title: Resolution of C1q deposition but not of the clinical nephrotic syndrome after immunomodulating therapy in focal sclerosis.
abstract: BACKGROUND
  The natural evolution of C1q nephropathy (C1qNP) during immunosuppressive treatment is relatively little studied or understood.
  
  
  METHODS
  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.
authors.affiliation: Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS, USA.
authors.forename: Tibor
authors.initials: T
authors.lastname: Tibor Fülöp
authors.affiliation: Department of Internal Medicine, University of Mississippi Medical Center, Jackson, MS, USA ; Department of Medicine, Medical and Health Science Centre University of Debrecen, Hungary.
authors.forename: Éva
authors.initials: É
authors.lastname: Csongrádi
authors.affiliation: Department of Anesthesiology, University of Mississippi Medical Center, Jackson, Mississippi, USA.
authors.forename: Anna A
authors.initials: AA
authors.lastname: Lerant
authors.affiliation: ProPath, Dallas, TX, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Lewin
authors.affiliation: Department of Pathology, University of Mississippi Medical Center, Jackson, Mississippi, USA Case Report.
authors.forename: Jack R
authors.initials: JR
authors.lastname: Lewin
keywords: C1q nephropathy; Cyclophosphamide; Focal glomerulosclerosis; Nephrotic syndrome; Sleep apnea