title: [Pleuroparenchymal fibroelastosis is a new entity which can be mistaken for sarcoidosis].
abstract: Pleuroparenchymal fibroelastosis is an idiopathic interstitial pneumonia, recently recognized as a new entity by American Thoracic Society/European Respiratory Society. We present the first Danish cases of pleuroparenchymal fibroelastosis in two young sisters, who had complained of dyspnoea since childhood. Their lung function was reduced at referral (forced expiratory volume in 1st sec.: 36-43%, forced vital capacity: 34-41%, diffusing capacity for carbon monoxide: 35%). High resolution CT showed apical peripheral consolidation, pleural thickening, traction bronchiectasis and enlarged lymph nodes of both patients; compatible with pleuroparenchymal fibroelastosis. Due to low lung function, lung biopsies were not performed.
authors.affiliation: Lungemedicinsk Afdeling B5, Aarhus Universitetshospital, Nørrebrogade 44, 8000 Aarhus C. thobjerr@rm.dk.
authors.forename: Thomas
authors.initials: T
authors.lastname: Prior
authors.forename: Ole
authors.initials: O
authors.lastname: Hilberg
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bendstrup
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D054988:Idiopathic Interstitial Pneumonias / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000188:drug therapy; D012129:Respiratory Function Tests; D012507:Sarcoidosis / Q000175:diagnosis; D035781:Siblings; D014057:Tomography, X-Ray Computed; D000070857:Walk Test; D055815:Young Adult