Clinical features of Hermansky–Pudlak syndrome (HPS) pulmonary fibrosis and idiopathic pulmonary fibrosis (IPF)

HPS-1 or HPS-4 pulmonary fibrosisHPS-2 pulmonary fibrosisIPF
Onset of pulmonary symptomsMiddle ageChildren or young adults>50 years of age
Acute exacerbationsYesUnknownYes
Oculocutaneous albinismYesYesNo
Excessive bleedingYesYesNo
HRCT scan finding of ground-glass opacificationYesYesNo
Type II epithelial cell enlargementYesYesNo
Giant lamellar bodiesYesYesNo
Foamy alveolar macrophagesYesUnknownNo
Ceroid lipofuscin tissue depositionYesYesNo
Pulmonary histopathologyUIP, NSIP, DADUIP, NSIP, DIPUIP

HRCT: high-resolution computed tomography; UIP: usual interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; DAD: diffuse alveolar damage; DIP: desquamative interstitial pneumonitis.