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Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification
Sanjeev Sethi1, Fernando C Fervenza
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA. sethi.sanjeev@mayo.edu
Seminars in Nephrology
|August 16, 2011
Summary
Membranoproliferative glomerulonephritis (MPGN) can be classified into Ig-mediated or complement-mediated types. This classification guides diagnostic workups for infections, autoimmune diseases, or complement alternative pathway dysregulation.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Membranoproliferative glomerulonephritis (MPGN) is characterized by immune deposits in the kidney glomeruli.
- Common causes include chronic infections, autoimmune diseases, and monoclonal gammopathies leading to Ig-mediated MPGN.
- MPGN can also stem from complement dysregulation, particularly the alternative pathway (AP), causing complement-mediated MPGN.
Purpose of the Study:
- To propose a new histologic classification for MPGN.
- To differentiate between Ig-mediated and complement-mediated MPGN.
- To guide appropriate diagnostic workups based on the MPGN classification.
Main Methods:
- Histologic classification of MPGN into two major groups: Ig-mediated and complement-mediated.
- Review of underlying causes and diagnostic approaches for each type.
- Recommendation of specific diagnostic tests for complement-mediated MPGN.
Main Results:
- MPGN is categorized into Ig-mediated and complement-mediated forms.
- Ig-mediated MPGN necessitates investigation for infections, autoimmune diseases, and monoclonal gammopathies.
- Complement-mediated MPGN requires evaluation of the alternative complement pathway.
Conclusions:
- A simplified classification of MPGN into Ig-mediated and complement-mediated is proposed.
- This classification aids in directing clinical investigations.
- Complement-mediated MPGN diagnosis involves screening for alternative pathway dysregulation, including genetic mutations and autoantibodies.
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