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Pathology, Clinical Presentations, and Outcomes of C1q Nephropathy

Alenka Vizjak^*, Duan Ferluga^*, Mojca Roi^*, Anastazija Hvala^*, Jelka Lindi, Tanja Kersnik Levart, Vesna Juri^* and J. Charles Jennette

^* Institute of Pathology, Faculty of Medicine, University of Ljubljana, and Clinical Department of Nephrology and Department of Pediatric Nephrology, University Medical Centre, Ljubljana, Slovenia; and Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina, Chapel Hill, North Carolina

Correspondence: Dr. Alenka Vizjak, Institute of Pathology, Faculty of Medicine, Korytkova 2, SI-1000 Ljubljana, Slovenia. Phone: +386-1-5437133; Fax: +386-1-5437104; E-mail: alenka.vizjak{at}mf.uni-lj.si

Received for publication August 22, 2007. Accepted for publication May 16, 2008.

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.

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J. Am. Soc. Nephrol. 2008 19: A10. [Full Text] [PDF]

Copyright © 2008 by the American Society of Nephrology. Online ISSN: 1533-3450 Print ISSN: 1046-6673