Child Kidney Dis.  2017 Oct;21(2):165-168. 10.3339/jkspn.2017.21.2.165.

A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy

• Her SM
• Lee KH
• Shin JI

• KMID: 2394458
• DOI: http://doi.org/10.3339/jkspn.2017.21.2.165

Abstract

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

Keyword

Focal segmental glomerulosclerosis (FSGS); End stage renal disease (ESRD); Soluble urokinase-type plasminogen activator receptor (suPAR); Anti-CD40 autoantibody

MeSH Terms

Child
Diagnosis
Female
Glomerulosclerosis, Focal Segmental*
Humans
Hypoalbuminemia
Immunologic Factors
Kidney Failure, Chronic
Kidney Transplantation
Methylprednisolone*
Nephrotic Syndrome
Plasmapheresis*
Prognosis
Proteinuria
Recurrence
Urokinase-Type Plasminogen Activator
Young Adult
Immunologic Factors
Methylprednisolone
Urokinase-Type Plasminogen Activator