{"id":8355,"date":"2021-01-22T21:57:51","date_gmt":"2021-01-22T21:57:51","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=8355"},"modified":"2026-01-06T20:46:09","modified_gmt":"2026-01-06T20:46:09","slug":"igg4-rkd","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/igg4-rkd\/","title":{"rendered":"Diagnostic Criteria for IgG4-Related Kidney Disease (IgG4-RKD)"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>IgG4-related disease is characterized by a high level of serum IgG4 and dense infiltration of IgG4-positive plasma cells into multiple organs, with the kidney being one representative target. Although several sets of <a href=\"https:\/\/medicalcriteria.com\/web\/autoimmune-pancreatitis\/\" target=\"_blank\" rel=\"noopener noreferrer\">diagnostic criteria for autoimmune pancreatitis (AIP)<\/a> are available and renal lesion is recognized as an extrapancreatic manifestation of AIP, it is difficult to differentiate IgG4-related tubulointerstitial nephritis (TIN) without<br \/>\nAIP from other types of TIN.<!--more--><\/p>\n<p><strong>Diagnostic criteria for IgG4-related kidney disease (IgG4-RKD)<\/strong><br \/>\n1. Presence of some kidney damage, as manifested by abnormal urinalysis or urine marker(s) or decreased kidney function with either elevated serum IgG level, hypocomplementemia, or elevated serum IgE level<br \/>\n2. Abnormal renal radiologic findings:<\/p>\n<p style=\"padding-left: 40px;\">a. Multiple low-density lesions on enhanced computed tomography<br \/>\nb. Diffuse kidney enlargement<br \/>\nc. Hypovascular solitary mass in the kidney<br \/>\nd. Hypertrophic lesion of renal pelvic wall without irregularity of the renal pelvic surface<\/p>\n<p>3. Elevated serum IgG4 level (IgG4 \u2265 135 mg\/dl)<br \/>\n4. Histologic findings in the kidney<\/p>\n<p style=\"padding-left: 40px;\">a. Dense lymphoplasmacytic infiltration with infiltrating IgG4-positive plasma cells &gt;10\/high power field (HPF) and\/or IgG4\/IgG-positive plasma cells &gt;40%<br \/>\nb. Characteristic fibrosis surrounding nests of lymphocytes and\/or plasma cells<\/p>\n<p>5. Histologic findings in extra-renal organ(s):<br \/>\nDense lymphoplasmacytic infiltration with infiltrating IgG4-positive plasma cells &gt;10\/HPF and\/or IgG4\/IgG-positive plasma cells &gt;40% in extra-renal organ(s)<\/p>\n<p>Definite: 1) + 3) + 4) a, b<br \/>\n2) + 3) + 4) a, b<br \/>\n2) + 3) + 5)<br \/>\n1) + 3) + 4) a + 5)<br \/>\nProbable: 1) + 4) a, b<br \/>\n2) + 4) a, b<br \/>\n2) + 5)<br \/>\n3) + 4) a, b<br \/>\nPossible: 1) + 3)<br \/>\n2) + 3)<br \/>\n1) + 4) a<br \/>\n2) + 4) a<\/p>\n<p>Appendix:<br \/>\n1. Clinically and histologically, the following diseases should be excluded: Wegener\u2019s granulomatosis, Churg\u2013Strauss syndrome, extramedullary plasmacytoma<br \/>\n2. Radiologically, the following diseases should be excluded: malignant lymphoma, urinary tract carcinomas, renal infarction and pyelonephritis (rarely, Wegener\u2019s granulomatosis, sarcoidosis and metastatic carcinoma)<br \/>\n3. Cases with suspected disease according to the diagnostic algorithm are classified into probable or possible IgG4-RKD according to these criteria<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Saeki T, Kawano M, Nagasawa T, Ubara Y, Taniguchi Y, Yanagita M, Nishi S, Nagata M, Hisano S, Yamaguchi Y, Nomura H, Saito T, Nakashima H. Validation of the diagnostic criteria for IgG4-related kidney disease (IgG4-RKD) 2011, and proposal of a new 2020 version. Clin Exp Nephrol. 2021 Jan 4. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/33398598\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Wallace ZS, Naden RP, Chari S, Choi HK, Della-Torre E, Dicaire JF, et al. The 2019 American College of Rheumatology\/European League against rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79(1):77\u201387. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/31796497\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Kawano M, Saeki T, Nakashima H, Nishi S, Yamaguchi Y, Hisano S, Yamanaka N, Inoue D, Yamamoto M, Takahashi H, Nomura H, Taguchi T, Umehara H, Makino H, Saito T. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011 Oct;15(5):615-626. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/21898030\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Jan 21, 2021.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[473],"tags":[16,15,14,13,51,50,2314,2308,2313,900,2309,1563,199],"class_list":["post-8355","post","type-post","status-publish","format-standard","hentry","category-nephrology","tag-criteria","tag-criterios","tag-diagnostic","tag-diagnostico","tag-disease","tag-enfermedad","tag-err-igg4","tag-igg4","tag-igg4-rkd","tag-kidney","tag-relacionada","tag-related","tag-renal"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.1 - 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