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Published on: August 19, 2020
Etiology and Outcomes of Rapidly Progressive Glomerulonephritis in Children: A Retrospective Cohort Study
Bobbity Deepthi1, Sriram Krishnamurthy2, Rajesh Nachiappa Ganesh3
1Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry.
Insights
Lupus nephritis is the most common cause of rapidly progressive glomerulonephritis (RPGN) in children. Kidney recovery in pediatric RPGN depends on symptom duration and crescent characteristics.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Autoimmune Disorders
Background:
- Rapidly progressive glomerulonephritis (RPGN) is a severe kidney disease in children.
- Understanding the causes and outcomes of pediatric RPGN is crucial for effective management.
Purpose of the Study:
- To investigate the clinical and etiological spectrum of RPGN in children.
- To determine the kidney outcomes and factors influencing survival in pediatric RPGN patients.
Main Methods:
- Retrospective cohort study of pediatric patients (<18 years) diagnosed with RPGN between 2014 and 2022.
- Analysis of patient demographics, clinical presentation, etiology, treatment, and kidney outcomes.
- Evaluation of factors predicting kidney survival, including symptom duration and histopathological findings.
Main Results:
- Lupus nephritis (33.8%), C3 glomerulopathy (30.9%), and infection-related glomerulonephritis (22.1%) were the leading causes of RPGN in children.
- At presentation, significant proportions of patients experienced pulmonary edema (26.4%), hypertensive emergency (29.4%), and required dialysis (32.4%).
- At last follow-up, 57.3% achieved complete renal recovery, 19.1% partial recovery, and 23.5% progressed to chronic kidney disease (CKD) stage 2 or beyond, with a 7.7% prevalence of end-stage kidney disease (ESKD).
Conclusions:
- Lupus nephritis is the predominant etiology of RPGN in the pediatric population.
- Early symptom recognition (≥7 days), lower crescent percentage (≥37.5%), and absence of fibrous crescents/segmental sclerosis are associated with better kidney survival in pediatric RPGN.
Objective:
To study the clinico-etiological spectrum and outcomes of children with rapidly progressive glomerulonephritis (RPGN).
Methods:
This retrospective cohort study evaluated patients <18 years with RPGN, over an 8-year period (2014-2022), for etiology and kidney outcomes.
Results:
Among 68 RPGN cases [median age 10 (7,12) years], 23 (33.8%) had lupus nephritis, 21 (30.9%) C3 glomerulopathy, and 15 (22.1%) infection-related glomerulonephritis (IRGN). At presentation, 18 (26.4%) patients had pulmonary edema, 20 (29.4%) had hypertensive emergency and 22 (32.4%) required dialysis. Median (IQR) follow-up duration was 24.5 (12,48) months. The median (IQR) admission eGFR was 19 (10.93, 38.60) mL/min/1.73 m2, which increased to 126 (102.7,142) mL/min/1.73m2 at the last follow-up. At the last follow-up, 39 (57.3%) and 13 (19.1%) patients attained complete and partial renal recovery, respectively; while 16 (23.5%) progressed to CKD stage 2 and beyond. The prevalence of end stage kidney disease (ESKD) was 7.3% at 1-year and 7.7% at the last follow-up. Factors predicting kidney survival were duration of symptoms prior to presentation ≥7 days, crescents ≥37.5%, and presence of fibrous crescents/segmental sclerosis.
Conclusion:
Lupus nephritis, was the commonest etiology of RPGN in children. Renal outcomes were determined by pre-admission symptoms, and percentage and stage of crescents.
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