RESUMO
INTRODUCTION: Prevention of attacks is a major goal in management of patients with hereditary angioedema (HAE). We aimed to investigate the effects of a systematic intervention for HAE patients. METHODS: Thirty-three patients with HAE with C1-inhibitor deficiency, belonging to a single family, participated in a management program coordinated by an allergist/immunologist. Angioedema attacks before intervention were ascertained by interviews and emergency room charts and recorded prospectively by patients or caregivers after enrollment. Mean number of attacks/month was compared at 12 months preintervention and 8 and 14 months within intervention. Patient-reported outcome instruments were used to assess quality of life, including HAE Quality of Life (HAE-QoL) questionnaire, psychological conditions, and work impairment, at baseline and 8 and 14 months within intervention. Data were stored in REDCap platform and analyzed by adjusted Bayesian models of double Poisson regression. RESULTS: Mean number of attacks/month significantly decreased (95% credible interval [CrI] excluding 0) from 1.15 preintervention to 0.25 and 0.23, 8 and 14 months within intervention, with mean decreases of -0.89 (95% CrI: -1.21 to -0.58) and -0.92 (95% CrI: -1.22 to -0.60), respectively. HAE-QoL scores showed mean total increases of 15.2 (95% CrI: 1.23-29.77) and 26 (95% CrI: 14.56-39.02) at 8 and 14 months within the study, as compared to baseline, revealing marked improvement in quality of life. Significant increase in role-emotional and reduction of depression, stress, and anxiety were observed at 14 months. CONCLUSION: A systematic approach integrating HAE-specific care with effective handling of psychological issues decreased the number of attacks and improved quality of life, targets for best practice in HAE.
Assuntos
Angioedemas Hereditários/epidemiologia , Qualidade de Vida , Angioedemas Hereditários/prevenção & controle , Angioedemas Hereditários/psicologia , Angioedemas Hereditários/terapia , Ansiedade , Teorema de Bayes , Gerenciamento Clínico , Progressão da Doença , Emoções , Pesquisas sobre Atenção à Saúde , Humanos , Inquéritos e QuestionáriosRESUMO
Urticária é uma doença pruriginosa da pele na qual ocorrem urticas e/ou angioedema. A urticária é definida como crônica quando persiste por 6 semanas ou mais. A urticária crônica tem um grande impacto na vida diária do paciente. Atualmente, não há biomarcadores confiáveis para identificar e medir a atividade da doença na urticária crônica espontânea. Consequentemente, o uso de ferramentas conhecidas por patient-reported outcomes (PROs) é crucial ao avaliar e monitorar diferentes aspectos da urticária crônica, como atividade/gravidade da doença, controle da doença e qualidade de vida. Apresentamos uma visão geral de cinco PROs usados na avaliação da urticária crônica, e destacamos suas vantagens, limitações e uso na prática clínica e pesquisa.
Urticaria is an itching skin disease characterized by the presence of wheals and/or angioedema. Urticaria is defined as chronic when it persists for 6 weeks or more. Chronic urticaria has great impact on the daily lives of patients. Currently, there are no reliable biomarkers to identify and measure disease activity in chronic spontaneous urticaria. Consequently, the use of tools known as patient-reported outcomes (PROs) is crucial when evaluating and monitoring different aspects of chronic urticaria such as disease activity/severity, disease control, and quality of life. We present an overview of the five PROs used in the evaluation of chronic urticaria, highlighting their advantages, limitations, and use in clinical practice and research.