EULAR 2016 | Daily Highlights
PRONOSTIC FACTORS OF SURVIVAL IN ANCA-ASSOCIATED VASCULITIS (AAV). CHANGES IN THE NEW CENTURYAbstract: FRI0362
Authors: M. Rodriguez-Carballeira1,*, G. Fraile2, F. Martinez-valle3, L. Saez4, J. J. Rios5, X. Solanich6, F. Pasquau7, E. Fonseca8, M. Zamora9, J. L. Calleja10, J. Oristrell11, B. Frutos12, M. Abdilla13, M. J. Castillo14, L. Caminal15, P. Fanlo16, I. Garcia-Sanchez17, B. Sopeña18, M. Lopez-Dupla19, A. Perez20, R. Solans-Laqué3 on behalf of REVAS Study from GEAS-SEMI
Co Authors: 1Internal Medicine, H Mutua Terrassa, Terrassa, 2Internal Medicine, H Ramon y Cajal, Madrid, 3Internal Medicine, H Vall Hebron, Barcelona, 4Internal Medicine, H Miguel Servet, Zaragoza, 5Internal Medicine, H La Paz, Madrid, 6Internal Medicine, H Bellvitge, Barcelona, 7Internal Medicine, H Marina Baixa, Alicante, 8Internal Medicine, H Cabueñes, Asturias, 9Internal Medicine, H Virgen Nieves, 10Internal Medicine, H San Cecilio, Granada, 11Internal Medicine, H Parc Tauli, Sabadell, 12Internal Medicine, H Fuenlabrada, Madrid, 13Internal Medicine, H Alzira, Valencia, 14Internal Medicine, H Virgen Rocio, Sevilla, 15Internal Medicine, HUCA, Asturias, 16Internal Medicine, Clinica Navarro, Navarra, 17Internal Medicine, H Infanta Leonor, Madrid, 18Internal Medicine, CHUVI, Vigo, 19I Medicine, H Joan XXIII, Tarragona, 20I Medicine, H Orense, Orense, Spain
AAV are still related to a high morbidity and mortality in spite of new available therapies. To recognize factors associated with a worse prognosis is essential to tailor the treatment
To identify survival prognostic factors in a large cohort of patients with AAV from Spain and analyze changes in the new century
multicenter retrospective-longitudinal study that included patients diagnosed with AAV between Jan95 and Nov14 in 20 Hospitals from Spain (REVAS Study). Statistical analysis was done using SPSS vs21
455 patients were included:188(41.3%) GPA,167(36.7%) MPA,100(22%) EGPA. Mean age at diagnosis was 55.7±17.2 y. ANCA were positive in 86.8% of cases:35.8% C-ANCA and 51% P-ANCA. Median time to diagnosis was 4 weeks (IQR 10). Median follow-up was 80 months (IQR 105). Renal, lung, neurological and cardiac involvement were present in 56.7, 54.7%, 34.5% and 12.7% of cases,respectively. Pulmonary-renal syndrome was observed in 17%, ENT in 44.8% and eye involvement in 13.4%. Mean BVAS at diagnosis was 17.7±7.7. All patients received oral glucocorticoids; 342 (75%) cyclophosphamide (50.3% oral), and 6.6% biological therapy. Dialysis was required in 16.4% of cases and plasma exchange in 7%. During follow-up, 22.2% of patients suffered leucopenia, 39% bacterial infections, 12.7% opportunistic infections and 5.3% neoplasms. One hundred thirty(28.6%) patients died: 74(44.3%) with PAM, 42(22.3%) with GPA and 14(14%) with EGPA (p<0.001). Median time to death was 57.5 months (IQR 105): significantly higher for EGPA (94m vs 69m for GPA and 50m for PAM;LogRank=0.01). Comparing diagnostic century, the percentage of deaths was higher in the XXth than in XXIth (48% vs 21%, p<0.001). No differences were found in clinical manifestations, but yes in treatment and rate of infections: in the actual century more plasma exchange is used (9% vs 2.2%, p=0.008), more biological therapy (9% vs 0.7%, p<0.001), less oral CF (29% vs 57.8%, p<0.001) and more CF IV pulses (52% vs 35.6%, p=0.001). Multifactorial Cox regression analysis showed that prognostic factors associated with survival were age>65y (HR 4.6), cardiac (HR 3.9) and renal involvement (HR 2.0). ENT involvement was a protector factor (HR 0.4). Bacterial infections during follow-up were also independent predictor of poor prognosis (HR 2.0)
survival rate in AAV has significantly improved in the new century probably due to new treatment strategies, including less CF and biological therapy. Poor prognostic factors are age>65y, cardiac and renal involvement at diagnosis,and bacterial infections, while ENT involvement is protective.
Disclosure of Interest
None declared DOI: 10.1136/annrheumdis-2016-eular.3762
The authors report about treatment and outcome of a large cohort of patients with ANCA vasculitis (455 patients). It includes a surprisingly high %-age of eGPA (22% of cases). Of note, more than half of the patients suffered from pulmonary or renal involvement (17% with classical pulmonary-renal syndrome), followed by neurological and cardiac involvement (34.5% and 12.7%, respectively). In 75% of cases CYC was used, in 50.3% in oral form. 130 patients died; as expected MPA showed a worse prognosis than GPA and eGPA (44% versus 22% versus 14%).
The comparison of data of the XXth with data of the XXIth century showed a decrease in mortality: 48% versus 21%; more frequent use of plasma exchange: 9% versus 2.2%; current use of biological therapy in 9% of cases; a reduction in oral CYC prescription: 29% versus 57.8%.
The prognostic factors correspond with the published Five Factor Score, i.e. age>65y, cardiac and renal involvement as negative factors and ENT involvement as a protective factor. Remarkably, the authors do not comment GI-involvement as a negative factor, but they add bacterial infections to the list of negative factors.
In conclusion, the data differ in several aspects from other large cohorts and thereby add valuable information. The use of biologic agents appears low and the prescription of CYC in oral form remains high. However, as rituximab has been introduced few years ago, the data of the XXIth century probably do not represent current treatment strategies in Spain. It would have been interesting to display the data of the XXIth in 5 year intervals.
Prof. Dr. Peter Villiger
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