Background/Purpose: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal-limited vasculitis (RLV). We aimed to study the clinical features and renal outcomes of ANCA vasculitides patients in a county hospital.

Methods: 37 consecutive patients with biopsy proven glomerulonephritis related to ANCA vasculitides were included to participate in the study. IRB approval was taken. Patients with minimum follow up of 6 months were considered to be eligible. Data was collected through retrospective medical chart reviews including on demographics, ANCA type and titers, other organ involvement, kidney biopsy findings, treatment received, baseline and follow up at creatinine at 6 months, and outcomes with respect to renal function, and death. Chi-square and t-tests were done to study correlates of crescentic glomerulonephritis, progression to ESRD and mortality.

Results: Mean (SD) age was 54 years (14) with 21 female and 16 male patients. Almost half of the patients (17/37; 46%) were of Hispanic origin (Table 1). Clinical diagnosis was MPA in 16/37 (43%) patients while 10/37 (27%) had GPA, and rest 11/37 (30%) had RLV. Serology was positive for pANCA in 22/37 (59.5%) patients and rest were cANCA positive (12/37; 32.4%). 36/37 (97.3%) patients had hematuria on their urinalysis with 12/37 (32%) having nephrotic range proteinuria (>3.5 g). 27/37 (73%) patients had crescents on kidney biopsy. Pulmonary involvement was seen in over 50% patients (19/37) with diffuse alveolar hemorrhage in 8/37 (21%). Most patients were treated with cyclophosphamide (35/37; 94.6%) with only 8/37 (21%) and 6/37 (16%) being treated with plasmapheresis and rituximab, respectively. Mean (95% CI) baseline and 6 month follow up creatinine were 4.93 (3.65 – 6.22) and 2.82 (1.95 – 3.69), respectively. Median (IQR) follow up was 22 (12-53) months. 10 out of 37 patients (27%) progressed to ESRD and needed dialysis. Mean time (SD) to progression to ESRD was 9.05 (5.08) months. Gender, ethnicity, ANCA type and titers did not predict crescentic glomerulonephritis on kidney biopsy. Crescentic glomerulonephritis did not predict progression to ESRD but correlated with mortality (P value 0.002). Degree of fibrosis on renal biopsy also did not correlate with progression to ESRD but predicted mortality (P value 0.03).

Conclusion: Crescentic glomerulonephritis was more common in this multi-ethnic population. Crescentic glomerulonephritis and degree of fibrosis on kidney biopsy did not predict progression to ESRD but correlated with mortality.

Table 1: Clinical characteristics and outcomes of ANCA related vasculitides associated renal disease

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-characteristics-and-outcomes-of-anca-vasculitides-associated-renal-disease-in-a-multi-ethnic-population-from-a-county-hospital/