Sugino Keishi, Ono Hirotaka, Saito Mikako, Igarashi Seiji, Kurosaki Atsuko, Tsuboi Eiyasu
Department of Respiratory Medicine Tsuboi Hospital Koriyama City Japan.
Department of Diagnostic Pathology Tsuboi Hospital Koriyama City Japan.
Respirol Case Rep. 2022 Mar 8;10(4):e0925. doi: 10.1002/rcr2.925. eCollection 2022 Apr.
A 79-year-old former smoking Japanese man was admitted to our hospital with a 2-year history of dry cough and dyspnoea on exertion. High-resolution computed tomography of the chest revealed reticulation and perilobular opacity with bronchial wall thickening and ground-glass opacities (GGOs) in both lungs, in addition to subpleural dense consolidation (pleuroparenchymal fibroelastosis-like lesion; PPFE-like lesion) predominantly in the bilateral upper lobes. Serum immunoglobulin G4 (IgG4) was elevated (348 mg/dl). Lung biopsy specimens obtained by video-assisted surgery revealed a mixture of usual interstitial pneumonia (IP) and non-specific IP pattern admixed with PPFE. In addition, immunohistochemical staining of IgG4 showed numerous IgG4-positive plasma cells. Consequently, he was diagnosed with IgG4-positive IP associated with PPFE. We initiated a combination therapy with prednisolone and cyclosporine as a calcineurin inhibitor. During prednisolone tapering, his clinical conditions and GGOs improved gradually over 12 months. However, reticular opacities and PPFE-like lesions remained unchanged, and pulmonary function test findings slightly deteriorated.
一名79岁有吸烟史的日本男性因干咳和劳力性呼吸困难2年入住我院。胸部高分辨率计算机断层扫描显示双肺有网状影和小叶周围模糊影，伴有支气管壁增厚和磨玻璃影（GGO），此外双侧上叶主要有胸膜下致密实变（胸膜实质纤维弹性样病变；PPFE样病变）。血清免疫球蛋白G4（IgG4）升高（348mg/dl）。通过电视辅助手术获取的肺活检标本显示普通间质性肺炎（IP）和非特异性IP模式与PPFE混合存在。此外，IgG4免疫组化染色显示大量IgG4阳性浆细胞。因此，他被诊断为与PPFE相关的IgG4阳性IP。我们开始使用泼尼松龙和作为钙调神经磷酸酶抑制剂的环孢素进行联合治疗。在泼尼松龙减量期间，他的临床症状和GGO在12个月内逐渐改善。然而，网状影和PPFE样病变保持不变，肺功能测试结果略有恶化。
