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 Watts, Peter Merkel, Raashid A. Luqmani, Katerina Achilleos, Matthew Adler, Marco A. Alba, Daniel A. Albert, Fatma Alibaz-Oner, Paul Allcoat, Koichi Amano, Manishka Amarasuriya, Naomi A. Amudala, Jacqueline Andrews, Amy M. Archer, Yoshihiro ArimuraInoshi 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, Daniel Blockmans, Janet Blood, Juliana Bordignon Draibe, Sarah Brand, Mariana Brandao, Ian N. Bruce, Amanda Butler, Leonard H. Calabrese, Daniel Camprubi Ferrer, Simon Carette, Diana Carmona, Helga Ceunen, Kuntal Chakravarty, Peter T. Chapman, Zdenka Chocova, Sharon A. Chung, Weiping Ci, Maria C. Cid, Tiffany M. Clark, Michael R. Clarkson, Felipe De Jesus Contreras-Rodríguez, Richard Conway, Kelly Cooke, Xavier Corbella Virós, Ana Cordeiro, Andreia Costa, Anthea Craven, Karen Culfear, Thomas Daikeler, Debashish Danda, Siddharth K. Das, Bhaskar Dasgupta, Alice M. De Castro, Natasha Dehghan, Roni Devassy, Navjot Dhindsa, Andreas P. Diamantopoulos, Haner Direskeneli, Hiroaki Dobashi, Du Juan, Maumer Durrani, Clive Edelsten, Johanna Eifert, Sallie Elhayek, Sunhoury Elsideeg, Tomomi Endo, Abdulsamet Erden, Burak Erer, Per Eriksson, Zeynep Erturk, Georgina Espígol-Frigolé, Mara Felicetti, Alaistair Ferraro, José M. Ferro, Aurore Fifi-Mah, Luis Felipe Flores-Suárez, Oliver Flossmann, Deirdre Flynn, Joaõ Eurico Fonseca, Jayne Foot, Michelle Foote, Lindsy Forbess, Shouichi Fujimoto, Kazuhito Fukuoka, Carolina Furtado, Shunsuke Furuta, Angelo L. Gaffo, Phil Gallagher, Na Gao, Paul Gatenby, Nagui Gendi, Ruth Geraldes, Anneleen Gerits, Andrea Gioffredi, Luke Gomples, Maria Joaõ Goncąlves, Prisca Gondo, Anne Graham, Rebecca Grainger, David T. Gray, Peter C. Grayson, Laura Griffiths, Yanqiu Guo, Rajiva Gupta, Micael Gylling, Rula A. Hajj-Ali, Nevin Hammam, Masayoshi Harigai, Lorraine Hartley, Janine Haslett, Alaa Hassan, Gulen Hatemi, Bernhard Hellmich, Liesbet Henckaerts, Joerg C. Henes, Joanna Hepburn, Vera Herd, Christoph Hess, Catherine Hill, Andrea Hinojosa-Azaola, Junichi Hirahashi, Fumio Hirano, Alojzija Hočevar, Julia Holle, Nicole Hollinger, Sakae Homma, Theresa Howard, Rachel K. Hoyles, Zdenka Hruskova, Gayle Hutcheon, Maria Ignacak, Annette Igney-Oertel, Kei Ikeda, Noriko Ikegaya, Samyukta Jagadeesh, Jane Jaquith, David R.W. Jayne, Teresa Jewell, Colin Jones, Abhay Joshi, Umut Kalyoncu, Sevil Kamall, Sanjeet Kamath, Kan Sow Lai, Shinya Kaname, Suresh Kanchinadham, Ömer Karadaǧ, Miho Karube, Marek Kaszuba, Ramanjot Kaur, Tamihiro Kawakami, Soko Kawashima, Nader Khalidi, Asad Khan, Masao Kikuchi, Levent Kilic, Makiko Kimura, Maria J. King, Sebastian Klapa, Rainer Klocke, Tatsuo Kobayashi, Shigeto Kobayashi, Yoshinori Komagata, Andreas Kronbichler, Pawel Kuczia, Mandal Santosh Kumar, Miho Kurosawa, Peter Lamprecht, Carol A. Langford, Peter Lanyon, Catherine Laversuch, Sang Jin Lee, Simona Leoni, Jing Li, Kimberly Liang, Patrick Liang, Hua Liao, Lim Ai Lee, Raashid A. Luqmani, Amanda Lyle, Matthew Macdonald, Sarah L. Mackie, Leah Madden, Malgorzata Magliano, Hirofumi Makino, Ashima Makol, Ritu Malaiya, Anshuman Malaviya, Ramesh Manthri, Federica Maritati, Ana Martins Da Silva, Justin C. Mason, Cecilia Matara, Kazuo Matsui, Eric L. Matteson, Dawn Mcbride, Keith Mccullough, Lucy Mcgeoch, John Mclaren, Caitlin Mcmillian, Naval Mendiratta, Ajit Menon, Dimos Merinopoulos, Peter Merkel, Peter Merkel, Sandra