Department of Pulmonary Medicine, Nippon Medical School Musashikosugi Hospital, Kawasaki-shi, Japan.
Department of Pulmonary Medicine, Nippon Medical School Musashikosugi Hospital, 1-383 Kosugi-machi, Nakahara-ku, Kawasaki-shi 211-8533, Japan.
Ther Adv Respir Dis. 2023 Jan-Dec;17:17534666231158279. doi: 10.1177/17534666231158279.
Sarcoidosis is a multisystem disease with an unknown etiology and is characterized by the formation of noncaseating granulomas in the affected organs. We present the case of a 69-year-old male Japanese patient with bilateral hilar lymphadenopathy on chest radiographs for more than 10 years, left without further investigation. The patient reported no clinical symptoms. Chest computed tomography revealed ground-glass opacities and reticular shadows in both lungs, along with bilateral hilar and mediastinal lymphadenopathy. Lymphocytosis was observed in bronchoalveolar lavage fluid. Pathological examination of transbronchial lung biopsy revealed noncaseating, epithelioid granulomas congruous with sarcoidosis, together with other findings. There were no abnormalities on electrocardiogram, echocardiogram, and ophthalmic examination.For progressive dyspnea on exertion, systemic corticosteroid therapy with oral prednisolone (25 mg/day) was initiated in 2017 and gradually tapered. Despite this intervention, the decline in forced vital capacity (FVC) was accelerated. Three years later, the patient noticed swelling in his right wrist. Further investigation revealed elevated anti-cyclic citrullinated peptide antibodies and absence of noncaseating epithelioid granuloma on surgical biopsy, leading to the diagnosis of rheumatoid arthritis (RA). Thereafter, the anti-fibrotic agent nintedanib was initiated, because interstitial lung disease (ILD) was considered to have converted into a progressive fibrosing phenotype (PF-ILD) with overlapping RA-associated lung involvement. With treatment, the progression of decline in FVC was slowed, although home oxygen therapy was introduced.
结节病是一种病因不明的多系统疾病，其特征是受累器官形成非干酪样肉芽肿。我们报告了一例 69 岁的日本男性患者，其胸部 X 线片显示双侧肺门淋巴结肿大超过 10 年，左侧未进一步检查。患者无临床症状。胸部计算机断层扫描显示双肺磨玻璃影和网状影，伴双侧肺门和纵隔淋巴结肿大。支气管肺泡灌洗液中淋巴细胞增多。经支气管肺活检的病理检查显示非干酪样、上皮样肉芽肿与结节病一致，同时还存在其他发现。心电图、超声心动图和眼科检查均无异常。由于进行性活动后呼吸困难，2017 年开始给予全身皮质类固醇治疗，口服泼尼松（25mg/天），并逐渐减量。尽管进行了干预，但用力肺活量（FVC）的下降加速。三年后，患者注意到右手腕肿胀。进一步检查显示抗环瓜氨酸肽抗体升高，手术活检未见非干酪样上皮样肉芽肿，诊断为类风湿关节炎（RA）。此后，开始使用抗纤维化药物尼达尼布，因为间质性肺病（ILD）被认为已转化为具有重叠 RA 相关肺受累的进行性纤维化表型（PF-ILD）。尽管开始了治疗，但 FVC 下降的进展仍有所减缓，尽管引入了家庭氧疗。
