alexa Orbital IgG4 Related Disease (IgG4RD): Incidence and Accompanying Histological Features Using the Latest Consensus Criteria
ISSN: 2157-7099

Journal of Cytology & Histology
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Research Article

Orbital IgG4 Related Disease (IgG4RD): Incidence and Accompanying Histological Features Using the Latest Consensus Criteria

Anita SY Chan1,2,3, Sunny Shen2,3, Audrey Looi Lee Geok2,3, Seah Lay Leng2,3, Leonard Tan Hwan Cheong1 and Issam Al Jajeh1*


1Histopathology, Pathology Department, Singapore General Hospital, 20 College Road, Academia, Level, 10, Diagnostics Tower, Singapore

2Singapore National Eye Centre, 11 Third Hospital Avenue, Singapore

3Singapore Eye Research Institute, 11 Third Hospital Avenue, Singapore

*Corresponding Author:
Issam Al Jajeh
Histopathology, Pathology Department
Singapore General Hospital, 20 College Road
Academia, Level 10, Diagnostics Tower, Singapore 169856
Tel: 65-6321-4875
Fax: 65-6227-6562
E-mail: [email protected]

Received Date: September 16, 2014; Accepted Date: October 16, 2014; Published Date: October 18, 2014

Citation: Chan ASY, Shen S, Geok ALL, Leng SL, Cheong LTH, et al. (2014) Orbital IgG4 Related Disease (IgG4RD): Incidence and Accompanying Histological Features Using the Latest Consensus Criteria. J Cytol Histol 5:286. doi:10.4172/2157-7099.1000286

Copyright: © 2014 Chan ASY, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.



Purpose: Orbital Immunoglobulin 4 (IgG4) related disease (IgG4RD) is a fibro-inflammatory condition that mimics sclerosing orbital inflammatory disease (OID). The recently published IgG4RD consensus criteria included its defining histological features. Using the published criteria, this study aims to describe the frequency of orbital IgG4RD and its histological features in OID biopsies over a 1 year period.
Method: Thirty-seven consecutive orbital biopsies for OID over 1 year were prospectively examined for the features of fibrosis, inflammation, and vasculitis. Immunohistochemistry (IHC) evaluation was performed when significant fibrosis and/or lymphoplasmacytic inflammation ( >25% of the biopsy section) was present.
Results: Ten of 37 (27%) orbital biopsies showed significant fibrosis and/ or lymphoplasmacytic inflammation with the remaining cases showing only non-specific chronic inflammation or reactive lymphoid hyperplasia. Only 3 cases (30%) fulfilled the IgG4RD consensus criteria. The histological patterns included sclerosing dacroadenitis, sclerosing xanthogranulomatous orbital inflammation, and eosinophilic angiocentric fibrosis. Storiform fibrosis was the most common histological feature present (70%), followed by dense lymphoplasmacytic inflammation (60%). When both are present, an almost 2-fold elevation of tissue IgG4 plasma cells and ratio above the diagnostic cut-off was detected. Xanthogranulomatous inflammation and eosinophilia were occasionally present.
Conclusions: Using the consensus criteria, IgG4RD was diagnosed in 30% of our orbital biopsies with significant fibrosis and/ or inflammation and 11% of all OID biopsied in 1 year. Although the storiform fibrosis and lymphplasmacytic inflammation was most commonly seen, associated eosinophilia and xanthogranulomatous inflammation may also be seen in IgG4RD.

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