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Published on: July 8, 2020
Clinical significance and long-term evolution of minimal change histopathologic variants and of IGM nephropathy among
A F Donia1, M A Sobh, F E Moustafa
1Urology and Nephrology Center, University of Mansoura, Egypt.
Abstract:
In children, the most frequent idiopathic nephrotic syndrome is minimal change nephrotic syndrome (MCNS). Typically, MCNS shows no abnormalities by light microscopy: "nil disease". Beside this classic picture, there are other minor light microscopic abnormalities which are considered as MCNS variants. Our 172 MCNS patients were divided into a nil disease group, two groups of MCNS variants (mild mesangial hypercellularity and mild mesangial thickening) and a fourth group with normal light microscopy and diffuse IgM deposition (IgM nephropathy group). The relation of this fourth group to MCNS is controversial in the literature. Age and serum creatinine were significantly different in the four histologic groups (P=0.03 for age and 0.047 for serum creatinine). Comparing the groups in pairs, it appeared that these significant differences were due to significantly higher age and serum creatinine in the mild mesangial hypercellularity group than in the IgM nephropathy group (P = 0.02 for age and 0.01 for serum creatinine). The groups were similar as regards follow-up creatinine clearance and early and late steroid response. We concluded that mild mesangial hypercellularity may differ from other MCNS forms as regards age at presentation and renal function. We also suggest that IgM nephropathy with normal light microscopy is similar to MCNS.
Insights
Minimal change nephrotic syndrome (MCNS) variants show distinct clinical features. Mild mesangial hypercellularity in MCNS differs in age and renal function, while IgM nephropathy appears similar to classic MCNS.
Area of Science:
- Pediatric Nephrology
- Renal Pathology
- Immunopathology
Background:
- Minimal change nephrotic syndrome (MCNS) is the most common idiopathic nephrotic syndrome in children.
- MCNS typically presents with normal light microscopy ('nil disease'), but variants with minor abnormalities exist.
- The classification and relationship of certain histological variants, like IgM nephropathy, to MCNS remain debated.
Purpose of the Study:
- To analyze clinical and histological differences among MCNS variants and IgM nephropathy.
- To investigate the relationship between histological findings and patient demographics/renal function.
- To compare treatment response across different MCNS subgroups.
Main Methods:
- Retrospective analysis of 172 pediatric MCNS patients.
- Classification into four groups: nil disease, mild mesangial hypercellularity, mild mesangial thickening, and IgM nephropathy.
- Statistical comparison of age, serum creatinine, creatinine clearance, and steroid response.
Main Results:
- Significant differences in age and serum creatinine were observed across the four histological groups.
- Mild mesangial hypercellularity group showed significantly higher age and serum creatinine compared to the IgM nephropathy group.
- All groups demonstrated similar follow-up creatinine clearance and steroid responsiveness.
Conclusions:
- Mild mesangial hypercellularity may represent a distinct MCNS variant regarding age at presentation and renal function.
- IgM nephropathy with normal light microscopy shares similarities with classic MCNS.
- Histological classification impacts certain clinical parameters in pediatric nephrotic syndrome.
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