ABSTRACT
Pulmonary hemosiderosis (PH) is a rare disease which primarily affects children and adolescents, and was described first by Virchow in 1864. It is characterized by acute and chronic blood loss which occurs from pulmonary capillaries and often results in the triad of hemoptysis, interstitial pulmonary infiltrates, and anemia. The presentation and clinical course of idiopathic PH (IPH) are variable and depend upon the intensity, duration and frequency of the hemorrhages. Chronic and acute blood loss occurs in IPH, and since the iron contained in hemosiderin trapped within the lung is only scantily reutilized for erythropoiesis, iron deficiency anemia develops in most cases. Pulmonary functions improve during remissions, and may revert to normal before interstitial fibrosis supervenes. Cardiovascular disease leading to pulmonary venous hypertension, especially mitral valve stenosis, can cause diffuse pulmonary hemorrhage and PH. Pulmonary symptoms generally precede renal involvement, and although the clinical course is variable, the disease is often fatal despite treatment with corticosteroids, immunosuppressives and plasmapheresis.
