In the short term, IPH typically presents with hemoptysis, anemia, and diffuse pulmonary infiltrates. In contrast, its long-term presentation is characterized by severe exercise limitation, growth failure, and clubbing. IPH is characterized by repeated episodes of intraalveolar bleeding from an unknown cause, with concomitant hemosiderin accumulation in pulmonary macrophages and thickening of the alveolar basement membrane, eventually leading to pulmonary fibrosis. Is there an explanation for the emphysematous findings in IPH? Although there are no pathognomonic findings on chest CT, varying degrees of fibrosis, honeycombing, and emphysematous changes have been described.