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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2016  |  Volume : 27  |  Issue : 3  |  Page : 622-623
Remarks about the study on immunoglobulin G4-related disease: Two case reports and review of the recent literature

Department of Medicine, Division of Rheumatology, The University of Jordan, Amman, Jordan

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Date of Web Publication13-May-2016

How to cite this article:
Adwan MH. Remarks about the study on immunoglobulin G4-related disease: Two case reports and review of the recent literature . Saudi J Kidney Dis Transpl 2016;27:622-3

How to cite this URL:
Adwan MH. Remarks about the study on immunoglobulin G4-related disease: Two case reports and review of the recent literature . Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2022 Jan 16];27:622-3. Available from: https://www.sjkdt.org/text.asp?2016/27/3/622/182446
To the Editor,

We read with great interest the article by Anandh et al entitled "immunoglobulin G4- (IgG 4)-related disease (IgG4-RD): two case reports and review of the recent literature."[1]We would like to add a few important points and share our experience with this rather elusive condition. As a matter of fact, IgG4- RD is an old disease under a new name; or rather a new collective name for a group of previously known and presumed unrelated conditions, which now come under the um- brella name "IgG4-RD." These conditions in- clude tubulointerstitial nephritis and retroperi- toneal fibrosis as exemplified by the two cases presented by Anandh et al.[1]

Other manifestations of IgG4-RD include se- veral conditions which physicians have encoun- tered for many years such as autoimmune pancreatitis,[2]Mikulicz's syndrome,[3]Kuttner's tumor (chronic sclerosing sialadenitis),[4]Riedel's thyroiditis,[5]multifocal fibrosclerosis,[5]eosino- philic angiocentric fibrosis,[6]retroperitoneal fibrosis, aortitis, periaortitis and inflammatory aortic aneurysm,[7]as well as inflammatory pseudotumor. The latter can affect various organs including the orbit,[8]liver,[9]heart,[10]breast,[11]uterus,[12]colon,[13]bone,[14]kidney,[15]and ureter.[16]More recently, other manifestations are being recognized such as hypophysitis,[17]pachymeningitis,[18]and idiopathic cervical fib- rosis.[19]Many other new sites are increasingly being reported and it is likely that almost any organ or tissue in the body could turn out be involved.

Perhaps IgG4RD deserves to be called the great mimicker of the 21st century as it may affect any organ and present to virtually any specialty, including medicine, surgery, ophthal- mology, and even gynecology as can be seen from its truly protean manifestations.

Our own experience thus far is mainly of patients with orbital pseudotumors who res- ponded well to therapy with corticosteroids. However, as the disease may present with fea- tures of established diagnostic entities, it needs to be kept in the differential diagnosis of autoimmune systemic diseases and specifically looked for whenever there may be a diagnostic perplexity.

It is likely that some cases around the world in the past may have been misdiagnosed as granulomatosis with polyangiitis (previously Wegener's granulomatosis) (orbital mass) or Sjogren's syndrome (salivary gland involve- ment) or various other conditions that may present in a similar fashion. With the avai- lability of modern diagnostic techniques inclu- ding IgG4 immunostaining and Immunoglobu- lin subclass measurement, misdiagnosis becomes rather rare and the disease will hopefully become increasingly identified. However, aware- ness of this newly described disease entity and its various manifestations among physicians and postulating it as a diagnostic possibility is important for such a wish to materialize. Unfortunately, IgG4 immunostaining is still not widely available in many centers.

