Prior Thomas, Hilberg Ole, Bendstrup Elisabeth
Lungemedicinsk Afdeling B5, Aarhus Universitetshospital, Nørrebrogade 44, 8000 Aarhus C.
Ugeskr Laeger. 2014 May 26;176(22).
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.
胸膜实质纤维弹性组织增生症是一种特发性间质性肺炎，最近被美国胸科学会/欧洲呼吸学会认定为一种新的疾病实体。我们报告了丹麦首例胸膜实质纤维弹性组织增生症，患者为两名年轻姐妹，她们自幼就有呼吸困难的症状。转诊时她们的肺功能下降（第1秒用力呼气量：36 - 43%，用力肺活量：34 - 41%，一氧化碳弥散量：35%）。高分辨率CT显示两名患者均有肺尖外周实变、胸膜增厚、牵拉性支气管扩张和淋巴结肿大；符合胸膜实质纤维弹性组织增生症的表现。由于肺功能低下，未进行肺活检。
