Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: Three types of clinical response

Arbus, Gerald S.; Poucell, S; Bacheyie, Godfrey S.; Baumal, Reuben

doi:10.1016/s0022-3476(82)80177-7

Cited by 66 publications

(36 citation statements)

References 6 publications

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“…In recent years prednisone has became the treatment of choice for focal segmental glomerulosclerosis and its therapeutic response has been recognized as a predictor of long-term prognosis (7,12,16,(26)(27)(28)(29). In contrast, the persistence of nephrotic syndrome portends a reserved prognosis (15,30).…”

Section: Discussionmentioning

confidence: 99%

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis

Martinelli

Pereira

Silva

et al. 2004

Braz J Med Biol Res

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Prednisone is the initial treatment of primary focal segmental glomerulosclerosis. However, when immunosuppressive agents in combination with steroids are used in the treatment of prednisone-dependent and prednisone-resistant patients the remission rate is variable. We report a long-term trial using cyclophosphamide (2.0 to 3.0 mg/kg body weight for 12 weeks) in combination with prednisone (1.0 to 2.0 mg/kg body weight), as compared with prednisone alone for the treatment of prednisone-resistant and frequently relapsing nephrotic syndrome and focal segmental glomerulosclerosis. Fifty-four patients (34 males and 20 females) with a diagnosis of idiopathic nephrotic syndrome and focal segmental glomerulosclerosis, followed-up for an average of 86.1 ± 82.4 months, were evaluated. Complete remission occurred in 20.4% and partial remission in 14.8% of the patients treated with steroids and in 26.7 and 20.0% of the patients treated with cyclophosphamide + prednisone, respectively. Of the 24 prednisoneresistant patients treated with steroids in combination with cyclophosphamide, 33.3% obtained a complete/partial response. At the time of final evaluation, 25% of the patients treated with prednisone and 10.0% of those treated with prednisone in combination with cyclophosphamide had reached end-stage renal disease. Persistent nephrotic syndrome and progressive renal insufficiency were more frequently observed among the patients treated with prednisone alone (50.0 vs 33.3% and 33.3 vs 16.7%, respectively). The treatments were well tolerated and no patient experienced adverse reactions requiring discontinuation of medications. Although open-label and non-randomized, the present trial showed that cyclophosphamide is a reasonable choice for the treatment of primary focal segmental glomerulosclerosis and prednisone-resistant nephrotic syndrome.

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Section: Discussionmentioning

confidence: 99%

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis

Martinelli

Pereira

Silva

et al. 2004

Braz J Med Biol Res

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“…However, low dose alternate day steroid therapy was later found to improve the prognosis in childhood FSGS, especially when combined with a cytotoxic medication such as cyclophos-phamide [20]. Data on long term prognosis of prednisone plus cyclophosphamide showed kidney survival of 60 % at 15 years of follow up in the Toronto study [20]. In the 1980's, intravenous, pulse methylprednisolone [24] over long periods was recommended on the basis of significantly improved kidney survival.…”

Section: Prognosis In Fsgsmentioning

confidence: 99%

Primary Focal Segmental Glomerulosclerosis, an Important Component of Childhood Nephrotic Syndrome: Therapeutic Options and Genetic Basis

Chan¹,

Roth²

2009

TOPEDJ

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Introduction:We aim to review the therapeutic options in ameliorating the progression of focal segmental glomerulosclerosis (FSGS). We shall comment on the long-term prognosis and the clinical implications of candidate genes in familial cases of FSGS. Materials and Method:Key references from the past concerning FSGS were analyzed, together with a PubMed search of the literature from 1998 to 2008.Results and Discussion: Treatment of FSGS consists of one or more of the following medications: vitamin E, prednisone, angiotensin-converting enzyme inhibitor, angiotensin receptor blocker; in the severe cases, methylprednisolone, cyclophosphamide, cyclosporine, calcineurin inhibitors, rituximab and mycophenolate. The prognoses with lengths of followup to 14 years were analyzed. The genetics of familial FSGS and the strategy of kidney transplants in such cases need special attention. Conclusion:We reviewed the available modalities of treatment and the long-term prognosis. The new findings on familial forms of FSGS and the clinical implications are succinctly presented.

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“…Patients with FSGS are usually steroid resistant and cytotoxic resistant and the majority of them progress to renal insufficiency within 1-15 years [3,8,14], Their clinical management is often very difficult, and there fore, a trial with CS seemed to be justified. We included all children with idiopathic FSGS between 2 and 16 years who were steroid resistant (i.e.…”

Section: Focal Segmental Glomerulosclerosismentioning

confidence: 99%