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Post-infectious Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Deposits Associated with Complement
Eriko Takehara1, Shintaro Mandai, Satomi Shikuma
1Department of Nephrology, Shuuwa General Hospital, Japan.
Internal Medicine (Tokyo, Japan)
|April 7, 2017
Summary
A rare kidney disease, infection-associated glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID), can mimic post-infectious glomerulonephritis (PIGN). A complement factor H mutation may predispose individuals to this severe condition.
Area of Science:
- Nephrology
- Immunology
- Genetics
Background:
- Infection-associated glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) is a rare kidney disease.
- Distinguishing PGNMID from post-infectious glomerulonephritis (PIGN) can be challenging due to overlapping clinical presentations.
Observation:
- A 55-year-old man presented with rapidly progressive glomerulonephritis and nephrotic syndrome.
- Kidney biopsy revealed diffuse proliferative and crescentic glomerulonephritis with specific immune deposits (monoclonal IgG1κ, humps, nephritis-associated plasmin receptor).
- Despite initial dialysis-dependent renal failure, the patient experienced spontaneous renal function recovery.
Findings:
- The patient was diagnosed with PGNMID, which mimicked PIGN.
- Genetic testing identified a heterozygous complement factor H mutation.
- This mutation may cause persistent complement pathway activation following infection, leading to severe glomerulonephritis.
Implications:
- PGNMID and PIGN may share underlying pathogenetic mechanisms involving the complement system.
- Identifying complement gene mutations is crucial for understanding and potentially managing severe glomerulonephritis.
- This case highlights the importance of comprehensive genetic testing in atypical presentations of glomerulonephritis.