Abstract | BACKGROUND: METHODS: Patients with AAV were identified by performing a case review of medical records of 705 patients who received PLEX in a single tertiary referral centre between 2000 and 2010. Patient characteristics and outcomes were recorded. The Kaplan-Meier method, log-rank tests and Cox regression analysis were used for survival analyses. RESULTS: A total of 94 patients with AAV were identified (44 men, 50 women; median age 60 years, range 21-90 years; 52 proteinase 3- ANCA, 41 myeloperoxidase- ANCA and 1 ANCA-negative; 8 double-positive for ANCA and anti-glomerular basement membrane; 93 newly diagnosed/1 relapse; 55 [58.5 %] required dialysis). The reasons for initiating PLEX therapy were severe renal involvement alone in 52 %, AH in 10 %, both renal involvement and AH in 35 %, and "other" in 3 %. The patients had 3-27 (median 7) PLEX sessions. At 3 months, 81 (86 %) of 94 were alive and 62 (66 %) of 94 were alive and dialysis-independent. The median follow-up was 41 months (minimum-maximum 0.5-137 months), when 56 (59.6 %) of 94 patients were alive and 47 (50 %) were dialysis-independent. The estimated overall survival rates were 75.3 % at 1 year and 61.1 % at 5 years. Patient survival decreased with increasing age at presentation (5-year survival 85 % for age <50 years, 64.4 % for ages 50-65 years, and 41 % for >65 years; p < 0.01 for comparison between all groups). Estimated renal survival rates were 65.5 % at 1 year and 43 % at 5 years. Renal survival was worse in patients aged >65 years than in the younger patients (5-year survival 25.1 % in patients >65 years vs. 50.8 % for those ≤65 years, p < 0.01). The estimated renal survival was better in patients with higher Disease Extent Index (DEI) >6 than in patients with DEI ≤6 (5-year survival 52.1 % vs. 39.4 %, p = 0.04), even though this was not confirmed in multivariate analysis. CONCLUSIONS: The mortality of patients presenting with severe manifestations of AAV remains high despite the use of PLEX. Older age at presentation is associated with worse overall and renal prognosis.
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Authors | Doubravka Frausová, Zdenka Hrušková, Věra Lánská, Jana Lachmanová, Vladimír Tesař |
Journal | Arthritis research & therapy
(Arthritis Res Ther)
Vol. 18
Pg. 168
(07 13 2016)
ISSN: 1478-6362 [Electronic] England |
PMID | 27412443
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Adult
- Aged
- Aged, 80 and over
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
(mortality, therapy)
- Female
- Humans
- Kaplan-Meier Estimate
- Male
- Middle Aged
- Plasma Exchange
(methods)
- Proportional Hazards Models
- Retrospective Studies
- Time
- Young Adult
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