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Distinct impact of malignancy and allergy on the clinical and immunological features of IgG4-related disease

1, 2, 3, 4, 5, 6, 7, 8, 9

 

  1. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
  2. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
  3. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
  4. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
  5. Research Unit/Immunology and Inflammation Sohyaku, Mitsubishi Tanabe Parma Corporation, Kanagawa, Japan.
  6. Research Unit/Immunology and Inflammation Sohyaku, Mitsubishi Tanabe Parma Corporation, Kanagawa, Japan.
  7. Research Unit/Immunology and Inflammation Sohyaku, Mitsubishi Tanabe Parma Corporation, Kanagawa, Japan.
  8. Research Unit/Immunology and Inflammation Sohyaku, Mitsubishi Tanabe Parma Corporation, Kanagawa, Japan.
  9. Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan. ykaneko.z6@keio.jp

CER16282
2023 Vol.41, N°9
PI 1754, PF 1761
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PMID: 36719757 [PubMed]

Received: 14/10/2022
Accepted : 21/11/2022
In Press: 31/01/2023
Published: 17/08/2023

Abstract

OBJECTIVES:
To clarify the clinical and immunological characteristics of IgG4-RD based on the underlying diseases.
METHODS:
Consecutive patients with IgG4-RD treated at Keio University Hospital between 2010 and 2021 were divided according to the presence of malignancy or allergy into three groups. The clinical characteristics and 56 immune cell subsets in the peripheral blood were compared among the groups.
RESULTS:
Among 123 patients, 18 (14.6%) had malignancy including 4 with allergy (malignancy group), 57 (46.3%) had allergy alone (allergy group), and 48 (39.0%) had neither (idiopathic group). In the malignancy group, the patients were older (70.1 vs. 54.4 vs. 64.9 years, p<0.001), male-dominant (83.3 vs. 42.1 vs. 54.2%, p=0.008), and had smoking habits (77.8 vs. 42.1 vs. 43.8%, p=0.02). They also had significant involvement of the aorta/large vessels (33.3 vs. 7.0 vs. 20.8%, p=0.02), while the patients in the allergy group tended to have orbital/lacrimal gland involvement. Remission and relapse rates were not different between the groups; however, overall survival was significantly poorer in the malignancy group (p=0.02). Comprehensive immunophenotyping of the peripheral blood revealed that the increase in CXCR5+CD2-double negative T cells and the decrease in naive CD8 T cells were characteristic of the malignancy group.
CONCLUSIONS:
The clinical and immunological phenotypes of IgG4-RD differ among those with underlying diseases.

DOI: https://doi.org/10.55563/clinexprheumatol/7g2na0

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