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2018 ; 3
(3
): 671-676
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Patient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis
With Inactive Histology
#MMPMID29854975
Novick TK
; Chen M
; Scott J
; Cortazar FB
; Ayoub I
; Little MA
; Hruskova Z
; Salama AD
; Pagnoux C
; Geetha D
Kidney Int Rep
2018[May]; 3
(3
): 671-676
PMID29854975
show ga
INTRODUCTION: Little is known about the anticipated disease course for
individuals who present with renal-limited antineutrophil cytoplasmic antibody
(ANCA)-associated vasculitis but who lack inflammation on a kidney biopsy. The
impact of immunosuppression on renal and overall survival is unknown. METHODS:
Patients were recruited from 2005 to 2016 from 8 centers worldwide (N = 16) for
this descriptive study. All had positive ANCA, elevated serum creatinine with
active urine sediment, histologic evidence of pauci-immune glomerulonephritis
without active lesions, and had no evidence of extrarenal vasculitis. We describe
the characteristics of this cohort and the differences in the clinical,
histologic, and therapeutic parameters of those who developed primary outcomes of
end-stage renal disease (ESRD) and vasculitis relapse. RESULTS: The cohort was
63% Caucasian, and 75% were men, with a median age of 62 years. At entry, the
mean ± SD estimated glomerular filtration rate (eGFR) was 24 ± 20 ml/min per 1.73
m(2), and 5 patients required dialysis. Twelve patients received
immunosuppressive therapy, 25% experienced disease relapse, and 38% developed
ESRD. Patients who developed ESRD had lower baseline eGFRs (8 ± 5 ml/min per 1.73
m(2) vs. 35 ± 18 ml/min per 1.73 m(2); P = 0.001) and more often required
dialysis at presentation (83% vs. 0%; P = 0.001). Patients who relapsed were less
likely to receive immunosuppression (25% for the relapsed group vs. 92% for the
nonrelapsed group; relative risk: 0.27, risk difference: 67%; P = 0.03).
CONCLUSION: Among these patients, lower initial eGFR and dialysis dependence at
presentation might increase the risk for ESRD. Immunosuppression did not affect
renal outcomes in this sample of patients but was associated with a reduced risk
for vasculitis relapse. More information is needed on factors that predict
treatment response in this high-risk group.
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