Author/Editor		Monti, Sara; Craven, Anthea; Klersy, Catherine; Montecucco, Carlomaurizio; Hočevar, Alojzija; Rotar, Žiga; Tomšič, Matija
Title		Association between age at disease onset of anti-neutrophil cytoplasmic antibody‐associated vasculitis and clinical presentation and short-term outcomes
Type		članek
Vol. and No.		Letnik 60, št. 2
Publication year		2021
Volume		str. 617-628
ISSN		1462-0324 - Rheumatology (Oxford, England)
Language		eng
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 s65 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 s65 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 s5, 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.
Keywords		vaskulitis nevtrofilci citoplazmatska protitelesa vasculitis neutrophil cytoplasmic antibodies