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J Nephropathol. 2015;4(2): 54-58.
doi: 10.12860/jnp.2015.11
PMID: 25964890
PMCID: PMC4417671
Scopus ID: 84927126901
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Case Report

Resolution of C1q deposition but not of the clinical nephrotic syndrome after immunomodulating therapy in focal sclerosis

Tibor Tibor Fülöp 1 * , Éva Csongrádi 1,2, Anna A. Lerant 3, Matthew Lewin 4, Jack R. Lewin 5

1 Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS, USA
2 Department of Medicine, Medical and Health Science Centre University of Debrecen, Hungary
3 Department of Anesthesiology, University of Mississippi Medical Center, Jackson, Mississippi, USA
4 ProPath, Dallas, TX, USA
5 Department of Pathology, University of Mississippi Medical Center, Jackson, Mississippi, USA Case Report
*Corresponding author: Tibor Fülöp, Department of Medicine, University of Mississippi Medical Center, Mississippi, USA. tfulop@umc.edu

Abstract

Background: The natural evolution of C1q nephropathy (C1qNP) during immunosuppressive treatment is relatively little studied or understood.

Case Presentation: A 30 year-old Caucasian female was referred to us for further management of biopsy-proven C1qNP and severe nephrotic syndrome. Serologic work-up remained negative, including complement C3 and C4 levels and repeated testing for antinuclear antibodies. A renal biopsy revealed minimal change nephropathy vs. focal sclerosis on light microscopy and C1qNP on immunopathology. She has failed trials of high-dose oral prednisone, mycophenolate mofetil 1,500 mg twice a day and a subsequent regimen of monthly IV cyclophosphamide 750 mg × 9 cycles. She also received the maximum tolerated angiotensin-converting enzyme inhibitor and spironolactone therapy. Random urine protein-to-creatinine (UPC) ratio predicted proteinuria in the range between 5-35 gm/day, while serum creatinine rose progressively from 1.0 mg/dL to 1.4 mg/dL (to convert to μmol/L, multiply by 88.4). A decision was made to repeat renal biopsy to reassess the underlying histology. The biopsy revealed focal sclerosis but no C1q deposition.

Conclusions: Our case illustrates at least two points: first, an established pathologic diagnosis does not obviate the need for repeated renal biopsy later on, should diagnostic uncertainty persist. Second, histological diagnoses may evolve over time, especially in a patient receiving active and powerful immune-modulating treatment. In our case, the clinical nephrosis did not change with immunosuppressive therapy while C1q deposition ceased, making this latter entity likely the immunologically mediated process.

Keywords: C1q nephropathy, Cyclophosphamide, Focal glomerulosclerosis, Nephrotic syndrome, Sleep apnea

Implication for health policy/practice/research/medical education:

C1q nephropathy (C1qNP) is an uncommon histopathologic finding, with a predominant deposition of C1q in glomerular mesangium. Herewith, we have documented a complete resolution of C1q deposition after immunomedulating therapy, without meaningfully changing the clinical picture of nephrotic syndrome. The overall presentation and clinical course of our patient was compatible with steroid-resistant focal glomerular sclerosis.

Please cite this paper as: Fülöp T, Csongrádi E, Lerant AA, Lewin M, Lewin JR. Resolution of C1q deposition but not of the clinical nephrotic syndrome after immunomodulating therapy in focal sclerosis. J Nephropathol. 2015; 4(2):54-58. DOI: 10.12860/jnp.2015.11

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ePublished: 01 Apr 2015
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