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Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case-control study based on

Qiaozhu Zeng1, Jingyuan Gao2, Xinyu Zhang3

  • 1Department of Rheumatology and Immunology, Peking University People's Hospital, 11, Xizhimen South Street, Beijing, 100044, China.

Scientific Reports
|May 18, 2021
PubMed

Insights

This study compared kidney involvement in IgG4-related disease (IgG4-RD). IgG4-related kidney disease (IgG4-RKD+) patients were older, more frequently male, and often had multi-organ involvement, suggesting distinct disease mechanisms.

Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition.
  • Kidney involvement, known as IgG4-related kidney disease (IgG4-RKD+), is a significant manifestation.
  • Understanding the distinct characteristics of IgG4-RKD+ is crucial for diagnosis and management.

Purpose of the Study:

  • To compare demographic, clinical, and laboratory features of IgG4-related kidney disease (IgG4-RKD+) versus extrarenal IgG4-related disease (IgG4-RKD-).
  • To describe the radiological and pathological characteristics specific to IgG4-RKD+.
  • To identify risk factors associated with kidney involvement in IgG4-RD patients.

Main Methods:

  • Retrospective analysis of 450 IgG4-RD patients from a large Chinese cohort (2004-2020).
  • Comparison of demographic, clinical, laboratory, radiological, and pathological data between IgG4-RKD+ (n=53) and IgG4-RKD- groups.
  • Exclusion of IgG4-RD patients with other causes of renal impairment.

Main Results:

  • IgG4-RKD+ patients presented at older ages with a higher male-to-female ratio.
  • Commonly involved organs in IgG4-RKD+ were salivary glands, lymph nodes, and pancreas.
  • Renal function impairment occurred in ~40% of IgG4-RKD+ patients; common imaging showed bilateral hypodense lesions.
  • Male sex, multi-organ involvement (>3), and low serum C3 were risk factors for IgG4-RKD+.

Conclusions:

  • IgG4-related kidney disease (IgG4-RKD+) exhibits distinct demographic and clinical features compared to extrarenal IgG4-RD.
  • Specific risk factors like male sex, extensive organ involvement, and low C3 levels are associated with IgG4-RKD+.
  • These findings suggest potential differences in the underlying pathogenesis between IgG4-RKD+ and IgG4-RKD- phenotypes.

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