Department of Internal Medicine, Nagaoka Red Cross Hospital, Senshu 2-297-1, Nagaoka, Niigata, 940-2085, Japan.
Department of Rheumatology, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8641, Japan.
Clin Exp Nephrol. 2021 Feb;25(2):99-109. doi: 10.1007/s10157-020-01993-7. Epub 2021 Jan 4.
In 2011, the IgG4-related kidney disease (IgG4-RKD) working group of the Japanese Society of Nephrology proposed diagnostic criteria for IgG4-RKD. The aim of the present study was to validate those criteria and develop a revised version.
Between April 2012 and May 2019, we retrospectively collected Japanese patients with kidney disease, for whom data on serum IgG4 values and/or immunohistological staining for IgG4 in renal biopsy samples were available. These patients were classified as IgG4-RKD or non-IgG4-RKD based on the diagnostic criteria for IgG4-RKD 2011, and the results were evaluated by expert opinion. Accordingly, we developed some revised versions of the criteria, and the version showing the best performance in the present cohort was proposed as the IgG4-RKD criteria for 2020.
Of 105 included patients, the expert panel diagnosed 55 as having true IgG4-RKD and 50 as mimickers. The diagnostic criteria for IgG4-RKD 2011 had a sensitivity of 72.7% and a specificity of 90.0% in this cohort. Of the 15 patients with true IgG4-RKD who were classified as non-IgG4-RKD, all lacked biopsy-proven extra-renal lesions, although many had clinical findings highly suggestive of IgG4-RD. The revised version to which "bilateral lacrimal, submandibular or parotid swelling, imaging findings compatible with type 1 autoimmune pancreatitis or retroperitoneal fibrosis" was added as an item pertaining to extra-renal organ(s) improved the sensitivity to 90.9% while the specificity remained at 90.0%.
The revised version has considerably improved test performance after addition of the new extra-renal organ item (imaging and clinical findings).
2011 年，日本肾脏病学会 IgG4 相关肾病（IgG4-RKD）工作组提出了 IgG4-RKD 的诊断标准。本研究旨在验证这些标准并开发修订版。
2012 年 4 月至 2019 年 5 月，我们回顾性收集了日本肾脏病患者的资料，这些患者的血清 IgG4 值和/或肾活检标本中 IgG4 的免疫组织化学染色数据可用。这些患者根据 2011 年 IgG4-RKD 诊断标准分为 IgG4-RKD 或非 IgG4-RKD，并由专家意见进行评估。因此，我们开发了一些标准的修订版，并提出在本队列中表现最佳的版本作为 2020 年的 IgG4-RKD 标准。
在 105 例纳入患者中，专家组诊断 55 例为真正的 IgG4-RKD，50 例为模拟病例。该标准在本队列中的敏感性为 72.7%，特异性为 90.0%。在被归类为非 IgG4-RKD 的 15 例真正 IgG4-RKD 患者中，所有患者均缺乏经活检证实的肾外病变，尽管许多患者具有高度提示 IgG4-RD 的临床发现。修订版中增加了“双侧泪腺、颌下腺或腮腺肿胀、与 1 型自身免疫性胰腺炎或腹膜后纤维化相符的影像学表现”作为与肾外器官相关的项目，敏感性提高到 90.9%，而特异性仍为 90.0%。
在增加新的肾外器官项目（影像学和临床发现）后，修订版的测试性能有了显著提高。
