IgG4相關疾病的臨床表現─南臺灣某醫學中心的病歷回溯調查

Clinical characteristics of patients with IgG4-related disease: the experience of a medical center in southern Taiwan

目的：調查南臺灣某醫學中心IgG4相關疾病的病人，運用新的診斷標準來確認診斷，整理其臨床表現與器官侵犯。方法：我們收集院內於民國九十七年至一百零三年間，所有曾經接受過IgG4抽血檢查、或切片報告提及IgG4的病人，運用日本厚生勞動省於2011年公佈的診斷標準來確認診斷，對符合診斷病人進行病歷回溯調查。我們紀錄病人的基本資料、臨床表現、器官侵犯、血清IgG4與IgG數值、切片結果、以及其他全身性疾病，進行分析統計。結果：在運用診斷標準確認、並排除其他疾病後，共有32位符合IgG4相關疾病的診斷。其中18位為確定診斷、6位是高度可能診斷、8位是可能診斷。病人有28位（87.5%）為男性，平均診斷年齡為62.4 ± 16歲。最多的器官侵犯是肝膽胰臟疾病11位（34.4%），其次是唾腺侵犯7位（21.9%），其他器官侵犯有主動脈旁與後腹腔、肺部與肋膜、淚腺、皮膚、淋巴結。診斷時血清中IgG4的數值，在同器官侵犯的不同病人間有高度的差異；然而不同器官侵犯的組別之間，其平均血清IgG4數值並無法得到顯著的統計差異。僅有一成不到的病患由風濕科醫師診斷，而有接近一半的半人由風濕科醫師追蹤治療。結論：南臺灣IgG4相關疾病的病患與國外研究相比，有類似的器官侵犯分佈、與類似的發病性別年齡。「病理切片」符合典型病理表現是診斷IgG4相關疾病最重要的步驟；然而血清IgG4檢查是簡單又好的篩檢工具，且陽性的結果常引導醫師更進一步的檢查。由於IgG4相關疾病可侵犯多種不同的器官，因此不只風濕科醫師，其他不同領域的醫師也應對此病有基本認識，以期能達到早期診斷與更妥善的處置。

Objective: This study aims to collect patients with IgG4-related disease (IgG4-RD), validate their diagnosis by the updated diagnostic criteria, investigate clinical characteristics, and compare the data thus obtained with that from other studies, based on a cohort from a single hospital. Methods: We retrospectively reviewed all possible patients with IgG4-RD between June 2008 and October 2014, using laboratory and pathology databases. Diagnosis is made according to the comprehensive diagnostic criteria published in Japan in 2011. Serial serum levels of IgG4 and IgG, pathology reports, clinical presentations, other systemic illness, demographic data and medical services, were all recorded and analyzed. Results: A total of 144 patients were reviewed, of whom 142 had available serum IgG4 data and 31 had received biopsy or surgery. After evaluation, 32 patients fulfilled the diagnosis of IgG4-RD, including 18 with definite diagnosis, six with probable diagnosis, and eight with possible diagnosis. Mean age at diagnosis was 62.4 years, with male predominance (87.5%). Pancreatobiliary disease (n=11, 34.4%) was the most common organ involvement, followed by salivary gland (n=7, 21.9%), and other organs, including retroperitoneum, lung and pleura, skin, and lacrimal gland. Serum IgG4 levels were highly variable between each patient of the same organ involvement, but the mean levels in different groups of involved organ had no significant difference. Less than 10% of the overall patients saw a rheumatologist during their initial visit, but nearly half of the patients were followed by a rheumatologist. Conclusion: In our study IgG4-RD occurred more commonly in elderly males, with pancreatobiliary disease being the most common form. Pathological confirmation is the most important step for the diagnosis, whereas serum IgG4 is easy to perform and an elevated level usually suggests possible target patients, and that further examinations may be needed. IgG4-RD is a multidisciplinary disease, with many patients diagnosed or treated by non-rheumatologists. In order to achieve earlier diagnosis and better management, all related specialists should have some understanding of the disease.