Longterm outcome of Wegener's granulomatosis in patients with renal disease requiring dialysis.
ABSTRACT It is known that renal failure is a poor prognostic marker for survival in Wegener's granulomatosis (WG). We investigated the longterm outcome of patients with WG who have severe renal disease requiring dialysis.
We performed a retrospective analysis of 104 patients with WG followed at our institution between 1982 and 1997. Twenty-three patients who required dialysis were studied in detail to determine outcomes and factors that influenced survival and restoration of renal function.
Of 23 dialysis dependent patients with WG, 11 died (Group 1). 7 either remained dialysis dependent or received successful renal transplants (Group 2), and 5 substantially recovered renal function (Group 3). Mean serum creatinine at the end of a mean followup period of 38.4 months for Group 3 was 1.8 mg/dl. There was no apparent difference between groups in regard to disease profile, e.g., distribution of organ involvement or serum creatinine when renal impairment was first recognized (mean serum creatinine for groups: 1: 3.0 mg/dl; 2: 5.6 mg/dl; 3: 5.5 mg/dl) and peak serum creatinine prior to dialysis (means for groups: 1: 9.5 mg/dl; 2: 10.5 mg/dl; 3: 9.6 mg/dl). Infection secondary to immunosuppression was the leading cause of death in Group I patients.
Because the clinical profile and degree of renal failure, as judged by serum creatinine, did not differ among patients who did or did not regain dialysis independent renal function, we recommend aggressive immunosuppressive therapy in all cases of active WG with acute rapidly worsening renal failure, regardless of the severity of renal impairment.
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ABSTRACT: Rituximab (RTX) is approved for remission induction in ANCA associated vasculitis (AAV). However, data on use of RTX in patients with severe renal disease is lacking. We conducted a retrospective multi-center study to evaluate the efficacy and safety of RTX with glucocorticoids (GC) with and without use of concomitant cyclophosphamide (CYC) for remission induction in patients presenting with e GFR less than 20 ml/min/1.73 m(2). We evaluated outcomes of remission at 6 months (6 M), renal recovery after acute dialysis at diagnosis, e-GFR rise at 6 M, patient and renal survival and adverse events. A total 37 patients met the inclusion criteria. The median age was 61 years. (55-73), 62 % were males, 78 % had new diagnosis and 59 % were MPO ANCA positive. The median (IQR) e-GFR at diagnosis was 13 ml/min/1.73 m(2) (7-16) and 15 required acute dialysis. Eleven (30 %) had alveolar hemorrhage. Twelve (32 %) received RTX with GC, 25 (68 %) received RTX with GC and CYC and seventeen (46 %) received plasma exchange. The median (IQR) follow up was 973 (200-1656) days. Thirty two of 33 patients (97 %) achieved remission at 6 M and 10 of 15 patients (67 %) requiring dialysis recovered renal function. The median prednisone dose at 6 M was 6 mg/day. The mean (SD) increase in e-GFR at 6 months was 14.5 (22) ml/min/m(2). Twelve patients developed ESRD during follow up. There were 3 deaths in the first 6 months. When stratified by use of concomitant CYC, there were no differences in baseline e GFR, use of plasmapheresis, RTX dosing regimen or median follow up days between the groups. No differences in remission, renal recovery ESRD or death were observed. This study of AAV patients with severe renal disease demonstrates that the outcomes appear equivalent when treated with RTX and GC with or without concomitant CYC.Journal of nephrology 05/2015; DOI:10.1007/s40620-015-0208-y · 2.00 Impact Factor
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ABSTRACT: Pauci-immune glomerulonephritis is rare in African Americans (AA) and the clinical presentation and treatment outcomes of vasculitis have not been well described. We identified patients who were 2-92 years of age between 1983 and 2011 with a diagnosis of biopsy-proven pauci-immune glomerulonephritis (GN) at any point during their disease course. Comparing AA to Caucasian patients, we examined demographics, clinical features at presentation, treatment and outcomes of relapse, end-stage renal disease (ESRD), and death. Of the 672 patients, 75 were AA with the remainder being Caucasian. Compared to Caucasians, disease onset in AA was at an earlier age (52 vs. 57 years, p = 0.05) and was more often myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive (71% vs. 54%, p = 0.01). AA patients had a shorter median time between onset of symptoms and biopsy compared to Caucasians [median (IQR): 0.23 (0.00, 1.22) months vs. 0.66 (0.00, 3.62) months, p = 0.003]. Median [Interquartile range (IQR)] follow-up in months was 28 (5, 52) in AA and 26 (10, 55) in Caucasian patients. Median estimated glomerular filtration rate was similar at presentation (21 vs. 22ml/min/m(2)). Both groups had similar induction treatment regimens. There was less favorable treatment response among AA compared to Caucasians for initial treatment resistance (28% vs. 18%, p = 0.05) and complete remission (72% vs. 82%, p = 0.05). There were no differences in the number of renal relapses or number of deaths between the 2groups. Overall, in multivariable analyses controlling for age, race, ANCA type, and entry serum creatinine, there were not differences by race in treatment response, renal relapse, ESRD, or death over the entire time of follow-up. AA patients with pauci-immune GN are younger and more often MPO-ANCA positive compared to Caucasians. Despite a shorter time to diagnosis for AA patients, there were no differences compared to Caucasians in treatment response, ESRD, renal relapse, or death rates by race over the entire duration of follow-up.Seminars in arthritis and rheumatism 12/2013; DOI:10.1016/j.semarthrit.2013.11.011 · 3.63 Impact Factor
Rheumatic Disease Clinics of North America 11/2001; 27(4):863-886. DOI:10.1016/S0889-857X(05)70240-4 · 1.74 Impact Factor