse alveolar hemorrhage in the absence of vasculitis, leading to aggregates of hemosiderin-laden macrophages and eventual interstitial fibrosis. IPH is thought to be due to environmental triggers (household pathogenic molds have been suggested but not proven) in genetically predisposed individuals (e.g., decreased levels of von Willebrand factor). In addition, there is an association with celiac disease (known as Lane-Hamilton syndrome), suggesting a possible autoimmune process.

References

Gordon IO, Cipriani N, Arif Q, Mackinnon AC, Husain AN. Update in nonneoplastic lung diseases. Arch Pathol Lab Med. 2009 Jul;133(7):1096-105.