Idiopathic Pulmonary Hemosiderosis
Key Facts
Terminology
Intrapulmonary hemorrhage of unknown etiology that is unassociated with other systemic manifestations
Clinical Issues
Affects children more commonly than adults
About 20% of patients are adults > 30 years old
Hemoptysis
Iron deficiency anemia
Cough
Dyspnea
Microscopic Pathology
Hemorrhage diffusely involving alveolar spaces
Abundance of intraluminal hemosiderin-laden macrophages within bronchial lumens
Iron stains are strongly positive in alveolar macrophages
 Histologic appearance of the lung in idiopathic pulmonary hemosiderosis shows massive fresh intraalveolar hemorrhage. |
 Idiopathic pulmonary hemosiderosis shows abundance of intraluminal hemosiderin-laden macrophages in alveolar lumens. |
TERMINOLOGY
Abbreviations
Idiopathic pulmonary hemosiderosis (IPH)
Synonyms
Idiopathic pulmonary hemorrhage
Definitions
Intrapulmonary hemorrhage of unknown etiology that is unassociated with other systemic manifestations
ETIOLOGY/PATHOGENESIS
Etiology
Unknown
No immunologic mechanism has been identified
Familial clustering has been reported, supporting genetic susceptibility
CLINICAL ISSUES
Epidemiology
Age
Affects children more commonly than adults
About 20% of patients are adults, usually < 30 years old
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
