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Abstract: TH-PO669

Podocytic Infolding Glomerulopathy: A Systematic Report of 44 Cases

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

  • Shen, Zhirang, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China
  • Zhang, Changrong, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China
  • Zhuang, Jing, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China
  • Qu, Yue, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China
  • Tian, Xuefei, Yale University School of Medicine, New Haven, Connecticut, United States
  • Jiang, Hong, People's Hospital of Xinjiang Uygur Autonomous Region, Urumqi, Xinjiang, China
Background

Podocytic infolding glomerulopathy (PIG), a newly recognized rare disease entity with unique histopathological features characterized by podocytic cytoplasmic invagination into the glomerular basement membrane (GBM) and presenting as "microsphere" or "microtubule"-like structures visualized under a transmission electron microscope, has attracted increasing attention in recent years. Since PIG was first reported in 1965, the clinical features and pathogenesis of PIG are still poorly understood. Delving into the understanding of PIG, elucidating its etiology, clinical implications, and optimal treatment strategies is needed.

Methods

A total of 1713 kidney biopsies from January 2020 to April 2024 in our kidney center were retrospectively analyzed for PIG screening. The diagnostic criteria for PIG are as follows: Electron microscopy shows microspheres or microtubules associated with podocyte cytoplasm folded into the GBM , along with a complete medical record. Forty-four patients were enrolled in the study.

Results

We reviewed 44 PIG patients, of whom 16 were female (36.4%) and 28 were male (63.6%), with an average age of 40±15.9 years. The median [Min, Max] of 24-hour urine protein quantitation was 2.14[0.0280,8.32]g/24h. The median [Min, Max] of Serum creatinine was 84.0[41.6,688]μmol/l. 43.2% of PIG patients had IgA nephropathy. The pathological results of all patients were consistent with PIG features.

Conclusion

Most PIG patients are complicated with immune system-mediated kidney diseases, such as IgA nephropathy, IgA vasculitis associated nephritis, ANCA-associated glomerulonephritis, etc. However, the pathogenic mechanism of PIG is unknown and needs further study.

Funding

  • Government Support – Non-U.S.