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Published on: August 23, 2024
Segmental membranous nephropathy
Hae Yoon Grace Choung1, Bruce Goldman2
1Department of Pathology and Laboratory Medicine, Division of Renal Pathology and Electron Microscopy Laboratory, University of Rochester Medical Center, 601 Elmwood Ave, Box 626, Rochester, NY, 14642, USA. haeyoon_choung@urmc.rochester.edu.
Abstract:
Most cases of membranous nephropathy (MGN) present with global and diffuse distribution of subepithelial deposits. However, segmental MGN, in which there is focal or diffuse segmental subepithelial deposits, are occasionally encountered. The clinical and pathologic significance of segmental MGN is not well understood and thought to be more likely due to either early or resolving phase of the global form of MGN. Several case reports and literature available suggest that it may be a manifestation of secondary causes based on pathologic features such as presence of C1q and mesangial deposits, extra-glomerular deposits involving tubular basement membranes, absence of PLA2R and THSD7A, IgG1 and IgG3 subclass dominance, and the presence of other co-existing renal disease. Other reports, however, suggest that some of these cases may be a variant of the primary form of MGN. In this review, we integrate what is known about segmental MGN in order to better direct interpretation of renal biopsies in which it is identified.
Insights
Segmental membranous nephropathy (MGN) presents focal deposits, unlike typical diffuse MGN. Its significance is unclear, possibly indicating early/resolving disease or secondary causes, impacting renal biopsy interpretation.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Membranous nephropathy (MGN) typically shows global subepithelial deposits.
- Segmental MGN, with focal segmental deposits, is less common and its significance is debated.
- It may represent an early or resolving phase of primary MGN or a manifestation of secondary causes.
Purpose of the Study:
- To review and integrate current knowledge on segmental MGN.
- To aid in the interpretation of renal biopsies showing segmental MGN.
- To clarify the clinical and pathological significance of segmental MGN.
Main Methods:
- Literature review and synthesis of case reports and existing studies on segmental MGN.
- Analysis of pathological features associated with segmental MGN, including immune deposits and subclass dominance.
- Integration of clinical and pathological data to differentiate primary vs. secondary causes.
Main Results:
- Segmental MGN may be associated with secondary causes, indicated by C1q and mesangial deposits, extra-glomerular deposits, absence of PLA2R/THSD7A, and IgG1/IgG3 dominance.
- Some cases might represent a variant of primary MGN.
- Pathological features are key to distinguishing potential etiologies.
Conclusions:
- Segmental MGN's interpretation requires careful consideration of specific pathological findings.
- Distinguishing between primary and secondary causes is crucial for patient management.
- Further research is needed to fully elucidate the clinical significance of segmental MGN.
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