Authors: Yamada, K.¹; Kawano, M.; Inoue, R.¹; Hamano, R.¹; Kakuchi, Y.¹; Fujii, H.¹; Matsumura, M.¹; Zen, Y.²; Takahira, M.³; Yachie, A.⁴; Yamagishi, M.¹

Source: Clinical & Experimental Immunology, Volume 152, Number 3, June 2008 , pp. 432-439(8)

Publisher: Wiley-Blackwell

Abstract:

Summary

Mikulicz's disease (MD) is gaining acceptance as an immunoglobulin G4 (IgG4)-related disease characterized by bilateral lacrimal and salivary gland swelling. The aetiology of MD and other IgG4-related diseases is still unclear. The present work was performed to study the clonality of infiltrating IgG4-positive plasma cells in lacrimal glands and circulating peripheral blood cells in patients with MD, and compare the clonal relationship between infiltrating and circulating IgG4 positive cells. Total cellular RNA was extracted from the lacrimal glands and peripheral blood in five MD patients. Reverse transcription polymerase chain reaction was performed with primers specific for activation-induced cytidine deaminase (AID) and for Ig VH and IgG4. Sequences of Ig VH were compared with the structure of Ig VH of the lacrimal glands and the peripheral blood cells. AID was expressed to varying degrees in lacrimal glands of all MD patients. Most IgG4-positive cells infiltrating lacrimal glands and in peripheral blood were polyclonal, although several clonally related pairs were detected. In one patient, two of the circulating IgG4 VH4-59 clones shared identical CDR3 sequences with the clones within the lacrimal glands. In conclusion, while most tissue-infiltrating and circulating IgG4-positive cells in MD are polyclonal, some clonally related IgG4 positive cells exist between lacrimal gland and peripheral blood, accounting for the clinical features of MD as an IgG4-related disease involving multiple organs.

Keywords: activation-induced cytidine deaminase; IgG4; immunoglobulin; Milulicz's disease; somatic hypermutation

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1365-2249.2008.03651.x

Affiliations: 1: Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, 2: Division of Pathology, Kanazawa University Hospital, 3: Department of Ophthalmology, Graduate School of Medical Science, Kanazawa University, and 4: Department of Laboratory Sciences, School of Health Sciences, Faculty of Medicine, Kanazawa University, Kanazawa, Japan

Publication date: 2008-06-01

Related content

• In this: publication
• By this: publisher
• In this Subject: Microbiology ,  Allergy & Immunology
• By this author: Yamada, K. ;  Kawano, M. ;  Inoue, R. ;  Hamano, R. ;  Kakuchi, Y. ;  Fujii, H. ;  Matsumura, M. ;  Zen, Y. ;  Takahira, M. ;  Yachie, A. ;  Yamagishi, M.

Website © Publishing Technology. Article copyright remains with the publisher, society or author(s) as specified within the article.

• Terms and conditions
• Privacy policy
• Information for advertisers