medicina58巻10号

2021年09月発行

特集 腎疾患—エキスパートへの質問で学ぶ診療のキホンと最新情報 膠原病

IgG4関連疾患の診断と最新の治療について教えてください

著者： 水島伊知郎¹ 川野充弘¹

所属機関： ¹金沢大学附属病院リウマチ・膠原病内科

ページ範囲：P.1572 - P.1578

Point
◎IgG4関連疾患（IgG4-RD）は，多くの臓器を標的とし，高度な免疫細胞浸潤かつ/もしくは線維化により臓器機能障害をきたす慢性全身性疾患である．
◎IgG4-RDでは，唾液腺，涙腺・眼窩，膵臓，胆道，後腹膜・動脈周囲，肺などに加え，腎臓も主要な罹患臓器であり，IgG4関連腎臓病（IgG4-RKD）と呼ばれる．
◎IgG4-RKDの診断は，臨床像，血清所見，画像所見，病理所見などを総合的に勘案して行う必要があり，わが国のIgG4-RKD診断基準や，ACR/EULAR分類基準を用いる．
◎IgG4-RKDはステロイド治療に良好に反応するが，治療前の腎機能障害の改善は一定程度にとどまり，慢性の機能障害を遺しうる．

参考文献

1）Umehara H, et al：A novel clinical entity, IgG4-related disease（IgG4RD）；General concept and details. Mod Rheumatol 22：1-14, 2012

2）Kawano M, et al：Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol 15：615-626, 2011

3）Saeki T, et al：Validation of the diagnostic criteria for IgG4-related kidney disease（IgG4-RKD）2011, and proposal of a new 2020 version. Clin Exp Nephrol 25：99-109, 2021

4）Yamada K, et al：New clues to the nature of immunoglobulin G4-related disease；A retrospective Japanese multicenter study of baseline clinical features of 334 cases. Arthritis Res Ther 19：262, 2017

5）Saeki T, et al：Clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney Int 78：1016-1023, 2010

6）Raissian Y, et al：Diagnosis of IgG4-related tubulointerstitial nephritis. J Am Soc Nephrol 22：1343-1352, 2011

7）Kasashima S, et al：Inflammatory features, including symptoms, increased serum interleukin-6, and C-reactive protein, in IgG4-related vascular diseases. Heart Vessels 33：1471-1481, 2018

8）Strehl JD, et al：Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol 64：237-243, 2011

9）水島伊知郎，他：Ⅰ-10腎疾患の鑑別．川　茂幸，川野充弘（編）：IgG4関連疾患　実践的臨床から病因へ—IgG4研究会モノグラフ，pp 78-88，前田書店，2015

10）Yamamoto M, et al：Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases. Mod Rheumatol 22：419-425, 2012

11）Houghton DC, et al：An abundance of IgG4＋ plasma cells is not specific for IgG4-related tubulointerstitial nephritis. Mod Pathol 24：1480-1487, 2011

12）Wallace ZS, et al：The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis 79：77-87, 2020

13）Mizushima I, et al：Positive disease-specific autoantibodies have limited clinical significance in diagnosing IgG4-related disease in daily clinical practice. Rheumatology（Oxford）, 2020［Online ahead of print］

14）Saeki T, et al：The clinical course of patients with IgG4-related kidney disease. Kidney Int 84：826-833, 2013

15）Saeki T, et al：Recovery of renal function after glucocorticoid therapy for IgG4-related kidney disease with renal dysfunction. Clin Exp Nephrol 20：87-93, 2016

16）Mizushima I, et al：Factors related to renal cortical atrophy development after glucocorticoid therapy in IgG4-related kidney disease；A retrospective multicenter study. Arthritis Res Ther 18：273, 2016

17）Shirakashi M, et al：Factors in glucocorticoid regimens associated with treatment response and relapses of IgG4-related disease；A multicentre study. Sci Rep 8：10262, 2018

18）Carruthers MN, et al：Rituximab for IgG4-related disease；A prospective, open-label trial. Ann Rheum Dis 74：1171-1177, 2015

19）Ebbo M, et al：A Long-term efficacy and safety of rituximab in IgG4-related disease；Data from a French nationwide study of thirty-three patients. PLoS One 12：e0183844, 2017