Baroke Eva, Heussel Claus Peter, Warth Arne, Eichinger Monika, Oltmanns Ute, Palmowski Karin, Herth Felix J F, Kreuter Michael
Department of Pneumology and Respiratory Critical Care Medicine, Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
Translational Lung Research Center Heidelberg, Heidelberg, Germany.
Respirology. 2016 Jan;21(1):191-4. doi: 10.1111/resp.12654. Epub 2015 Oct 13.
PPFE is a rare disease characterized by upper lobe pleural fibrosis and parenchymal fibroelastosis. Its aetiology is considered idiopathic, although possible causative factors have been described. An association of PPFE with solid tumours is unknown and has not been considered previously. We identified six patients with PPFE, four of them with a coexisting malignancy. The case series suggests that PPFE might be an implication of varying factors rather than being an exclusively idiopathic condition.
胸膜肺弹力纤维增生症（PPFE）是一种罕见疾病，其特征为上叶胸膜纤维化和实质纤维弹性组织增生。尽管已有可能的致病因素描述，但其病因仍被认为是特发性的。PPFE与实体肿瘤的关联尚不明确，此前也未被考虑过。我们识别出6例PPFE患者，其中4例同时存在恶性肿瘤。该病例系列表明，PPFE可能是多种因素导致的结果，而非单纯的特发性疾病。
