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Published on: January 18, 2019
A "Mini-Epidemic" of anti-glomerular basement membrane disease: Clinical and epidemiological study
Umesh Lingaraj1, Shivaprasad Sasivehalli Mallappa1, Rajiv Elkal Neminah1
1Department of Nephrology, Institute of Nephrourology, Victoria Hospital Campus, Bengaluru, Karnataka, India.
Abstract:
Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM) antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute. All cases were analyzed for demographic and clinical profile, pathological findings, treatment received and for any common environmental antigenic source. A total of 11 new biopsy proven anti-GBM cases were seen within a span of three months. Age group varied from 17-80 years. Seven were males and four were females. All were dialysis dependent at presentation. Seven had active cellular crescents, and four had fibrocellular. Only one patient was a smoker and none had a history of exposure to any forms of hydrocarbons. The peak seen from January 2015 to March 2015 does not correlate with any of seasonal occurrence of infections in southern India. Although there was clustering of cases to southern territories of Karnataka state, no common etiological agents could be identified. No patient had any previous urological surgeries. All patients received methylprednisolone with plasmapheresis 5-7 sessions and cyclophosphamide. All 11 patients were dialysis dependent at the end of three months. We conclude anti-GBM disease cannot be regarded as a rare cause of renal failure and lung hemorrhage. The occurrence of such epidemic within a short period suggests a possible unidentified environmental factor like infection or occupational agents as inciting agents. Identification of such inciting agents could help us in instituting appropriate preventing measures.
Insights
Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM) antibody disease, though rare, presented as a cluster of 11 cases. This suggests a potential unidentified environmental factor may trigger this serious kidney disease.
Area of Science:
- Nephrology
- Immunology
- Epidemiology
Background:
- Anti-glomerular basement membrane (anti-GBM) antibody disease is a rare cause of acute glomerulonephritis and rapidly progressive glomerulonephritis.
- Cases presenting in a cluster are exceptionally uncommon, prompting investigation into potential shared etiological factors.
Purpose of the Study:
- To analyze a cluster of biopsy-proven anti-GBM disease cases occurring within a three-month period.
- To investigate demographic, clinical, and pathological profiles for common environmental or occupational triggers.
- To assess treatment outcomes and identify potential preventative measures.
Main Methods:
- Retrospective analysis of 11 biopsy-proven anti-GBM disease cases from January to March 2015.
- Evaluation of patient demographics, clinical presentation, dialysis dependency, pathological findings (crescents), and treatment regimens.
- Investigation for common environmental exposures, including infections and hydrocarbons, and geographical clustering.
Main Results:
- Eleven cases of anti-GBM disease were identified within three months, a rare clustering.
- Patients presented with severe kidney injury, requiring dialysis, with varied crescent types.
- No clear link to smoking, hydrocarbon exposure, or seasonal infections was found, despite geographical clustering in Karnataka.
Conclusions:
- The observed clustering of anti-GBM disease suggests a possible unidentified environmental factor, such as infection or occupational agents.
- Anti-GBM disease may not be as rare a cause of renal failure and lung hemorrhage as previously thought.
- Identifying the inciting agents is crucial for developing preventive strategies against this autoimmune kidney disease.
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