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J Nephropathol. 2015;4(2): 54-58.
doi: 10.12860/jnp.2015.11
PMID: 25964890
PMCID: PMC4417671
Scopus ID: 84927126901
  Abstract View: 2514
  PDF Download: 1340

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: *Corresponding author: Tibor Fülöp, Department of Medicine, University of Mississippi Medical Center, Mississippi, USA. , Email: 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.


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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