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C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic
Aishwarya Ravindran1, Fernando C Fervenza2, Richard J H Smith3
1Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.
Mayo Clinic Proceedings
|August 6, 2018
Summary
C3 glomerulopathy is a complex kidney disease involving complement abnormalities. While often progressive, treatment with immunosuppressants shows variable outcomes, highlighting the need for further research into effective therapies.
Area of Science:
- Nephrology
- Immunology
- Genetics
Background:
- C3 glomerulopathy (C3G) is a rare kidney disease characterized by complement system dysregulation.
- Understanding the clinicopathological spectrum and outcomes of C3G is crucial for patient management.
Purpose of the Study:
- To delineate the clinical, pathological, and genetic features of C3 glomerulopathy.
- To investigate the triggers, treatment strategies, and long-term outcomes in a cohort of C3G patients.
Main Methods:
- Retrospective analysis of 114 C3G patients diagnosed at Mayo Clinic between 2007 and 2016.
- Evaluation of clinicopathological data, complement levels, genetic variants, and treatment responses.
Main Results:
- The study identified diverse triggers including infection, autoimmune findings, and monoclonal gammopathy (MIg), with MIg more prevalent in older patients.
- Low C3 levels were observed in 44.6% of patients, and genetic variants in complement genes were present in 37.1%.
- While most patients received immunosuppressive therapy, outcomes varied, with 9.2% progressing to end-stage renal disease.
Conclusions:
- C3 glomerulopathy is a heterogeneous condition with complex alternative complement pathway abnormalities and varied triggers.
- The disease course is often progressive, and response to immunosuppressive therapy is inconsistent, necessitating personalized treatment approaches.