Association between age at disease onset of anti-neutrophil cytoplasmic antibody–associated vasculitis and clinical presentation and short-term outcomes
Authors: Monti Sara , Craven Anthea , Klersy Catherine , Montecucco Carlomaurizio , Caporali Roberto , Watts Richard , Merkel Peter A , Luqmani Raashid ,   DCVAS Collaborators, Achilleos Katerina , Adler Matthew , Alba Marco A , Alba Marco A , Albert Daniel A , Alibaz-Oner Fatma , Allcoat Paul , Amano Koichi , Amarasuriya Manishka , Amudala Naomi A , Andrews Jacqueline , Archer Amy M , Arimura Yoshihiro , Atukorala Inoshi , Azevedo Elsa , Bajad Shruti , Baldwin Corisande , Barra Lillian J , Baslund Bo , Basu Neil , Baykal Mahire , Berger Christoph , Berglin Ewa , Besada Emilio , Bhardwaj Mamta , Bischof Antje , Blockmans Daniel , Blood Janet , Draibe Juliana Bordignon , Brand Sarah , Brandao Mariana , Bruce Ian N , Butler Amanda , Calabrese Leonard H , Ferrer Daniel Camprubi , Carette Simon , Carmona Diana , Ceunen Helga , Chakravarty Kuntal , Chapman Peter T , Chocova Zdenka , Chung Sharon A , Ci Weiping , Cid Maria C , Clark Tiffany M , Clarkson Michael R , de Jesus Contreras-Rodríguez Felipe , Conway Richard , Cooke Kelly , Virós Xavier Corbella , Cordeiro Ana , Costa Andreia , Craven Anthea , Culfear Karen , Daikeler Thomas , Danda Debashish , Das Siddharth K , Dasgupta Bhaskar , De Castro Alice M , Dehghan Natasha , Devassy Roni , Dhindsa Navjot , Diamantopoulos Andreas P , Direskeneli Haner , Dobashi Hiroaki , Juan Du , Durrani Maumer , Edelsten Clive , Eifert Johanna , Elhayek Sallie , Elsideeg Sunhoury , Endo Tomomi , Erden Abdulsamet , Erer Burak , Eriksson Per , Erturk Zeynep , Espígol-Frigolé Georgina , Felicetti Mara , Ferraro Alaistair , Ferro José M , Fifi-Mah Aurore , Flores-Suárez Luis Felipe , Flossmann Oliver , Flynn Deirdre , Fonseca João Eurico , Foot Jayne , Foote Michelle , Forbess Lindsy , Fujimoto Shouichi , Fukuoka Kazuhito , Furtado Carolina , Furuta Shunsuke , Gaffo Angelo L , Gallagher Phil , Gao Na , Gatenby Paul , Gendi Nagui , Geraldes Ruth , Gerits Anneleen , Gioffredi Andrea , Gomples Luke , Gonçalves Maria João , Gondo Prisca , Graham Anne , Grainger Rebecca , Gray David T , Grayson Peter C , Griffiths Laura , Guo Yanqiu , Gupta Rajiva , Gylling Micael , Hajj-Ali Rula A , Hammam Nevin , Harigai Masayoshi , Hartley Lorraine , Haslett Janine , Hassan Alaa , Hatemi Gulen , Hellmich Bernhard , Henckaerts Liesbet , Henes Joerg C , Hepburn Joanna , Herd Vera , Hess Christoph , Hill Catherine , Hinojosa-Azaola Andrea , Hirahashi Junichi , Hirano Fumio , Hočevar Alojzija , Holle Julia , Hollinger Nicole , Homma Sakae , Howard Theresa , Hoyles Rachel K , Hruskova Zdenka , Hutcheon Gayle , Ignacak Maria , Igney-Oertel Annette , Ikeda Kei , Ikegaya Noriko , Jagadeesh Samyukta , Jaquith Jane , Jayne David R W , Jewell Teresa , Jones Colin , Joshi Abhay , Kalyoncu Umut , Kamalı Sevil , Kamath Sanjeet , Lai Kan Sow , Kaname Shinya , Kanchinadham Suresh , Karadağ Ömer , Karube Miho , Kaszuba Marek , Kaur Ramanjot , Kawakami Tamihiro , Kawashima Soko , Khalidi Nader , Khan Asad , Kikuchi Masao , Kilic Levent , Kimura Makiko , King Maria J , Klapa Sebastian , Klocke Rainer , Kobayashi Tatsuo , Kobayashi Shigeto , Komagata Yoshinori , Kronbichler Andreas , Kuczia Pawel , Kumar Mandal Santosh , Kurosawa Miho , Lamprecht Peter , Langford Carol A , Lanyon Peter , Laversuch Catherine , Lee Sang Jin , Leoni Simona , Li Jing , Liang Kimberly , Liang Patrick , Liao Hua , Lee Lim Ai , Luqmani Raashid A , Lyle Amanda , MacDonald Matthew , Mackie Sarah L , Madden Leah , Magliano Malgorzata , Makino Hirofumi , Makol Ashima , Malaiya Ritu , Malaviya Anshuman , Manthri Ramesh , Maritati Federica , da Silva Ana Martins , Mason Justin C , Matara Cecilia , Matsui Kazuo , Matteson Eric L , McBride Dawn , McCullough Keith , McGeoch Lucy , McLaren John , McMillian Caitlin , Mendiratta Naval , Menon Ajit , Merinopoulos Dimos , Merkel Peter A , Merkel Peter , Messier Sandra , Micheletti Robert G , Mills Karen , Milman Nataliya , Minoda Masahiro , Minz Ranjana Walker , Möck Claudia , Mohammad Aladdin J , Moiseev Sergey , Moitinho Marta , Molloy Eamonn , Monach Paul A , Montgomery Marian , Moosig Frank , Moradizadeh Manoosh , Morgan Matthew , Morgan Ann W , Morgan Ann-Marie , Muir Alice , Mukhtyar Chetan , Müller Antje , Muratore Francesco , Muso Eri , Nada Ritambhra , Nakajima Hiroshi , Nakajima Toshiki , Nakano Hiroto , Nandagudi Anupapama , Neumann Thomas , Ng Ying Fun , Ng Kooi Heng , Nogueira Estela L , Nolkha Nilesh , Nordström Dan , Novikov Pavel , Nugaliyadde Asanka , O’Donnell John L , O’Donoghue Jennifer , O’Neill Lorraine , O’Riordan Edmond , Oatley Margaret , Okubo Koshu , Oliva Elena , Oshikawa Hideto , Ota Yuichiro , Padoan Roberto , Pagnoux Christian , Pan Lili , Panaritis Kalliopi , Park Jin Kyun , Patel Sanjeev , Patil Pravin , Pazzola Giulia , Peall Adrian , Pearce Fiona , Pehlevan Seval , Pereira Liliana , Pettersson Tom , Pineau Christian A , Pirilä Laura , Poglodek Bartlomiej , Ponte Cristina , Prieto-González Sergio , Priya Sangeetha R , Purewal Bally , Purschke Silke , Putaala Jukka , Quickert Stefanie , Quincey Vicki , Raghuvanshi Subhra , Rajasekhar Liza , Ranganathan Dwarakanathan , Rathi Manish , Rees David , Rees Frances , Renken Ulrike , Restuccia Giovanna , Rhee Rennie L , Rice Brian , Robins Diane , Robson Joanna , Robson Joanna , Rodrigues Miguel , Romão Vasco C , Rotar Žiga , Ruediger Carlee , Rutgers Abraham , Sá Ana C , Saavedra Maria João , Sada Ken-ei , Sahbudin Ilfita , Salvarani Carlo , Sandhu Namneet , Santos Ernestina , Sato Yuji , Schäfer Valentin S , Schiavon Franco , Schmidt Wolfgang A , Segelmark Mårten , Shahin Amira , Sharma Aman , Shotton Julie , Silva Cristiana , Singer Ora Gewurz , Sivasuthan Goutham , Smolen Susan , Solanich-Moreno Xavier , Boixader Laura Soldevila , Song Yeong Wook , Springer Jason , Sreih Antoine G , Sreih Antoine G , Srivastava Ragini , Stamp Lisa K , Stevens Robert , Strbian Daniel , Sugino Keishi , Sunderkötter Cord , Suppiah Ravi , Suzuki Katsuya , Suzuki Kazuo , Szekanecz Zoltán , Sznajd Jan , Taimen Kirsi , Tak Paul P , Takeuchi Tsutomu , Takizawa Naoho , Tames Lilian , Tan Bee Eng , Tanaka Mototsugu , Tang Man Wai , Tatlisumak Turgut , Tesar Vladimir , Thomas Alan , Tian Xinping , Tokunaga Kenichiro , Tombetti Enrico , Tomšič Matija , Toz Bahtiyar , Tsukamoto Tatsuo , Uchida Shunya , Unal Ali Ugur , Urban Maria L , Usui Joichi , Vaglio Augusto , Venkatachalam Srinivasan , Vermaak Erin , Viswanath Vishad , Wada Takashi , Wagh Shrikant , Wallace Daniel J , Walters Giles , Walz Bastian , Wan Jin , Wang Tian , Wang Guochun , Warrington Kenneth J , Watts Richard A , Wawrzycka-Adamczyk Katarzyna , Weeratunga Praveen , Weisman Michael H , Wickramasinghe Sugeesha , Williams Mark , Williams Megan , Wojcik Krzysztof , Woodruff Laticia , Xenitidis Theodoros , Yamada Hidehiro , Yamagata Kunihiro , Yee Chee-Seng , Yoon Myeongjae , Yoshida Kazuki , Yoshifuji Hajime , Ytterberg Steven R , Yumura Wako , Zayed Hania , Zeng Xiaofeng , Zhao Ming-Hui , Zugaj Anna , Zuk Joanna 
Published: April 2020

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: &lt;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 &lt;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 &gt;65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.