Nikolic Sara, Brehmer Katharina, Panic Nikola, Valente Roberto, Löhr J-Matthias, Vujasinovic Miroslav
Department of Medicine, Huddinge, Karolinska Institute, 141 86 Stockholm, Sweden.
Department of Radiology, Karolinska University Hospital, 141 86 Stockholm, Sweden.
J Clin Med. 2020 Feb 3;9(2):409. doi: 10.3390/jcm9020409.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised pathologically by the infiltration of IgG4-bearing plasma cells into the involved organs. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis with a heavy lymphocytic infiltration and two distinct histopathological subtypes, namely: lymphoplasmacytic sclerosing pancreatitis (AIP type 1) and idiopathic duct-centric pancreatitis (AIP type 2). Lung involvement and aortic involvement have been reported in 12% and 9% of patients with systemic IgG4-RD, respectively. In series including patients with AIP, both lung and aortic involvement were described in 2% of the patients. Most of the epidemiological data come from Japan, and there is a lack of information from Europe, especially the Scandinavian countries.
We performed a single-centre retrospective study on a prospectively collected cohort of patients diagnosed with AIP at the Department for Digestive Diseases at Karolinska University Hospital in Stockholm, Sweden, from 2004 to 2019. Demographic and clinical data were collected from the medical charts.
One hundred and thirty-three patients with AIP were analysed. Six patients were excluded because they lacked some of the clinical data relevant to the study. Demographic and clinical features of 127 patients were presented. There were 98 patients with AIP type 1-35 (35.7%) female and 63 (64.3%) male, with a mean age of 55.4 ± 18.2. Among them, 15 (15.3%) patients had lung and/or cardiovascular involvement-11 (11.2%) patients had lung involvement, 10 (10.2%) patients had cardiovascular involvement (six patients had both). Most of them (67.0%) had never smoked. The mean follow-up time of the patients with AIP type 1 was 49 months.
Lung and/or cardiovascular involvement were diagnosed in 15 (15.3%) patients in our historical cohort of patients with AIP type 1. Most of the lung involvement was presented in the form of nodular lesions in the lungs, non-specific infiltrates, "ground-glass" appearance with pleura thickening, and effusion. Aortic involvement was a major form of vascular involvement in patients with AIP, as in previous published studies on patients with IgG4-RD.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种全身性免疫介导性疾病，其病理特征为携带IgG4的浆细胞浸润至受累器官。自身免疫性胰腺炎（AIP）是一种慢性胰腺炎，伴有大量淋巴细胞浸润，有两种不同的组织病理学亚型，即：淋巴细胞浆细胞硬化性胰腺炎（AIP 1型）和特发性导管中心性胰腺炎（AIP 2型）。据报道，在系统性IgG4-RD患者中，肺部受累和主动脉受累的发生率分别为12%和9%。在纳入AIP患者的系列研究中，2%的患者同时出现肺部和主动脉受累。大多数流行病学数据来自日本，欧洲尤其是斯堪的纳维亚国家缺乏相关信息。
我们对瑞典斯德哥尔摩卡罗林斯卡大学医院消化内科2004年至2019年期间前瞻性收集的确诊为AIP的患者队列进行了单中心回顾性研究。从病历中收集人口统计学和临床数据。
对133例AIP患者进行了分析。6例患者因缺乏一些与研究相关的临床数据而被排除。呈现了127例患者的人口统计学和临床特征。有98例AIP 1型患者，其中女性35例（35.7%），男性63例（64.3%），平均年龄55.4±18.2岁。其中，15例（15.3%）患者有肺部和/或心血管系统受累，11例（11.2%）患者有肺部受累，10例（10.2%）患者有心血管系统受累（6例患者两者均有）。他们中的大多数（67.0%）从不吸烟。AIP 1型患者的平均随访时间为49个月。
在我们的AIP 1型患者历史队列中，15例（15.3%）患者被诊断为肺部和/或心血管系统受累。大多数肺部受累表现为肺部结节性病变、非特异性浸润、伴有胸膜增厚的“磨玻璃”样外观及胸腔积液。与先前发表的关于IgG4-RD患者的研究一样，主动脉受累是AIP患者血管受累的主要形式。
