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C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis
T Srivastava1, V Chadha, E M Taboada
1Section of Nephrology, The Children's Mercy Hospital, University of Missouri at Kansas City, 64108, USA. tsrivastava@cmh.edu
Pediatric Nephrology (Berlin, Germany)
|September 7, 2000
Summary
C1q nephropathy, a kidney disease, typically affects older children and has a slow progression. This case highlights a rare, aggressive form in a young child, emphasizing the need for broader differential diagnoses in pediatric glomerulonephritis.
Area of Science:
- Nephrology
- Immunopathology
- Pediatric Nephrology
Background:
- C1q nephropathy is an immune complex glomerulonephritis characterized by mesangial C1q deposits, distinct from lupus nephritis.
- Typical presentation involves nephrotic proteinuria in older children/young adults with a generally slow progression to end-stage renal disease (ESRD).
- Severe crescentic glomerulonephritis is considered absent in established C1q nephropathy cases.
Observation:
- A 3-year-old Hispanic girl presented with renal insufficiency and biopsy-confirmed C1q nephropathy.
- This patient exhibited atypical features: early onset, severe crescentic glomerulonephritis, and rapid progression to ESRD within 14 weeks.
- Systemic lupus erythematosus and other causes were excluded clinically and serologically.
Findings:
- The case demonstrates a rare, aggressive variant of C1q nephropathy with severe crescent formation.
- Despite immunosuppressive therapy, the patient progressed rapidly to ESRD requiring peritoneal dialysis.
- This presentation challenges the previously understood clinical course and histological limitations of C1q nephropathy.
Implications:
- C1q nephropathy should be considered in the differential diagnosis of glomerulonephritis in young children.
- The presence of severe crescentic glomerulonephritis should not exclude C1q nephropathy, particularly in pediatric cases.
- This case underscores the importance of considering atypical presentations for timely diagnosis and management of pediatric kidney diseases.