IgG4-related disease: dataset of 235 consecutive patients.
IgG4-related disease: dataset of 235 consecutive patients.
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DOI：
10.1097/md.0000000000000680
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发表时间：
2015-04
期刊：
影响因子：
1.6
通讯作者：
Zen Y
中科院分区：
文献类型：
--
作者：
Inoue D;Yoshida K;Yoneda N;Ozaki K;Matsubara T;Nagai K;Okumura K;Toshima F;Toyama J;Minami T;Matsui O;Gabata T;Zen Y
免疫球蛋白G4相关性疾病（IgG4-RD）是最近发现的一种全身性疾病，其中各种器官表现与相似的组织学外观相关。我们对这种情况的了解仍然是零散的，因为大多数研究只检查了几十名患者或集中在特定的器官表现上。本研究使用大型队列研究IgG4-RD的人口统计学和患者特征。通过检索机构的放射学数据库，在同一医疗区的8家综合医院诊断出的235例连续IgG4-RD患者中，共确定了235例。入选标准为根据病理学共识声明经组织学证实的IgG4-RD和/或符合国际共识诊断标准的明确1型自身免疫性胰腺炎。对选定患者的临床记录和图像进行回顾性分析。所有患者均为成人（M/F = 4/1）。 中位年龄为67岁（范围35 - 86岁）。十分之九的人在50到70岁时被诊断出来。在总共确定的486种表现中，最常见的是142名患者（60%）诊断为胰腺炎，其次是涎腺炎（34%），肾小管间质性肾炎（23%），泪腺炎（23%）和牙周炎（20%）。大多数患者（95%）至少有5种最常见表现之一。男性和女性患者的器官表现不同（牙周炎在男性中更常见，涎腺泪腺炎在女性中更常见）。208例患者（88%）血清IgG 4（正常≤ 135 mg/dL）升高至> 135 mg/dL，167例患者（71%）升高至> 270 mg/dL。 多器官受累患者的IgG 4值显著高于单一表现患者（中位数629 mg/dL vs 299 mg/dL，P <0.01）。 在218名患者中，IgG4和IgG值均可用，194名（89%）的IgG4/IgG比值升高至> 10%。皮质类固醇是有效的，但复发率估计为24%，在研究期间（中位数37个月）。在随访期间，13例患者（6%）诊断出15种恶性疾病。这一数字与日本全国癌症流行病学研究预期的发病率（12.9例癌症）相似（标准化发病率比1.16）。总之，这一可靠的数据集可以改善IgG4-RD的表征，特别是其独特的人口统计学和每个器官表现的频率。
Immunoglobulin G4-related disease (IgG4-RD) is a recently discovered systemic condition, in which various organ manifestations are linked by a similar histological appearance. Our knowledge of this condition is still fragmented, as most studies have examined only a few dozen patients or focused on a particular organ manifestation. This study was conducted to learn the demography and patient characteristics of IgG4-RD using a large cohort. A total of 235 consecutive patients with IgG4-RD, diagnosed in 8 general hospitals in the same medical district, were identified by searching the institutions’ radiology database. Inclusion criteria were histology-proven IgG4-RD according to the Pathology Consensus Statement and/or definitive type 1 autoimmune pancreatitis meeting the International Consensus Diagnostic Criteria. Clinical notes and images of selected patients were retrospectively reviewed. All patients were adults (M/F = 4/1). The median age was 67 years (range 35–86). Nine tenths were diagnosed in their 50s to 70s. Among 486 manifestations identified in total, the most common was pancreatitis diagnosed in 142 patients (60%), followed by sialadenitis (34%), tubulointerstitial nephritis (23%), dacryoadenitis (23%), and periaortitis (20%). The majority of patients (95%) had at least 1 of the 5 most common manifestations. Male and female patients differed in their organ manifestations (periaortitis more common in males and sialodacryoadenitis more common in females). Serum IgG4 (normal ≤135 mg/dL) was elevated to >135 mg/dL in 208 patients (88%) and >270 mg/dL in 167 (71%). The IgG4 value was significantly higher in patients with multiorgan involvement than in those with a single manifestation (median 629 mg/dL vs 299 mg/dL, P < 0.01). Of 218 patients, for whom both IgG4 and IgG values were available, the IgG4/IgG ratio was raised to >10% in 194 (89%). Corticosteroids were effective, but the relapse rate was estimated to be 24% in the study period (median 37 months). During the follow-up, 15 malignant diseases were diagnosed in 13 patients (6%). This figure is similar to the incidence (12.9 cancers) expected from the Japanese nationwide study for cancer epidemiology (standardized incidence ratio 1.16). In conclusion, this reliable dataset could improve the characterization of IgG4-RD, particularly its unique demography and the frequency of each organ manifestation.