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A case of Podocytic Infolding Glomerulopathy with SLE and literature review
Xi Liu1, Jieli Huang1, Kun Zhang1
1The Department of Nephrology, Tongji Hospital, Tongji University School of Medicine, Shanghai, China.
Background:
Podocytic infolding glomerulopathy (PIG) is a rare pathological change which was characterized by the microspheres or microtubular structures in the thickened glomerular basement membrane (GBM). Only a few dozen cases have been reported worldwide so far. Here we present a case of PIG with systemic lupus erythematosus.
Case Presentation:
A 61-year-old Chinese female was diagnosed with systemic lupus erythematosus with clinical manifestations of proteinuria, pleural effusion, seroperitoneum, anemia, leukopenia, thrombocytopenia, antinuclear antibody positive, and hypocomplementemia. She also had benign ovarian tumor and Epstein-Barr virus infection. Renal biopsy immunofluorescent staining showed IgM and C3 were granularly deposited along the capillary wall instead of typical "full house" features. Electron microscopy showed lots of microspheres structures were seen in the thickened GBM.
Conclusion:
We present a case of PIG in a patient with systemic lupus erythematosus. The mechanisms of PIG are unknown, but may be associated with connective tissue disease and podocyte injury.
Insights
Podocytic infolding glomerulopathy (PIG), a rare kidney disease characterized by microspheres in the glomerular basement membrane, is presented in a patient with systemic lupus erythematosus.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Podocytic infolding glomerulopathy (PIG) is a rare kidney disease characterized by unique microsphere or microtubular structures within the glomerular basement membrane (GBM).
- Few cases of PIG have been documented globally, highlighting its rarity.
Observation:
- A 61-year-old female with systemic lupus erythematosus presented with proteinuria, effusions, cytopenias, and positive autoimmune markers.
- Renal biopsy revealed granular IgM and C3 deposition, atypical for lupus nephritis.
- Electron microscopy identified numerous microsphere structures within a thickened GBM.
Findings:
- The case highlights a rare instance of podocytic infolding glomerulopathy (PIG) co-occurring with systemic lupus erythematosus (SLE).
- Distinctive microsphere structures within the GBM were observed, characteristic of PIG.
- Immunofluorescence showed non-specific granular deposition of IgM and C3, differing from typical lupus nephritis patterns.
Implications:
- This case expands the understanding of PIG's clinical spectrum, suggesting an association with connective tissue diseases like SLE.
- Further research into the pathogenesis of PIG is warranted, potentially involving podocyte injury mechanisms.
- The findings underscore the importance of comprehensive renal pathology evaluation in patients with SLE presenting with atypical renal manifestations.
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