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Background: Although outcome of patients with AAV improved significantly over the past years, an important proportion of patients are still facing with risk of relapse, organ damage, and mortality.
Objectives: In this study, we aimed to evaluate the factors affecting development of damage and mortality in patients with AAV followed up at our tertiary referral center.
Methods: Data of patients with AAV who fulfilled Chapel Hill Consensus Conference criteria were included into this retrospective study. Patients were divided into c-ANCA/PR3(+) and p-ANCA/MPO(+) groups, and clinical data were compared.Permanent organ damage was recorded according to vasculitis damage index (VDI) [1].
Results: The data of 254 patients (53.5% female) were included into the analysis.Clinical diagnosis was GPA in 186 (73.2%) and MPA in 68 (26.8%) patients.Remission status was evaluated in 174 patients and 107 (64.1%) were in remission after induction treatment.Twenty-seven patients (16.2%) had active disease,14 patients (8.4%) had persistent disease and 19 patients (11.4%) were in partial remission at 6 th months.During the follow-up, 217 of 242 (89.7%) patients developed organ damage and median VDI score of the cohort was 2 (IQR: 2). VDI scores were higher in the first period (1997-2011) than in the second period (2011-2021) for the entire cohort and in patients with GPA compared to MPA (p=0.034).VDI scores were weakly correlated with baseline BVAS scores (r=0.247; p=0.004), creatinine levels at baseline (r=0.218; p=0.004), and older age (r=0.2; p=0.002).VDI scores were higher in patients who died or relapsed (p=0.001 and p<0.001, respectively). Development of cardiovascular events (CVE) was 16% and tended to be higher in patients with c-ANCA/PR3(+) compared to those with p-ANCA/MPO(+) (20.5% vs 10.6%; p=0.06). Venous thromboembolism developed in 21 patients (8.8%) and avascular necrosis (AVN) was detected in 35 patients (15%). Twenty-two malignancies were observed in 20 patients (20/240, 8.3%; 11 c-ANCA/PR3(+), 3 in p-ANCA/MPO(+) and 2 in ANCA negative patients) which were 3 lung, 7 genitourinary tract, 5 hematological, 3 thyroid, 2 breast, 1 sarcoma and one in oral cavity. A significant association was observed between malignancy and CVE (95% confidence interval: 2.2-83; OR:13.4, p=0.005) in multivariate analysis. Five-year and overall survival rates were 88.1% and 80.3% for entire cohort; 87.8% and 81% in c-ANCA/PR3(+);86.2% and 76% in p-ANCA/MPO(+); 91% and 84.5% in GPA; 81% and 67.2% in MPA patients(Log-Rank: p<0.001).Development of malignancy, severe infection, and active/persistent disease after induction phase were associated with higher mortality rates.
Conclusion: In our AAV cohort permanent organ damage was detected in the majority of the patients. Although median VDI score decreased over time, mortality did not change.Mortality was associated with advanced age, diagnosis of MPA, malignancy, severe infections, higher VDI scores, and active/persistent disease at 6 th month.Patients should be screened for risk factors for cardiovascular disease and malignancies, especially for the genitourinary tract in patients with AAV.
