Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Nov 17;35(3):193-197.
doi: 10.4103/SJOPT.SJOPT_91_21. eCollection 2021 Jul-Sep.

Plasma cell IgG4 positivity in orbital biopsies of non-IgG4-related conditions

Antonio A V Cruz  1 , Maria A B Camacho  1 , Barbara S Cunha  1 , Hind M Alkatan  2   3 , Naiara F Xavier  1
Affiliations

Plasma cell IgG4 positivity in orbital biopsies of non-IgG4-related conditions

Antonio A V Cruz et al. Saudi J Ophthalmol. .

Abstract

The IgG4-related disease (IgG4-RD) is a systemic condition defined as a fibro-inflammatory disorder, characterized by the occurrence of tumor-like lesions in multiple organs including the eye adnexa. The main diagnostic criterion is based on histopathological findings, especially on the IgG4+/IgG+ plasma cell ratio. In this article, we reviewed the literature of non-IgG4-RD orbital conditions with IgG4 positivity. There were 20 reports of inflammatory non-IgG4-RD orbital lesions and 14 reports of orbital lymphoid proliferations with significant IgG4 positivity. The role of plasma cells IgG4 in the pathogenesis of non-IgG4-RD is not clear. Considering the large spectrum of diseases caused by a variety of different etiopathogenic mechanisms, we think that the common denominator of IgG4+ in these conditions might be related to the peculiar properties of down regulation of immune response of the IgG4 and not to a specific link to IgG4-RD.

Keywords: IgG4; IgG4-related disease; IgG4/IgG ratio; RosaiDorfman disease; orbit.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

References

    1. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8. - PubMed
    1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366:539–51. - PubMed
    1. Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92. - PubMed
    1. Yamamoto M, Hashimoto M, Takahashi H, Shinomura Y. IgG4 disease. J Neuroophthalmol. 2014;34:393–9. - PubMed
    1. Yun J, Wienholt L, Adelstein S. Poor positive predictive value of serum immunoglobulin G4 concentrations in the diagnosis of immunoglobulin G4-related sclerosing disease. Asia Pac Allergy. 2014;4:172–6. - PMC - PubMed