Abstract: SA-PO646
Analysis of Clinical Features in ANCA-Associated Vasculitis with Rapidly Progressive Glomerulonephritis: Thirty-Five Years of a Single-Center Experience
Session Information
- Glomerular Diseases: ANCA, Anti-GBM, Kidney Biopsy
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Kawashima, Soko, Kyorin University School of Medicine, Mitaka, TOKYO, Japan
- Uchida, Hiroko, Kyorin Univercity, Tokyo, Japan
- Komagata, Yoshinori, Kyorin University School of Medicine, Mitaka, TOKYO, Japan
- Kaname, Shinya, Kyorin University School of Medicine, Mitaka, TOKYO, Japan
Background
The clinical features of AAV, with respect to renal involvement, may have been changing recently in Japan, where MPO-ANCA-associated vasculitis (MPO-AAV) are dominant in contrast to the Western countries. Thus, we retrospectively analyzed the clinical database of the141 AAV-patients with RPGN who were admitted to our hospital for the last 35 years.
Methods
At the onset, all patients fulfilled the Chapel Hill Consensus Conference (CHCC) classification criteria for MPA, GPA and EGPA. 141 patients (56 male, 85 female: MPA 118, MPO-GPA 12, PR3-GPA 7, EGPA 4) who underwent initial treatment diagnosed with AAV and presented rapidly progressive glomerulonephritis (RPGN) at our hospital from 1983 to 2018. We divided the AAV patients into the 3 groups(Group1 (1983-2000: 31 cases), Group2 (2001-2010: 56 cases), 3 (2011-2018: 54 cases)), and compared the clinical features and renal prognosis.
Results
The frequencies of RPGN in AAV were 76% (31/41) in group1, 50% (56/111) in group2 and 47% (54/115) in group 3, respectively. The average ages at the first onset were 67.2 ± 10.9, 69.1 ± 12, 75.4 ± 9.1 years (mean±SD). The BVAS were 24.8 ± 3.9, 22.8 ± 8.1, and 17.8 ± 4.7. Serum creatinine levels (mg/dl) were 6.5 ± 4.2, 4.6 ± 3.5, 3.2 ± 2.1, and the frequencies of renal death were 71% (22/31), 46% (26/56), 22% (12/54). The dialysis withdrawal rates were 0% (0/22), 7% (2/28), 20% (3/15).
The immunosuppressants were used in the 19% (6/31), 25% (14/56), 44% (24/54) of the patients in initial treatment. Cyclophosphamide (CY) was used for 16% (5/31), 23% (13/56), 33% (18/54) of the patients. RTX was used only in group III for 9% of the patients (5/54). PE were used 3% (1/31), 2% (1/56), 6% (3/54). One-year survival rates were 52% (16/31), 85% (47/55), 88% (36/41), with the survival observation period for 40.0 ± 56.2 months, 47.3 ± 42.7 months, 29.6 ± 29.5 months, respectively. Death due to vasculitis was seen in 30% (8/27), 17% (4/24), 11% (1/9), and death due to infection in 30% (8/27), 21% (5/24), 56% (5/9). Relapse rates were 32% (10/31), 21% (12/56), 11% (6/54).
Conclusion
These results clearly show the changing features of ANCA-associated renal vasculitis, with an earlier detection and the improvement of renal and patient survival during the last decades in Japan.