Hemosiderosis, Pulmonary

Classically affects infants and children (80% manifest before 10 y old), but can affect any age group.

No predilection for males or females.

Restrictive lung disease

Pulm Htn

Cor pulmonale

Alveolar hemorrhage

Increased need for transfusion secondary to acute and chronic anemia

Adrenal insufficiency secondary to chronic steroid use

Rare disorder of unknown etiology characterized by repeated episodes of intraalveolar hemorrhage and deposition of hemosiderin in alveolar macrophages. The cycle of recurrent hemorrhage frequently leads to the development of pulm fibrosis, pulm Htn, and cor pulmonale. Disease course is variable and can be marked by multiple spontaneous remissions, and the extent of pulm hemorrhage can be massive, leading to early death, or can be clinically insignificant.

Presents with classically with the triad of hemoptysis, anemia, and pulm infiltrates on CXR.

Unknown; thought to be immune-mediated due to its responsiveness to immunosuppressive therapy

Associated with immune-mediated disorders such as Goodpasture syndrome, SLE, Heiner syndrome, and Wegener granulomatosis, which can cause diffuse alveolar hemorrhage via immune-mediated mechanisms

Immunosuppression with steroids (IV and PO) and supportive care. Other immunosuppressive agents such as azathioprine, chloroquine, and cyclophosphamide may be tried in steroid-unresponsive pts.