Baseline clinical and laboratory features of the patients with ANCA-associated vasculitis
| Variables | Total (n=254) | c-ANCA/PR3 + (n=131) | p-ANCA/MPO + (n=88) | p-value (OR) |
|---|---|---|---|---|
| Age, years* | 55.6± 14.1 (17-88) | 52.5±13.2 (23-81) | 60±14.9 (17-88) | <0.001 |
| Gender, female ⱡ | 136 (53.5) | 57 (43.5) | 56 (63.6) | 0.003 (8.5 ) |
| Organ involvement ⱡ | ||||
| Upper respiratory tract | 140/246 (56.9) | 98/129 (76) | 21/85 (24.7) | <0.001 (54.5 ) |
| Ear nose throat | 64/244 (26.2) | 48/128 (37.5) | 10/85 (11.8) | <0.001 (17.1 ) |
| Eye | 40/246 (16.3) | 32/129 (24.8) | 4/86 (4.7) | <0.001 (15 ) |
| Lower respiratory tract | 186/250 (74.4) | 105/129 (81.4) | 60/88 (68.2) | 0.025 (5 ) |
| Alveolar hemorrhage | 33/247 (13.4) | 15/129 (11.6) | 16/87 (18.4) | 0.16 |
| Interstitial lung disease | 44/192 (22.9) | 18/96 (18.8) | 24/75 (32) | 0.046 (4 ) |
| Heart | 16/248 (6.5) | 9/131 (6.9) | 7/87 (8) | 0.7 |
| Kidney involvement | 191/252 (75.8) | 100/131 (76.3) | 74/88 (84.1) | 0.16 |
| Central nervous system | 16/248 (6.5) | 8/131 (6.1) | 6/87 (6.9) | 0.8 |
| Peripheral nervous system | 51/247 (20.6) | 24/130 (18.5) | 20/87 (23) | 0.4 |
| Mononeuritis multiplex | 24/246 (9.8) | 12/130 (9.2) | 9/87 (10.3) | 0.8 |
| Gastrointestinal system | 13/248 (5.2) | 9/131 (6.9) | 2/87 (2.3) | 0.13 |
| Skin | 55/246 (22.4) | 35/129 (27.1) | 11/87 (12.6) | 0.01 (6.5 ) |
| BVAS score* | 17±6.9 (3-40) | 17.5±7.2 (5-40) | 17.5±5.7 (7-30) | 1 |
| Relapse ⱡ | 81/218 (35.1) | 51/120 (42.5) | 24/83 (28.9) | 0.049 (3.9 ) |
| Severe infection ⱡ | 83/218 (38.1) | 49/118 (41.5) | 30/78 (38.5) | 0.7 |
| VDI score ĸ | 2 (2) | 2 (3) | 2 (2) | 0.9 |
| Mortality ⱡ | 50 (19.7) | 24 (18.3) | 21 (23.9) | 0.3 |
*mean± standard deviation (range), ⱡ n (%), ĸ median (interquartile range)
BVAS:Birmingham vasculitis activity score, VDI:Vasculitis damage index, OR:Odds ratio
Univariate analysis of treatment modalities, damage and mortality in patients with ANCA-associated vasculitis according to the first and second periods (before and after 2011)
| Variables | First period (n=60) | Second period (n=194) | p-value (OR) |
|---|---|---|---|
| Presence of any VDI item ⱡ | 55/58 (94.8) | 162/184 (88) | 0.14 |
| VDI score ĸ | |||
| Total | 3 (3) | 2 (3) | 0.012 |
| GPA | 3 (3) | 2 (3) | 0.034 |
| MPA | 4 (4) | 2 (2) | 0.064 |
| Cumulative steroid (MP) dose ĸ | 7.7 (7.5) | 7.5 (9.9) | 0.5 |
| CYC treatment at induction phase ⱡ | 48/55 (87) | 136/180 (76) | 0.065 |
| Cumulative CYC dose ĸ | 12 (32) | 5 (5.8) | 0.006 |
| Remission at 6th month ⱡ | 24/37 (64.9) | 83/130 (63.8) | 0.9 |
| Relapse ⱡ | 26/54 (48) | 55/177 (31) | 0.02 (5.3 ) |
| Severe infection ⱡ | 17/47 (36.2) | 66/171 (38.6) | 0.8 |
| Mortality ⱡ | 7 (11.7) | 43 (22.2) | 0.074 |
*mean± standard deviation (range), ⱡ n (%), ĸ median (interquartile range)
MP:Methylprednisolone, CYC:Cyclophosphamide, eGFR:Estimated glomerular filtration rate, ESRD: End-stage renal disease.
REFERENCES: [1] Exley AR et al.Damage occurs early in systemic vasculitis and is an index of outcome.QJM:monthly journal of the Association of Physicians.1997;90(6):391-9.
Acknowledgements: NIL.
Disclosure of Interests: None declared.
© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (