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C3 glomerulonephritis associated with monoclonal gammopathy of renal significance: case report
Juana Alonso-Titos1, Lara Perea-Ortega1, Eugenia Sola1
1Nephrology Department, Carlos Haya Regional University Hospital, University of Malaga, IBIMA, REDinREN (RD16/0009/0006), Avda. Carlos Haya s/n, E-29010, Malaga, Spain.
Insights
Monoclonal gammopathy of renal significance (MGRS) involves kidney damage and systemic issues. Early diagnosis and chemotherapy are crucial for managing this condition and preserving renal function.
Area of Science:
- Nephrology
- Immunology
- Pathology
Background:
- Monoclonal gammopathy of renal significance (MGRS) presents significant morbidity due to severe renal lesions and systemic complications.
- Early diagnosis and targeted chemotherapy to inhibit immunoglobulin production are essential for managing MGRS.
Observation:
- A 75-year-old male with chronic kidney disease experienced rapid renal function decline.
- Kidney biopsy revealed C3 glomerulonephritis with exclusive C3 deposits and a membranoproliferative pattern.
- Skin biopsy demonstrated endothelial complement deposition, supporting MGRS diagnosis.
Findings:
- The patient's condition was attributed to the alternative complement pathway activation by an anomalous immunoglobulin.
- Despite treatment with plasmapheresis, bortezomib, and steroids, advanced chronic kidney disease progressed.
Implications:
- Monoclonal gammopathy should be considered in adult patients presenting with membranoproliferative glomerulonephritis.
- This case highlights the complex interplay between monoclonal proteins, complement activation, and renal pathology in MGRS.
Background:
Morbidity associated with monoclonal gammopathy of renal significance is high due to the severe renal lesions and the associated systemic alterations. Accordingly, early diagnosis is fundamental, as is stopping the clonal production of immunoglobulins using specific chemotherapy.
Case Presentation:
A 75-year-old man with chronic renal disease of unknown origin since 2010 experienced rapid worsening of renal function over a period of 6 mos. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy showed the presence of C3 glomerulonephritis, with exclusive deposits of C3 visible on immunofluorescence and a membranoproliferative pattern on light microscopy. Skin biopsy showed endothelial deposition of complement. Given both the renal and cutaneous involvement the patient was considered to have monoclonal gammopathy of renal significance. We considered an underlying pathogenic mechanism for the renal alteration secondary to activation of the alternative complement pathway by the anomalous immunoglobulin. Despite treatment with plasmapheresis, bortezomib and steroids, advanced chronic kidney disease developed.
Conclusions:
The possible underlying cause of the monoclonal gammopathy of renal significance suggests that monoclonal gammopathy should be considered in adult patients with membranoproliferative glomerulonephritis.
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