73. Pulmonary Hemosiderosis
Definition
Pulmonary hemosiderosis is repeated or chronic episodes of intra-alveolar bleeding resulting in iron accumulation (hemosiderin) in alveolar macrophages, culminating in pulmonary fibrosis and severe anemia. It can occur as either a primary or secondary disease process.
Incidence
The rate of occurrence of pulmonary hemosiderosis is low but the disease is well recognized. Exact figures of the incidence of pulmonary hemosiderosis have not been reported. In patients younger than 10 years of age, there is a 1:1 male to female ratio, but after 10 years of age, it increases to a 2:1 male to female ratio.
Etiology
Primary pulmonary hemosiderosis has three established variants:
1. Associated with antiglomerular basement membrane (Goodpasture syndrome, see p. 152)
2. Associated with hypersensitivity to cow’s milk proteins (Heiner syndrome)
3. Idiopathic pulmonary hemosiderosis
The patient with idiopathic pulmonary hemosiderosis has a poor prognosis; the mean survival after diagnosis is generally 2.5 to 5 years. Secondary pulmonary hemosiderosis may develop as the result of a number of systemic disorders.
Causes of Secondary Hemosiderosis
Congenital/Acquired Cardiopulmonary Anomalies
• Bronchogenic cyst
• Congenital arteriovenous fistula
• Congenital pulmonary vein stenosis
• Eisenmenger syndrome (see p. 110)
• Left ventricular failure
• Mitral valve stenosis
• Pulmonary arterial stenosis
• Pulmonary embolism
• Pulmonary sequestration
• Pulmonic valve stenosis
• Tetralogy of Fallot
Drugs
• Cocaine
• Penicillamine
Environmental Molds
• Congenital hyperammonemia
• Memroniella echinata
Immunologic Disorders
• Allergic bronchopulmonary aspergillosis
• Henoch-Schönlein purpura
