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ANCA-associated vasculitis (AAV) is a group of autoimmune diseases that affect multiple orgysubtype (MPA, GPA, EGPA), organ involvement (kidney and lung) and ANCA type (MPO or PR3) that may influence the mortality risk of patients (pts).
The clinicopathological variants of AAV are categorized based on clinical, laboratory and pathologic findings: Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA).
Aim:To analyze the relationships between orgysubtype, organ involvement and ANCA type of AAV pts who died during follow-up.
Retrospective observational study including consecutive pts older than 18 years of age with a diagnosis AAV over a 12-year period (2012-2024). All pts met the American College of Rheumatology criteria for EGPA and GPA and the criteria of the last Chapell Hill CoTansensus Conference in 2012 for these vasculitides and MPA. Demographic variables, follow-up time, and mortality were analyzed.
Thirty-four pts were included in this study. Mean follow-up of 55.8± 45.84 months. Mean age at baseline was 67.21± 14.78 years (range, 33-92 years). Female to male ratio was 16:18. Eight pts (23.5%) met the criteria for GPA, three pts (8.8%) for EGPA and twenty-three (67.6%) for MPA. Kidney involvement (KI) was the most frequently manifestation, present in almost all MPA and in most GPA compared to EGPA (95.6%, 75%, 33.3% respectively, p=0.011). Lung involvement (LI) was more frequent in GPA and EGPA (87.5%, 66.6%, respectively), and it occurred in one third of MPA cases (34.8%) (p=0.031).
Nine pts died (26.5%), 55.6% were male. The mean age of survivors and those who died was 65.4±14.7 years and 73.3±12 years, respectively (p=0.13). Classification of deceased pts according to subtypes: 4 were affected by MPA (17.4%), 4 by GPA (50%) and 1 by EGPA (33.3%) (p=0.19). The mean follow-up time was 32.5±14.8 months. KI was the most prominent clinical finding (87.5% of cases) and LI was present in 75% of pts. Mean creatinine and proteinuria were 7.1±2.8 mg/dl and 1.4±1.1 g/day, respectively. MPO antibody was more prevalent than PR3 (66.7% vs 33.3%, p=0.068), Hazard ratio=0.188 (95% confidence interval CI): 0.037 - 0.968, p=0.078).
Table 1 shows the multivariate logistic regression analysis performed to identify factors associated with mortality risk:
1) The average age of deceased pts was higher than survivors. 2) Although the wide confidence interval suggesting variability, the risk of mortality was lower for MPO-positive pts compared to PR3-positive pts. 3) Advanced age, decreased kidney function (eGFR) and higher proteinuria levels were significant risk factors for mortality. On the contrary, higher hemoglobin levels and a diagnosis of MAP were associated with a better prognosis. These results underscore the importance of monitoring specific clinical markers in the management of this pts.
