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
Exclusions:
(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.
