Publication: Primary focal segmental glomerulosclerosis: a systematic review of clinical presentation, treatment and outcomes in adults

Primary focal segmental glomerulosclerosis: a systematic review of clinical presentation, treatment and outcomes in adults.
Melbourne: ANZSN Annual Scientific Meeting; 2014.

Abstract

Aim: To systematically review evidence which describes clinical features, treatment and outcomes in adult patients with primary Focal Segmental Glomerulosclerosis (FSGS). Background: Primary FSGS is one of the most common glomerular diseases to progress to end stage kidney disease (ESKD) unless remission is achieved with immunosuppression. Renewed interest is due to increasing incidence of ESKD attributed to FSGS in conjunction with the increasing burden of ESKD on the patient, family and society at large. Methods: We searched databases to identify studies which assessed clinical presentation, treatment and outcomes in adult patients with primary FSGS. Quality of studies was assessed using a modified validated checklist. Outcomes assessed included treatment response and renal dysfunction. Findings were summarized descriptively. Results: We included 11 retrospective studies of 1,091 patients. Six were single-centre and 5 were multi-centre. Follow-up ranged from 36 months to 10 years. Seven of 8 studies showed a male predominance with ethnicity (white/black) equally distributed between 4 studies with comparisons. The majority of patients in all studies presented with nephrotic range proteinuria and haematuria. Immunosuppressive therapy was the mainstay of treatment in 7 of 10 studies with 7 of these studies experiencing remission rates ≥50%. Five studies found response to treatment improved renal survival (p = <0.05) with 3 defining this treatment to be corticosteroids. Conclusions: Current evidence suggests that the best clinical indicator of long term prognosis is induction of remission by corticosteroid therapy. The role of histological subtypes and clinical variables at presentation requires further investigation. These findings are based on small observational studies of suboptimal quality. More large, well-designed and reported studies are needed to influence clinical practice and promote informed individualized patient management.


, from FoU-centrum för primärvård och folktandvård Södra Älvsborg
http://au.researchweb.org/is/en/foualvsborg/user/publication?ref=2039951