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Abstract：
Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a very rare and a slowly conspicuous progressing chronic lung disease, which usually involves the upper lobes of the lung. This unusual disease, first recognized as a rare idiopathic interstitial pneumonia in 2013, is characterized by dense fibrosis of the visceral pleura and the subjacent lung parenchyma accompanied by elastosis predominating in the subpleural alveolar walls. In the interest of improving our understanding of this uncommon disease, we report a case of IPPFE established by pathology results. Case report: A 73-year-old male patient, smoker, with a medical history of chronic obstructive pulmonary disease, presented since January 2022 with a gradual worsening of dyspnea on exertion and productive cough with weight loss. The chest X-ray detected a thoracic distention. The chest high resolution computed tomography revealed biapical subpleural parenchymatous condensations with tractive bronchiectasis and pleural retraction in the right upper lobe and diffuse bilateral cento-lobular emphysema. A scan-guided trans-parietal lung biopsy showed lung parenchyma tattooed with anthracosic deposits, largely remodeled by fibrous tissue, intermingled with numerous wavy and refractive dyselastotic structures in polarized light. The orcein staining confirmed the presence of excess elastosic fibers within these lesions. All etiological investigations were negative. His lung function studies revealed a reversible obstructive ventilatory disorder. Following a multidisciplinary discussion, the diagnosis of IPPFE was confirmed on the basis of the distribution in the upper lungs on chest computed tomography combined with pathology pattern. Conclusions: This case emphasizes the atypical misleading radiological presentation of IPPFE and the key role of pathological results in establishing the diagnosis. Hence, further studies are needed to improve our understanding of this uncommon disease and to establish clear-cut guidelines for IPPFE diagnosis and management.
摘要：
背景：特发性胸膜实质纤维弹性增生症（IPPFE）是一种非常罕见且缓慢明显进展的慢性肺部疾病。通常累及肺的上叶。这种不寻常的疾病,2013年首次被认为是罕见的特发性间质性肺炎，其特征是内脏胸膜和下肺实质的致密纤维化，并伴有主要在胸膜下肺泡壁的弹性变性。为了提高我们对这种罕见疾病的认识，我们报告一例由病理结果确定的IPPFE。病例报告：一名73岁的男性患者，吸烟者,有慢性阻塞性肺疾病病史,自2022年1月以来，劳累时呼吸困难逐渐恶化，并伴有体重减轻。胸部X线检查发现胸部扩张。胸部高分辨率计算机断层扫描显示双尖胸膜下实质凝结，右上叶有牵张性支气管扩张和胸膜回缩以及弥漫性双侧中央小叶气肿。扫描引导的跨顶叶肺活检显示肺实质纹身有炭疽病沉积，大部分被纤维组织重塑，在偏振光中与许多波浪状和屈光性畸形结构混合在一起。orcein染色证实了这些病变中存在过量的弹性纤维。所有病因调查均为阴性。他的肺功能研究显示可逆性阻塞性通气障碍。经过多学科的讨论，IPPFE的诊断是根据胸部计算机断层扫描在上肺的分布并结合病理类型来确定的。结论：该病例强调了IPPFE的非典型误导性放射学表现以及病理结果在建立诊断中的关键作用。因此，需要进一步的研究来提高我们对这种罕见疾病的认识,并建立明确的IPPFE诊断和治疗指南.
