title: The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.
abstract: BACKGROUND
  Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied.
  
  
  METHODS
  Case series.
  
  
  METHODS
  Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers.
  
  
  RESULTS
  Partial and complete remission, treatment resistance, relapse, complications, renal survival.
  
  
  RESULTS
  Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy.
  
  
  CONCLUSIONS
  Retrospective design, variable treatment protocols.
  
  
  CONCLUSIONS
  The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.
authors.affiliation: Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands. Electronic address: rutger.maas@radboudumc.nl.
authors.forename: Rutger J
authors.initials: RJ
authors.lastname: Maas
authors.affiliation: Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Jeroen K
authors.initials: JK
authors.lastname: Deegens
authors.affiliation: Department of Nephrology, Isala Klinieken, Zwolle, the Netherlands.
authors.forename: Johan R
authors.initials: JR
authors.lastname: Beukhof
authors.affiliation: Department of Nephrology, Rijnstate, Arnhem, the Netherlands.
authors.forename: Louis J
authors.initials: LJ
authors.lastname: Reichert
authors.affiliation: Department of Nephrology, Canisius Wilhelmina Ziekenhuis, Nijmegen, the Netherlands.
authors.forename: Marc A
authors.initials: MA
authors.lastname: Ten Dam
authors.affiliation: Department of Nephrology, Jeroen Bosch Ziekenhuis, 's Hertogenbosch, the Netherlands.
authors.forename: Jaap J
authors.initials: JJ
authors.lastname: Beutler
authors.affiliation: Department of Nephrology, Máxima Medisch Centrum, Veldhoven, the Netherlands.
authors.forename: A Warmold L
authors.initials: AWL
authors.lastname: van den Wall Bake
authors.affiliation: Department of Nephrology, St. Elisabeth Ziekenhuis, Tilburg, the Netherlands.
authors.forename: Pieter L
authors.initials: PL
authors.lastname: Rensma
authors.affiliation: Department of Nephrology, Catharina Ziekenhuis, Eindhoven, the Netherlands.
authors.forename: Constantijn J
authors.initials: CJ
authors.lastname: Konings
authors.affiliation: Department of Nephrology, Catharina Ziekenhuis, Eindhoven, the Netherlands.
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Geerse
authors.affiliation: Department of Nephrology, Gelderse Vallei, Ede, the Netherlands.
authors.forename: Geert W
authors.initials: GW
authors.lastname: Feith
authors.affiliation: Department of Nephrology, Viecuri Medisch Centrum, Venlo, the Netherlands.
authors.forename: Willi H
authors.initials: WH
authors.lastname: Van Kuijk
authors.affiliation: Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Jack F
authors.initials: JF
authors.lastname: Wetzels
mesh_terms: D058186:Acute Kidney Injury / Q000453:epidemiology*; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D003520:Cyclophosphamide / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000453:epidemiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology; D020127:Recovery of Function; D012008:Recurrence; D012074:Remission Induction; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D013927:Thrombosis / Q000453:epidemiology; D020246:Venous Thrombosis / Q000453:epidemiology; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Corticosteroids; acute kidney injury (AKI); adolescents; adult-onset MCNS; adults; case series; complete remission; cyclophosphamide; immunosuppression; minimal change disease (MCD); nephrotic syndrome (NS); partial remission; relapse; renal survival; treatment resistance