Ito M, Yasuo M, Yamamoto H, Tsushima K, Tanabe T, Yokoyama T, Hamano H, Kawa S, Uehara T, Honda T, Kawakami S, Kubo K
First Department of Internal Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Eur Respir J. 2009 Mar;33(3):680-3. doi: 10.1183/09031936.00051408.
Autoimmune pancreatitis is a unique form of chronic pancreatitis characterised by a high-serum immunoglobulin (Ig)G4 concentration involving various extra pancreatic lesions. A 63-yr-old female with autoimmune pancreatitis complained of cough. Chest computed tomography revealed an irregular stenosis of the central airway, lung hilar and mediastinal lymph node swelling, and a marked thickness of the bronchovascular bundle. Bronchoscopic examination revealed an irregular tracheobronchial stenosis accompanied with an oedematous mucosa and engorged vessels. Lung hilar and mediastinal lymph node swelling, central airway stenosis and bronchoscopic findings remarkably resembled those of sarcoidosis. Bronchial biopsy specimens demonstrated diffuse infiltrations of plasma cells, lymphocytes and eosinophils with fibrosis. Immunostaining showed infiltration of several IgG4-positive plasma cells. The patient was treated with oral prednisolone at 1 mg x kg(-1) x day(-1) for pancreatic lesions. A month later, the lung lesions, including central airway stenosis, lung hilar and mediastinal lymph node swelling, and bronchovascular bundle thickness, had dramatically improved along with improvement of pancreatitis, thus indicating a close association between the two conditions. This is the first report of a patient with autoimmune pancreatitis showing central airway stenosis similar to that of sarcoidosis.
自身免疫性胰腺炎是一种独特的慢性胰腺炎形式，其特征为血清免疫球蛋白（Ig）G4浓度升高，并累及各种胰腺外病变。一名63岁患有自身免疫性胰腺炎的女性主诉咳嗽。胸部计算机断层扫描显示中央气道不规则狭窄、肺门和纵隔淋巴结肿大以及支气管血管束明显增厚。支气管镜检查显示气管支气管不规则狭窄，伴有黏膜水肿和血管充血。肺门和纵隔淋巴结肿大、中央气道狭窄以及支气管镜检查结果与结节病极为相似。支气管活检标本显示浆细胞、淋巴细胞和嗜酸性粒细胞弥漫浸润并伴有纤维化。免疫染色显示有多个IgG4阳性浆细胞浸润。该患者针对胰腺病变接受了口服泼尼松龙治疗，剂量为1 mg×kg⁻¹×天⁻¹。一个月后，包括中央气道狭窄、肺门和纵隔淋巴结肿大以及支气管血管束增厚在内的肺部病变，随着胰腺炎的改善而显著改善，这表明两种病症之间存在密切关联。这是首例自身免疫性胰腺炎患者出现类似于结节病的中央气道狭窄的报告。
