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: 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; · 4.72 Impact Factor
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ABSTRACT: To retrospectively analyze disease activity and damage-associated factors in granulomatosis with polyangiitis (GPA) in Turkey. A retrospective analysis was carried out in 21 GPA patients. Assessments for activity were performed with the Birmingham Vasculitis Activity Score for GPA (BVAS/GPA) and for permanent organ damage by the Vasculitis Damage Index (VDI). Lower BVAS/GPA (P = 0.002), absence of renal involvement (P = 0.003) and higher creatinine clearence (P = 0.000) at diagnosis increased the likelihood of achieving remission at 6 weeks. Relapses were associated with high creatinine clearence (P = 0.021), low BVAS/GPA (P = 0.014), absence of renal involvement (P = 0.036) and proteinuria (< 0.5/24 h) (P = 0.013) at diagnosis, whereas achieving remission at 6 weeks (P = 0.012) was associated with absence of co-trimoxazole usage (P = 0.038) and less severe clinical subgroup (P = 0.034). Lower cumulative first 6 months of cyclophosphamide and methylprednisolone were associated with earlier (≤ 12 months) relapses (P = 0.048 and P = 0.083, respectively). Baseline damage (VDI ≥ 1) was associated with a delay in diagnosis (P = 0.032), presentation with milder clinical subgroups (P = 0.052) and low serum creatinine (P = 0.013). The increase in VDI in the first 12 months (early damage) constituted most (91%) of the total damage measured at the end of follow-up. Despite high early remission rates, relapse represents a major problem in localized GPA in our study. Baseline damage was associated with longer diagnostic delay and lower baseline serum creatinine. The initial phase of the disease seems to be the most crucial period for mortality and accumulated damage.International Journal of Rheumatic Diseases 02/2013; 16(1):61-71. · 1.65 Impact Factor
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ABSTRACT: The aim of this report is to describe the first instance of Wegner's granulomatosis (WG) in a 12-year-old boy at the Children's Medical Center in Tehran University. This report will analyse the variety of clinical manifestations observed in a 12-year-old boy who suffered sinobronchitis for two months before diagnosis. We present a 12-year-old patient who exhibited pathological symptoms of WG prior to diagnosis and treatment. Our patient was monitored for one year after initial therapy through the outpatients department of the Children's Medical Center in Tehran University. One year after treatment, the patient's WG appeared to be in complete remission and he remained entirely well. Although Wegener's granulomatosis is a rare condition in children and can accordingly be confused with more common ailments, accurate and timely diagnosis can be made based on an established record of clinicopathologic features of the disease, and confirmed by biopsy of nasal mucosa.Hong Kong Journal of Paediatrics 01/2008; 13(New series):60-64. · 0.11 Impact Factor