Messier, Robert G. Micheletti, Karen Mills, Nataliya Milman, Masahiro Minoda, Ranjana Walker Minz, Claudia Möck, Aladdin J. Mohammad, Sergey Moiseev, Marta Moitinho, Eamonn Molloy, Paul A. Monach, Marian Montgomery, Frank Moosig, Manoosh Moradizadeh, Matthew Morgan, Ann W. Morgan, Ann Marie Morgan, Alice Muir, Chetan Mukhtyar, Antje Müller, Francesco Muratore, Eri Muso, Ritambhra Nada, Hiroshi Nakajima, Toshiki Nakajima, Hiroto Nakano, Anupapama Nandagudi, Thomas Neumann, Ying Fun Ng, Kooi Heng Ng, Estela L. Nogueira, Nilesh Nolkha, Dan Nordström, Pavel Novikov, Asanka Nugaliyadde, John L. O'donnell, Jennifer O'donoghue, Lorraine O'neill, Edmond O'riordan, Margaret Oatley, Koshu Okubo, Elena Oliva, Hideto Oshikawa, Yuichiro Ota, Roberto Padoan, Christian Pagnoux, Lili Pan, Kalliopi Panaritis, Jin Kyun Park, Sanjeev Patel, Pravin Patil, Giulia Pazzola, Adrian Peall, Fiona Pearce, Seval Pehlevan, Liliana Pereira, Tom Pettersson, Christian A. Pineau, Laura Pirilä, Bartlomiej Poglodek, Cristina Ponte, Sergio Prieto-González, Sangeetha R. Priya, Bally Purewal, Silke Purschke, Jukka Putaala, Stefanie Quickert, Vicki Quincey, Subhra Raghuvanshi, Liza Rajasekhar, Dwarakanathan Ranganathan, Manish Rathi, David Rees, Frances Rees, Ulrike Renken, Giovanna Restuccia, Rennie L. Rhee, Brian Rice, Diane Robins, Joanna Robson, Miguel Rodrigues, Vasco C. Romaõ, Ciga Rotar, Carlee Ruediger, Abraham Rutgers, Ana C. Sá, Maria Joaõ Saavedra, Ken Ei Sada, Ilfita Sahbudin, Carlo Salvarani, Namneet Sandhu, Ernestina Santos, Yuji Sato, Valentin S. Schäfer, Franco Schiavon, Wolfgang A. Schmidt, Mårten Segelmark, Amira Shahin, Aman Sharma, Julie Shotton, Cristiana Silva, Ora Gewurz Singer, Goutham Sivasuthan, Susan Smolen, Xavier Solanich-Moreno, Laura Soldevila Boixader, Yeong Wook Song, Jason Springer, Antoine G. Sreih, Ragini Srivastava, Lisa K. Stamp, Robert Stevens, Daniel Strbian, Keishi Sugino, Cord Sunderkötter, Ravi Suppiah, Katsuya Suzuki, Kazuo Suzuki, Zoltán Szekanecz, Jan Sznajd, Kirsi Taimen, Paul P. Tak, Tsutomu Takeuchi, Naoho Takizawa, Lilian Tames, Bee Eng Tan, Mototsugu Tanaka, Man Wai Tang, Turgut Tatlisumak, Vladimir Tesar, Alan Thomas, Xinping Tian, Kenichiro Tokunaga, Enrico Tombetti, Matija Tomšič, Bahtiyar Toz, Tatsuo Tsukamoto, Shunya Uchida, Ali Ugur Unal, Maria L. Urban, Joichi Usui, Augusto Vaglio, Srinivasan Venkatachalam, Erin Vermaak, Vishad Viswanath, Takashi Wada, Shrikant Wagh, Daniel J. Wallace, Giles Walters, Bastian Walz, Jin Wan, Tian Wang, Guochun Wang, Kenneth J. Warrington, Richard A. Watts, Katarzyna Wawrzycka-Adamczyk, Praveen Weeratunga, Michael H. Weisman, Sugeesha Wickramasinghe, Mark Williams, Megan Williams, Krzysztof Wojcik, Laticia Woodruff, Theodoros Xenitidis, Hidehiro Yamada, Kunihiro Yamagata, Chee Seng Yee, Myeongjae Yoon, Kazuki Yoshida, Hajime Yoshifuji, Steven R. Ytterberg, Wako Yumura, Hania Zayed, Xiaofeng Zeng, Ming Hui Zhao, Anna Zugaj, Joanna Zuk

*Corresponding author for this work
3 Citations (Scopus)

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- A nd 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.

Original languageEnglish
JournalRheumatology (United Kingdom)
Volume60
Issue number2
Pages (from-to)617-628
Number of pages12
ISSN1462-0324
DOIs
Publication statusPublished - 01.02.2021

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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