Pulmonary alveolar proteinosis in association with Fanconi's anemia and psoriasis. A possible common pathogenetic mechanism.
We report a case of PAP which proved to be fatal despite whole lung lavage. The need for early BAL and transbronchial biopsies in diffuse infiltrative lung disorders of unknown etiology is highlighted. The occurrence of PAP in association with Fanconi's anemia and psoriasis raises the possibility of a common pathogenetic defect which may be related to abnormal cytokine metabolism. Investigation of cytokine metabolism in PAP is warranted.
Steens
R D
RD
Department of Respiratory Medicine, Royal Perth Hospital, Western Australia.
Summers
Q A
QA
Tarala
R A
RA
D000328:Adult; D001706:Biopsy; D001980:Bronchi / Q000473:pathology; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology / Q000382:microbiology; D005199:Fanconi Anemia / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging; D008271:Mycetoma / Q000175:diagnosis; D009894:Opportunistic Infections / Q000175:diagnosis; D011541:Pseudallescheria / Q000302:isolation & purification; D011565:Psoriasis / Q000175:diagnosis / Q000209:etiology*; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000209:etiology*; D011859:Radiography