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Podocytic infolding glomerulopathy: two new cases with connective tissue disease and literature review

Ting Zhang1, Wenjia Sun1, Jing Xue1

  • 1Division of Rheumatology, The Second Affiliated Hospital of Zhejiang University, School of Medicine, No.88, Jiefang Road, Shangcheng District, Hangzhou, 310005, Zhejiang, People's Republic of China.

Clinical Rheumatology
|March 18, 2019
PubMed

Insights

Podocytic infolding glomerulopathy (PIG), a rare kidney disease, often affects women and is linked to connective tissue diseases like lupus. Electron microscopy reveals characteristic podocyte changes within the glomerular basement membrane.

Area of Science:

  • Nephrology
  • Pathology
  • Rheumatology

Background:

  • Podocytic infolding glomerulopathy (PIG) is a recently identified kidney disease.
  • Characterized by specific ultrastructural podocyte abnormalities on electron microscopy.
  • Few cases reported globally, highlighting its rarity.

Observation:

  • Presents two new cases of PIG associated with connective tissue diseases (CTD): primary Sjögren's syndrome and systemic lupus erythematosus (SLE).
  • Systemic literature review of all reported PIG cases (total 31 patients).
  • Majority of patients (77.42%) are women, with a mean age of 41.2 years.

Findings:

  • Two-thirds of PIG patients (67.74%) have associated CTD, predominantly SLE (76.19% of CTD cases).
  • All patients exhibit proteinuria; some have hematuria (19.35%) or elevated serum creatinine (19.35%).
  • Pathological findings confirm PIG characteristics, with repeated biopsies offering deeper insights.

Implications:

  • Highlights a significant association between PIG and CTD, particularly SLE.
  • Emphasizes the importance of considering PIG in patients with proteinuria and CTD.
  • Further research into PIG pathogenesis and management is warranted, especially in the context of autoimmune diseases.
Keywords:
Podocytic infolding glomerulopathySjögren’s syndromeSystemic lupus erythematosus

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