Dataset Information

Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

ABSTRACT: Idiopathic collapsing FSGS has historically been associated with poor renal outcomes. Minimal clinical data exist on the efficacy of immunosuppressive therapy. Our study sought to provide a comprehensive description of renal survival in patients with collapsing and not otherwise specified FSGS after controlling for factors affecting renal prognosis.We performed a retrospective analysis of an inception cohort study of patients diagnosed between 1989 and 2012. All potential patients with collapsing FSGS fulfilling the inclusion criteria were identified and compared with patients with not otherwise specified FSGS (approximately 1:2 ratio) on the basis of biopsy report and record availability. Time to ESRD was analyzed using Cox proportional hazards models.In total, 187 patients were studied (61 collapsing and 126 not otherwise specified), with a mean follow-up of 96 months. At baseline, patients with collapsing FSGS had higher median proteinuria (12.2 [5.6-14.8] versus 4.4 [2.3-8.1] g/d, respectively; P<0.001), lower median albuminemia (2.4 [1.9-3.0] versus 2.9 [1.8-3.7] g/dl, respectively; P=0.12), and lower median eGFR (48 [26-73] versus 60 [42-92] ml/min per 1.73 m2, respectively; P=0.01) than patients with not otherwise specified FSGS. The proportion of patients with remission of proteinuria was similar in patients with collapsing FSGS and patients with not otherwise specified FSGS (65.7% [23 of 35] versus 63.2% [72 of 114], respectively; P=0.84). The overall renal outcome (ESRD defined as eGFR<15 ml/min per 1.73 m2, dialysis, or transplantation) of patients with collapsing FSGS was not poorer than that of patients with not otherwise specified FSGS in multivariate analyses after adjusting for baseline characteristics and immunotherapy (hazard ratio, 1.78; 95% confidence interval, 0.92 to 3.45).Compared with not otherwise specified FSGS, idiopathic collapsing FSGS presented with more severe nephrotic syndrome and lower eGFR but had a similar renal survival after controlling for exposure to immunosuppressive treatment. These results highlight the importance of early diagnosis and institution of immunosuppressive therapy in patients with collapsing FSGS.

SUBMITTER: Laurin LP

PROVIDER: S-EPMC5053801 | biostudies-other | 2016 Oct

REPOSITORIES: biostudies-other

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Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

Laurin Louis-Philippe LP Gasim Adil M AM Derebail Vimal K VK McGregor JulieAnne G JG Kidd Jason M JM Hogan Susan L SL Poulton Caroline J CJ Detwiler Randal K RK Jennette J Charles JC Falk Ronald J RJ Nachman Patrick H PH

Clinical journal of the American Society of Nephrology : CJASN 20160721 10

<h4>Background and objectives</h4>Idiopathic collapsing FSGS has historically been associated with poor renal outcomes. Minimal clinical data exist on the efficacy of immunosuppressive therapy. Our study sought to provide a comprehensive description of renal survival in patients with collapsing and not otherwise specified FSGS after controlling for factors affecting renal prognosis.<h4>Design, setting, participants, & measurements</h4>We performed a retrospective analysis of an inception cohort ...[more]

PMID: 27445167

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Project description:Despite the huge progress in the definition and classification of vestibular disorders within the last decade, there are still patients whose recurrent vestibular symptoms cannot be attributed to any of the recognized episodic vestibular syndromes, such as Menière's disease (MD), vestibular migraine (VM), benign paroxysmal positional vertigo (BPPV), vestibular paroxysmia, orthostatic vertigo or transient ischemic attack (TIA). The aim of the present international, multi-center, cross-sectional study was to systematically characterize the clinical picture of recurrent vestibular symptoms not otherwise specified (RVS-NOS) and to compare it to MD and VM. Thirty-five patients with RVS-NOS, 150 patients with VM or probable VM and 119 patients with MD were included in the study. The symptoms of RVS-NOS had been present for 5.4 years on average before inclusion, similar to VM and MD in this study, suggesting that RVS-NOS is not a transitory state before converting into another diagnosis. Overall, the profile of RVS-NOS vestibular symptoms was more similar to VM than MD. In particular, the spectrum of vestibular symptom types was larger in VM and RVS-NOS than in MD, both at group comparison and the individual level. However, in contrast to VM, no female preponderance was observed for RVS-NOS. Positional, head-motion and orthostatic vertigo were reported more frequently by patients with RVS-NOS than MD, while external vertigo was more prevalent in the MD group. At group level, the spectrum of attack durations from minutes to 3 days was evenly distributed for VM, while a small peak for short and long attacks in RVS-NOS and a big single peak of hours in MD were discernible. In general, vertigo attacks and associated vegetative symptoms (nausea and vomiting) were milder in RVS-NOS than in the other two disorders. Some patients with RVS-NOS described accompanying auditory symptoms (tinnitus: 2.9%, aural fullness and hearing loss: 5.7% each), migrainous symptoms (photophobia, phonophobia or visual aura in 5.7% each) or non-migrainous headaches (14%), but did not fulfill the diagnostic criteria for MD or VM. Absence of a life time diagnosis of migraine headache and attack duration of <5 min were further reasons not to qualify for VM. In some RVS-NOS patients with accompanying ear symptoms, attack durations of <20 min excluded them from being diagnosed with MD. These findings suggest that RVS-NOS is a stable diagnosis over time whose overall clinical presentation is more similar to VM than to MD. It is more likely to be composed of several disorders including a spectrum of mild or incomplete variants of known vestibular disorders, such as VM and MD, rather than a single disease entity with distinct pathognomonic features.

Dataset Information

Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

Publications

Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

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