Association between age at disease onset of anti-neutrophil cytoplasmic antibody–associated vasculitis and clinical presentation and short-term outcomes
Sara Monti, Anthea Craven, Catherine Klersy, Carlomaurizio Montecucco, Roberto Caporali, Richard A. Watts, Peter A. Merkel, Raashid Luqmani, DCVAS Collaborators, Katerina Achilleos, Matthew D. Adler, Marco A. Alba, Marco A. Alba, Daniel Albert, Fatma Alıbaz-Öner, Paul Allcoat, Koichi Amano, M. Amarasuriya, Naomi A. Amudala, Jacqueline Andrews, Amy M. Archer, Yoshihiro Arimura, Inoshi Atukorala, Elsa Azevedo, Shruti Bajad, Corisande Baldwin, Lillian J. Barra, Bo Baslund, Neil Basu, Mahire Baykal, Christoph Berger, Ewa Berglin, Emilio Besada, Mamta Bhardwaj, Antje Bischof, Daniël Blockmans, Janet Blood, Juliana Draibe, Sarah Brand, Mariana Brandão, Ian N Bruce, Amanda Butler, Leonard H. Calabrese, Daniel Camprubi Ferrer, Simon Carette, Diana Karina Delgado Carmona, Helga Ceunen, Kuntal Chakravarty, Peter T. Chapman, Zdeňka Chocová, Sharon A. Chung, Weiping Ci, María C. Cid, Tiffany Clark, Michael R. Clarkson, Felipe de Jesús Contreras-Rodríguez, Richard Conway, Kelly Cooke, Xavier Corbella Virós, Ana Cordeiro, Andreia Costa, Anthea Craven, Karen Culfear, Thomas Daikeler, Debashish Danda, Siddharth Das, Bhaskar Dasgupta, Alice M De Castro, Natasha Dehghan, Roni Devassy, Navjot Dhindsa, Andreas P. Diamantopoulos, Haner Di̇reskeneli̇, Hiroaki Dobashi, Juan Du, Maumer Durrani, Clive Edelsten, J. Eifert, Sallie Elhayek, Sunhoury Elsideeg, Tomomi Endo, Abdülsamet Erden, Burak Erer, Per Eriksson, Zeynep Ertürk, Georgina Espígol‐Frigolé, Mara Felicetti, Alaistair Ferraro, José M. Ferro, Aurore Fifi‐Mah, Luis Felipe Flores-Suárez, Oliver Floßmann, Deirdre Flynn, João Eurico Fonseca, Jayne Foot, Michelle Foote, Lindsy Forbess, Shouichi Fujimoto, Kazuhito Fukuoka, Carolina Furtado
Abstract
OBJECTIVES: ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. METHODS: We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). RESULTS: A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. CONCLUSION: Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.