Zhang Shuai, Xie Wanmu, Wang Zhiru, Tian Ye, Da Jiping, Zhai Zhenguo
Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, P.R. China.
National Clinical Research Center for Respiratory Diseases, China-Japan Friendship Hospital, Beijing 100029, P.R. China.
Exp Ther Med. 2019 Apr;17(4):2557-2560. doi: 10.3892/etm.2019.7275. Epub 2019 Feb 13.
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. The patient was admitted due to a 9-year history of progressive cough and exertional dyspnea, as well as a history of Hodgkin's lymphoma treated by autologous hematopoietic stem cell transplantation (HSCT). The patient had been initially diagnosed with tuberculosis and received regular anti-tuberculosis therapy for 18 months; however, the symptoms progressed. Serial chest computed tomography scans indicated a gradually worsening diffuse pleural thickening, dense subpleural opacification and volume loss, associated with evidence of fibrosis in the right lung. On physical examination the patient was cachectic, with a body mass index of 18.5 kg/m, and he had a flattened thoracic cage. Arterial blood gas analysis revealed hypoxia. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. The microbiological and cytological examinations were negative. Lung biopsy revealed a thickened pleura consisting of large amounts of collagen and elastic fibers, coexisting with subpleural intra-alveolar fibrosis with alveolar septal elastosis, without inflammatory infiltrates. The patient was diagnosed with PPFE secondary to HSCT and eventually succumbed to respiratory failure and infection while waiting for a lung transplant. Physicians should be aware of the typical and atypical characteristics of this rare disease, as its clinical and radiological characteristics may lead to misdiagnosis, particularly as chronic infections. The prognosis remains poor without effective long-term treatment.
胸膜实质纤维弹性组织增生症（PPFE）是一种罕见的间质性肺疾病。尽管近期报道的PPFE病例数量有所增加，但这种疾病的特征尚未得到充分描述。本研究报告了一名34岁男性患者的病例，该患者出现单侧肺部异常。患者因9年的进行性咳嗽和劳力性呼吸困难病史，以及曾接受自体造血干细胞移植（HSCT）治疗的霍奇金淋巴瘤病史而入院。患者最初被诊断为肺结核，并接受了18个月的常规抗结核治疗；然而，症状仍有进展。胸部计算机断层扫描系列检查显示弥漫性胸膜增厚逐渐加重、胸膜下致密性混浊和肺容积缩小，同时右肺有纤维化迹象。体格检查发现患者消瘦，体重指数为18.5kg/m，胸廓扁平。动脉血气分析显示缺氧。肺功能测试显示限制性通气功能障碍和弥散能力下降。微生物学和细胞学检查均为阴性。肺活检显示胸膜增厚，由大量胶原纤维和弹性纤维组成，同时存在胸膜下肺泡内纤维化伴肺泡间隔弹性组织增生，无炎症浸润。该患者被诊断为HSCT继发的PPFE，最终在等待肺移植期间死于呼吸衰竭和感染。医生应了解这种罕见疾病的典型和非典型特征，因为其临床和放射学特征可能导致误诊，尤其是误诊为慢性感染。若无有效的长期治疗，预后仍然很差。
