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Cyclophosphamide for the Treatment of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposition
Sabaa Asif1, Sumbal Nasir Mahmood1, Kunwer Naveed Mukhtar2
1Department of Nephrology, Ziauddin University Hospital, Karachi, Pakistan.
Abstract:
About 3% of the population aged more than 50 years, is affected by monoclonal gammopathy of undetermined significance (MGUS), a premalignant condition that may progress to lymphoproliferative disorders. Since MGUS does not represent the diseases associated with end organ damage, a new term, monoclonal gammopathy of renal significance (MGRS) is coined for the monoclonal gammopathies that are associated with renal disorders. MGRS is classified into various types, including monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID). PGNMID presents with membranoproliferative glomerulonephritis-type lesions associated with immunoglobulin deposition. This disease entity has a poor prognosis and its optimum treatment is yet to be established. We present the case of an elderly male, a known patient of light chain deposition disease, a form of MIDD, who initially lost to follow-up but later presented with PGNMID, wherein he was treated with steroid and cyclophosphamide, to which he responded.
Insights
Monoclonal gammopathy of renal significance (MGRS) encompasses kidney-related disorders. A case of proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID) showed positive response to steroid and cyclophosphamide treatment.
Area of Science:
- Nephrology
- Hematology
- Immunology
Background:
- Monoclonal gammopathy of undetermined significance (MGUS) affects over 3% of individuals over 50 and can progress to lymphoproliferative disorders.
- Monoclonal gammopathies associated with renal disorders are now termed monoclonal gammopathy of renal significance (MGRS).
- MGRS includes conditions like monoclonal immunoglobulin deposition disease (MIDD) and proliferative glomerulonephritis with monoclonal immunoglobulin deposition (PGNMID).
Observation:
- PGNMID is characterized by membranoproliferative glomerulonephritis-like lesions and immunoglobulin deposition.
- This condition has a poor prognosis, and optimal treatment strategies are still under investigation.
- A case study involved an elderly male with a history of light chain deposition disease (a form of MIDD).
Findings:
- The patient, initially lost to follow-up, later presented with PGNMID.
- Treatment with a combination of steroid and cyclophosphamide was administered.
- The patient demonstrated a positive response to the applied treatment regimen.
Implications:
- This case highlights a potential therapeutic approach for PGNMID, a severe renal disorder.
- Further research into MGRS and its subtypes is crucial for improving patient outcomes.
- Early recognition and management of MGRS may prevent irreversible kidney damage.
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