Spontaneous Remission Of Post-transplant Recurrent Focal And Segmental Glomerulosclerosis
Saeed, B., Mazloum, H., & Askar, M. (2011). spontaneous remission of post-transplant recurrent focal and segmental glomerulosclerosis. Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia, 22(6), 1219–1222. https://doi.org/10.1016/j.ekir.2024.02.923
View Original Source →Abstract
A 12-year-old girl with a history of steroid and cyclosporine (CsA) resistant nephrotic syndrome owing to focal and segmental glomerulosclerosis (FSGS) has progressed to end-stage renal disease (ESRD) for which she underwent hemodialysis for 18 months before she successfully received a fully matched kidney transplant from her sister at the age of nine years. The post transplantation (Tx) period was marked by an early and massive proteinuria indicating recurrent FSGS for which she received 12 sessions of plasmapheresis (PP); unfortunately, she did not appear to have any response to the PP therapy; thereafter, a conservative management comprising essentially enalapril and losartan has been initiated and was also not successful during the first four months, however, a very gradual response has been noticed to occur after five months of conservative therapy and ultimately, the patient attained complete remission after 21 months of treatment. Amazingly, 15 months after discontinuation of enalapril and losartan, she remained in a complete and sustained remission with a good renal allograft function. To the best of our knowledge, this is the first case ever reported in the literature of a "spontaneous" remission of post transplant recurrent FSGS.
Case Details
Disease Location
Kidney
Personal Characteristics
12-year-old girl with a long history of nephrotic syndrome that started at the age of three.
Clinical Characteristics
Diagnosed with steroid-resistant with focal and segmental glomerulosclerosis (fsgs). The patient had no response to cyclosporine. Csa was discontinued and conservative therapy was started. She reached end-stage renal disease at the age of 7.5 years. 18 months later (age 9) she received a kidney transplant requiring an immunosuppressive protocol. During the second week of post-operation, she started to have pitting edema in the lower limbs associated with massive proteinuria of 2.5 g per day with hypoalbuminemia and hypercholesterolemia, indicating an early recurrence of fsgs. Plasmapheresis therapy was initiated without response. The patient was started on conservative management comprising essentially enalapril in addition to diuretics and statin.
Remission Characteristics
The patient attained complete remission at 21 month post-transplantation fsgs relapse.
Treatment & Mechanisms
Proposed Remission Mechanisms
Spontaneous remission
Clinical Treatment
Cyclosporine, acei, diuretics, statin immunosuppressive protocol: tacrolimus (tac), mycophenolate mofetil (mmf), and steroids. Kidney transplant. Plasmapheresis
Non-Clinical Treatment
Supportive therapies (not specified)