Idiopathic Pulmonary Hemosiderosis (IPH) is a disease of unknown cause that follows a remitting course but which often is fatal. Diagnosis requires exclusion of other causes of diffuse alveolar hemorrhage.


Typical patient: child to young adult, with onset often before 10 years of age


Clinical features:

(1) recurrent episodes of hemoptysis

(2) cough

(3) dyspnea

(4) cyanosis


Imaging findings in the lungs:

(1) air space consolidation

(2) ground glass opacities

(3) eventually fibrosis


Laboratory findings:

(1) anemia, sometimes with iron deficiency

(2) sputum, broncho-alveolar lavage or biopsy show hemorrhage and/or large amounts of hemosiderin and hemosiderin-laden macrophages

(3) negative for ANA, ANCA and anti-GBM



(1) glomerulonephritis

(2) vasculitis (including microscopic polyangiitis)

(3) heart failure

(4) infectious pneumonia


Some cases are associated with celiac disease or milk hypersensitivity. Some patients may improve on a gluten-free or milk-free diet.


To read more or access our algorithms and calculators, please log in or register.