Department of Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry.
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry.
Monaldi Arch Chest Dis. 2023 Aug 2;94(2). doi: 10.4081/monaldi.2023.2632.
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition characterized by pleural and subpleural lung fibroelastosis with an upper lobe predominance. We present the third case of idiopathic PPFE from India, as well as the second ante-mortem diagnosis. A 27-year-old man presented with a 1-year history of modified Medical Research Council class II shortness of breath and dry cough. He described a 15-kg weight loss. After a clinico-radiological diagnosis, he was given anti-tubercular treatment and referred because he showed no improvement. A high-resolution computed tomography of the chest revealed bilateral upper lobe bullae, parenchymal and subpleural fibrosis, and irregular pleural thickening. PPFE was found in surgical lung and pleural biopsies. He was given systemic glucocorticoids but did not respond clinically or radiologically. Pirfenidone and a lung transplant were out of reach for him. He died 9 months after being diagnosed with his condition. Finally, idiopathic PPFE is an extremely rare entity, with only three cases reported from our subcontinent. As a result, it is easily underdiagnosed or misdiagnosed; clinician awareness of this condition is critical for better diagnosis and management.
肺胸膜弹力纤维增生症（PPFE）是一种罕见疾病，其特征为胸膜和肺下弹力纤维增生，以肺上叶为主。我们报告了印度的第三例特发性 PPFE 病例，也是第二例生前诊断。一名 27 岁男性，因 1 年的改良医学研究委员会 II 级呼吸困难和干咳就诊。他体重减轻了 15 公斤。经过临床和影像学诊断后，他接受了抗结核治疗，并因无改善而被转诊。胸部高分辨率计算机断层扫描显示双侧肺上叶大疱、实质和肺下纤维化以及不规则胸膜增厚。在手术肺和胸膜活检中发现了 PPFE。他接受了全身糖皮质激素治疗，但在临床和影像学上均无反应。吡非尼酮和肺移植对他来说遥不可及。他在确诊后 9 个月死亡。最后，特发性 PPFE 是一种极其罕见的疾病，我们次大陆仅报告了三例。因此，它很容易被漏诊或误诊；临床医生对这种疾病的认识对于更好的诊断和管理至关重要。
