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

  • Ting Zhang
  • Wenjia Sun
  • Jing Xue
  • Jiaxi Chen
  • Qifeng Jiang
  • Lijun MouEmail author
  • Hengjian DuEmail author
Case Based Review
  • 12 Downloads

Abstract

Podocytic infolding glomerulopathy (PIG) is a newly proposed disease entity, and only 29 cases have been reported worldwide so far, characterized by microspheres or microtubular structures or both associated with podocytic infolding into the glomerular basement membrane (GBM) on electron microscopy. We present two new cases of PIG with connective tissue disease (CTD), one with primary Sjögren’s syndrome and the other with systemic lupus erythematosus (SLE), and make a systemic review of the literature. In the entire 31 patients of PIG, 24 (77.42%) were women and seven (22.58%) were men, with an average age of 41.2 ± 15.2 (ranging from 14 to 79) years old. Almost two-thirds of patients (67.74%) were diagnosed with CTD, in which 76.19% were SLE. All patients presented with proteinuria and six (19.35%) patients were accompanied with hematuria. Serum creatinine was elevated in six (19.35%) patients. Pathological findings of all patients were consistent with PIG characteristics, and four patients with repeated renal biopsies further provided profound insights.

Keywords

Podocytic infolding glomerulopathy Sjögren’s syndrome Systemic lupus erythematosus 

Notes

Funding

The authors received financial support for the research, authorship, and/or publication of this article from Zhejiang Province Public Welfare Technology Application Research Project (Award Number 2017C33093).

Compliance with ethical standards

Disclosures

None.

Ethics

The case report was approved by the Research Ethics Committee of The Second Affiliated Hospital of Zhejiang University, School of Medicine, and informed consents were obtained in both patients.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Division of RheumatologyThe Second Affiliated Hospital of Zhejiang University, School of MedicineHangzhouPeople’s Republic of China
  2. 2.Division of NephrologyThe Second Affiliated Hospital of Zhejiang University, School of MedicineHangzhouPeople’s Republic of China
  3. 3.Division of Renal pathologyGuangzhou Kingmed Diagnostic Laboratory LtdGuangzhouPeople’s Republic of China
  4. 4.Division of Geriatric Infectious DiseaseSichuan Academy of Medical Sciences & Sichuan Provincial People’s HospitalChengduPeople’s Republic of China

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