Department of Respiratory Medicine, Tokyo Women's Medical University School of Medicine, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Division of Pathological Neuroscience, Department of Pathology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.
BMC Pulm Med. 2020 Mar 30;20(1):77. doi: 10.1186/s12890-020-1117-y.
Sarcoidosis is a systemic granulomatous disease caused by CD4+ cell-dominant inflammation. Meanwhile, diffuse panbronchiolitis is a chronic inflammatory respiratory disease predominantly caused by CD8+ lymphocytes and neutrophils. Herein, we report a rare case of sarcoidosis in which the clinical presentation had become evident as diffuse panbronchiolitis after splenectomy for sarcoidosis.
A 23-year-old Japanese woman was referred to our hospital due to splenomegaly of unknown etiology. Upon admission, chest computed tomography scan revealed centrilobular and randomly distributed small nodules in both lungs. Bronchoalveolar lavage revealed a high proportion of lymphocytes and a decreased CD4/CD8 ratio. However, the biopsy specimens obtained from both the liver and lungs revealed noncaseating epithelioid granulomas, which confirmed the diagnosis of sarcoidosis. The patient underwent splenectomy due to progressive cytopenia and high risk of splenic rupture. After the surgery, the condition of the patient was consistently good for 3 months. Then, she gradually developed productive cough and dyspnea. Both sinus and chest computed tomography scan revealed chronic paranasal sinusitis and deterioration of centrilobular nodules in both lung fields, respectively. The second bronchoalveolar lavage revealed a high proportion of neutrophils, and the bronchoalveolar lavage fluid tested positive for Hemophilus influenzae. The titer of cold agglutinin was elevated, thereby confirming the diagnosis of diffuse panbronchiolitis. On the basis of the clinical and radiological findings, the condition of the patient improved with low-dose macrolide therapy for 3 months.
The coexistence of sarcoidosis and diffuse panbronchiolitis has not been previously reported, and the hidden profiles of diffuse panbronchiolitis may have been revealed by splenectomy.
结节病是一种由 CD4+细胞主导的炎症引起的系统性肉芽肿性疾病。同时，弥漫性泛细支气管炎是一种主要由 CD8+淋巴细胞和中性粒细胞引起的慢性炎症性呼吸道疾病。在此，我们报告一例罕见的结节病病例，该病例在因结节病行脾切除术后，临床表现明显为弥漫性泛细支气管炎。
一名 23 岁的日本女性因原因不明的脾肿大被转诊至我院。入院时，胸部计算机断层扫描显示双肺有中轴和随机分布的小结节。支气管肺泡灌洗液显示淋巴细胞比例高，CD4/CD8 比值降低。然而，从肝脏和肺部获得的活检标本均显示为非干酪样上皮样肉芽肿，这证实了结节病的诊断。由于进行性血细胞减少症和脾破裂的高风险，患者接受了脾切除术。手术后，患者的病情持续 3 个月良好。然后，她逐渐出现咳痰和呼吸困难。鼻窦和胸部计算机断层扫描分别显示慢性副鼻窦炎和双肺中轴结节恶化。第二次支气管肺泡灌洗液显示中性粒细胞比例高，支气管肺泡灌洗液检测到流感嗜血杆菌阳性。冷凝集素滴度升高，从而确诊为弥漫性泛细支气管炎。基于临床和影像学表现，患者在接受低剂量大环内酯类药物治疗 3 个月后病情改善。
结节病和弥漫性泛细支气管炎同时存在尚未见报道，弥漫性泛细支气管炎的隐匿表现可能因脾切除而显露。