   References Top

Anandh U, Virarkar M, Shah A, Hastak M. Immunoglobin G4-related disease: Two case reports and review of the recent literature. Saudi J Kidney Dis Transpl 2015;26:1282-8.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Kamisawa T, Okamoto A. Autoimmune pan- creatitis: Proposal of IgG4-related sclerosing disease. J Gastroenterol 2006;41:613-25.  Back to cited text no. 2
Yamamoto M, Takahashi H, Ohara M, et al. A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006;16:335-40.  Back to cited text no. 3
Geyer JT, Ferry JA, Harris NL, et al. Chronic sclerosing sialadenitis (Küttner tumor) is an IgG4-associated disease. Am J Surg Pathol 2010;34:202-10.  Back to cited text no. 4
Dahlgren M, Khosroshahi A, Nielsen GP, Deshpande V, Stone JH. Riedel's thyroiditis and multifocal fibrosclerosis are part of the IgG4-related systemic disease spectrum. Arthritis Care Res (Hoboken). 2010;62(9): 1312-8.  Back to cited text no. 5
Deshpande V, Khosroshahi A, Nielsen GP, Hamilos DL, Stone JH. Eosinophilic angio- centric fibrosis is a form of IgG4-related sys- temic disease. Am J Surg Pathol 2011;35:701-6.  Back to cited text no. 6
Stone JR. Aortitis, periaortitis, and retroperi- toneal fibrosis, as manifestations of IgG4- related systemic disease. Curr Opin Rheumatol 2011;23:88-94.  Back to cited text no. 7
Oles K, Szczepanski W, Skladzien J, et al. IgG4-related inflammatory orbital pseudo- tumors - A retrospective case series. Folia Neuropathol 2015;53:111-20.  Back to cited text no. 8
Ahn KS, Kang KJ, Kim YH, et al. Inflam- matory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4-positi- vity and its clinical significance. J Hepato- biliary Pancreat Sci 2012;19:405-12.  Back to cited text no. 9
Ishizaka N. IgG4-related inflammatory pseu- dotumor in the heart. Methodist Debakey Cardiovasc J 2014;10:58.  Back to cited text no. 10
Moriya T, Hirakawa H, Nagashima M, Yasuda M, Kimijima I. IgG4-related disease of the breast: A systemic disease whose mammary manifestations mimic breast cancer. Int Canc Conf J 2015;4:67-72.  Back to cited text no. 11
Ohkubo H, Miyazaki M, Oguri T, Arakawa A, Kobashi Y, Niimi A. A rare case of IgG4- related disease involving the uterus. Rheuma- tology (Oxford) 2015;54:1124-5.  Back to cited text no. 12
Malik SM, Raina A, Hartman DJ. Immuno- globulin G4-related pseudotumor presenting as metastatic colon cancer. Clin Gastroenterol Hepatol 2015;13:e1-2.  Back to cited text no. 13
Terauchi R, Shirai T, Mizoshiri N, et al. Sub- periosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report. Mod Rheumatol 2014;:1-3.  Back to cited text no. 14
Inoue K, Okubo T, Kato T, et al. IgG4-related stomach muscle lesion with a renal pseudo- tumor and multiple renal rim-like lesions: A rare manifestation of IgG4-related disease. Mod Rheumatol 2015;:1-5.  Back to cited text no. 15
Moriarty MA, Dahmoush L, Nepple KG. IgG4 related disease of the ureter (inflammatory pseudotumor). J Urol 2014;191:1126-7.  Back to cited text no. 16
Leporati P, Landek-Salgado MA, Lupi I, Chiovato L, Caturegli P. IgG4-related hypo- physitis: A new addition to the hypophysitis spectrum. J Clin Endocrinol Metab 2011;96: 1971-80.  Back to cited text no. 17
Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis: A previously unrecognized form of central ner- vous system involvement in IgG4-related scle- rosing disease. Am J Surg Pathol 2009;33: 1249-52.  Back to cited text no. 18
Cheuk W, Tam FK, Chan AN, et al. Idiopathic cervical fibrosis - A new member of IgG4- related sclerosing diseases: Report of 4 cases, 1 complicated by composite lymphoma. Am J Surg Pathol 2010;34:1678-85.  Back to cited text no. 19

Correspondence Address:
Dr. Marwan H Adwan
Department of Medicine, Division of Rheumatology, The University of Jordan, Amman
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DOI: 10.4103/1319-2442.182446

PMID: 27215266

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