Pulmonary hemosiderosis (PH) is a rare form of lung disorder characterized by hemorrhage into the lungs, resulting in the classic symptoms of hemoptysis, dyspnea, anemia, and the presence of transient pulmonary infiltrates on X-rays. The anemia is primary and lung damage is secondary to the accumulation of iron within the lungs. Over time, the pulmonary hemorrhage results in thickening of the alveolar basement membrane and interstitial fibrosis. Three variants of PH have been recognized. These include a form of PH associated with antibodies to the lungs and kidney (Goodpasture Syndrome), PH associated with immune complex diseases, and idiopathic PH (IPH), without any measurable immunologic association. Apart from the major characteristics of the condition mentioned above, other symptoms may include diarrhea, vomiting, fatigue, weakness, otitis media, cyanosis, and a failure to thrive.
PH can be suspected, based on the symptoms. In such a case, assaying the blood for iron, studying the chest radiographs, or analyzing the lung aspirations and/or biopsy can lead to a confirmation of the diagnosis. Immediate management of the condition concentrates on respiratory therapy, immunosuppression, and oxygen therapy. In severe cases, blood transfusion may be necessary.
The molecular basis for pulmonary hemosiderosis is not known. However, most studies suggest that the condition is caused due to structural defects in the alveolar capillaries of the basement membrane and/or endothelial cells.