Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy
Open Access
- 1 November 2008
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of the American Society of Nephrology
- Vol. 19 (11), 2237-2244
- https://doi.org/10.1681/asn.2007080929
Abstract
C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change–like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change–like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex–mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.Keywords
This publication has 17 references indexed in Scilit:
- A Proposed Taxonomy for the PodocytopathiesClinical Journal of the American Society of Nephrology, 2007
- Distinguishing C1q nephropathy from lupus nephritisNephrology Dialysis Transplantation, 2004
- C1q nephropathy: A variant of focal segmental glomerulosclerosisKidney International, 2003
- Spontaneous improvement in a case of C1q nephropathyAmerican Journal of Kidney Diseases, 2000
- Pan-nephritis (glomerulonephritis, arteriolitis, and tubulointerstitial nephritis) associated with predominant mesangial C1q deposition and hypocomplementemia: a variant type of C1q nephropathy?American Journal of Kidney Diseases, 1996
- C1q Nephropathy: do C1q deposits have any prognostic significance in the nephrotic syndrome?Nephrology Dialysis Transplantation, 1992
- Clq Nephropathy: A Pediatric Clinicopathologic StudyAmerican Journal of Kidney Diseases, 1991
- Clq Nephropathy: A Distinct Pathologic Entity Usually Causing Nephrotic SyndromeAmerican Journal of Kidney Diseases, 1985
- Immunohistopathologic Evaluation of C1q in 800 Renal Biopsy SpecimensAmerican Journal of Clinical Pathology, 1985
- Nonsystemic Mesangiopathic Glomerulonephritis with “Full House” Immunofluorescence: Pathological and Clinical Observations in Five PatientsAmerican Journal of Clinical Pathology, 1982