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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.

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RESUMOO penfigoide bolhoso é uma patologia cutânea autoimune adquirida, mais frequente nos idosos. Estudos recentes demonstraram a associação causal entre a ocorrência de penfigoide bolhoso em doentes com síndromes demenciais. Apresentamos o caso de uma doente do sexo feminino, 80 anos, com antecedentes de doença de Parkinson, internada por dermatose bolhosa extensa, constituída por placas urticariformes e bolhas tensas com halo eritematoso, sem envolvimento das mucosas. Após confirmação por biópsia cutânea, imunofluorescência direta positiva e exclusão de outras causas, foi feito o diagnóstico de penfigoide bolhoso. Foi instituída terapêutica com prednisolona, azatriopina e doxiciclina, verificando-se evolução clínica favorável. Consideramos o presente caso clínico pertinente, pois realça a importância do diagnóstico precoce de penfigoide bolhoso. O atraso no início do tratamento pode levar a complicações graves, com elevada morbi-mortalidade. PALAVRAS-CHAVE