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Detection of MicroRNA Expression in the Kidneys of Immunoglobulin A Nephropathic Mice
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[Clinicopathological characteristics and prognosis of IgG4-related kidney disease]

T Su1, S S Shi2, H Wang3

  • 1Department of Nephrology, Peking University First Hospital, Beijing 100034, China.

Zhonghua Yi Xue Za Zhi
|August 18, 2021
PubMed

Insights

IgG4-related kidney disease (IgG4-RKD) often presents with elevated IgG4 and low C3 levels. Kidney biopsy is crucial for diagnosis, and immunosuppressive therapy improves renal function in most patients.

Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • IgG4-related kidney disease (IgG4-RKD) is an emerging entity characterized by IgG4-positive plasma cell infiltration.
  • Clinical and pathological features of IgG4-RKD can be diverse, often mimicking other glomerulopathies.

Purpose of the Study:

  • To analyze the clinical characteristics, pathological findings, and treatment outcomes of patients with biopsy-proven IgG4-RKD.
  • To highlight the importance of kidney biopsy in diagnosing IgG4-RKD and its concurrent renal pathologies.

Main Methods:

  • Retrospective analysis of clinical data from 18 patients diagnosed with IgG4-RKD.
  • Review of pathological examinations including tubulointerstitial nephritis and glomerulonephritis patterns.
  • Assessment of treatment responses to immunosuppressive therapy and patient follow-up.

Main Results:

  • Elevated serum IgG4 levels and hypocomplementemia (low C3) were common findings.
  • Acute kidney injury was present in 9 cases; pathological findings included IgG4-associated tubulointerstitial nephritis and various forms of glomerulonephritis.
  • Patients with elevated erythrocyte sedimentation rate showed higher acute tubulointerstitial injury scores.
  • Fifteen patients showed improved renal function with immunosuppressive therapy, though 3 experienced relapse.

Conclusions:

  • IgG4-RKD frequently involves concurrent glomerulopathy, underscoring the complexity of its presentation.
  • Kidney biopsy is essential for accurate diagnosis and management of IgG4-RKD.
  • Immunosuppressive therapy is effective but requires careful monitoring due to potential relapses.

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