External validation of the Damage Index for Antiphospholipid Syndrome (DIAPS) in a non-latin american thrombotic antiphospholipid cohort

Abstract

Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2022Introdução: A escala de dano da Síndrome do Anticorpo Antifosfolípido (SAAF) (DIAPS) está validada em Latino-americanos e correlaciona-se com qualidade de vida relacionada com a saúde (QVRS). Pretendemos validar o DIAPS num coorte português. Métodos: Estudo transversal unicêntrico incluindo todos os doentes com SAAF trombótico. O dano foi avaliado através do DIAPS e a QVRS através das escalas EuroQol de 3 e 5 níveis (EQ-D5-3L and 5L) e Visual Analogue Scale (VAS). Foi realizado um teste de correlação de Spearman entre o DIAPS e as escalas de QVRS. Significância estatística <0.05. Resultados: Incluímos 108 doentes (mulher 65.7%, Caucasianos 90.7%, SAAF primário 75.9%). A doença venosa tromboembólica (48.1%) e os eventos isquémicos cerebrais (42.6%) foram as manifestações mais frequentes. Dano estava presente em 48.2% dos doentes e o DIAPS médio foi 0.74±0.91. O domínio neuropsiquiátrico do DIAPS foi o mais afetado (24.2%), seguido do vascular periférico (20.3%). Utilizando a escala EQ-5D-3L, dificuldades na mobilidade (MO), cuidados pessoais (CP), atividades habituais (AH) foram reportadas em 30.6%, 17.6% e 36.1% dos doentes. A maioria dos doentes (57.4%) reportou dor/desconforto (D/D) e 52.8% ansiedade/depressão (A/D). Os itens do DIAPS correlacionaram-se com os domínios da EQ-5D-3L e EQ-5D-5L de forma semelhante. O DIAPS total correlacionou-se com MO (r=0.264, p=0.006), AH (r=0.248, p=0.009), D/D (r=0.221, p=0.022) e as escalas EQ-5D-3L (r=-0.231, p=0.016) e EQ-5D-5L (r=-0.209, p=0.030), mas não com a VAS. Conclusão: A acumulação de dano, medido pelo DIAPS, correlaciona-se com a QVRS em doentes com SAAF de uma ascendência étnica distinta daqueles nos quais o score foi inicialmente validado, contribuindo para a validação externa do DIAPS.Introduction: The Damage Index for Antiphospholipid Syndrome (APS) (DIAPS) is validated in Latin-americans and correlates with impaired health-related quality of life (HR-QoL). We aimed to externally validate the DIAPS in a Portuguese thrombotic APS cohort. Methods: Cross-sectional study including all thrombotic APS patients attending the Immune-mediated Disease Clinic. Damage was assessed using DIAPS and HR-QoL using the 3 and 5 level EuroQol HR-QoL (EQ-D5-3L and 5L) and Visual Analogue Scale (VAS). Spearman’s correlation between DIAPS and the QoL scales was performed. Significance was set at 0.05. Results: We included 108 patients (female 65.7%, Caucasians 90.7%, primary APS 75.9%). The most common manifestations were thromboembolic venous disease (48.1%) and cerebral ischemic events (42.6%). Damage was present in 48.2% of patients with a mean of 0.74±0.91. DIAPS’ neuropsychiatric domain was the most affected (24.2%), followed by the peripheral vascular (20.3%). Using the EQ-5D-3L scale, impairment in mobility (MO), self-care (SC), and usual activities (UA) were reported by 30.6%, 17.6% and 36.1% of patients. Most patients (57.4%) reported pain/discomfort (P/D) and 52.8% anxiety/depression (A/D). The DIAPS items correlated similarly with the EQ-5D-3L and EQ-5D-5L scales. Total DIAPS correlated with MO (r=0.264, p=0.006), UA (r=0.248, p=0.009), P/D (r=0.221, p=0.022), the EQ-5D-3L (r=-0.231, p=0.016) and EQ-5D-5L (r=-0.209, p=0.030) indexes, but not with VAS. Conclusion: Accrual damage, as measured by the DIAPS, correlates with impaired HR-QoL in APS patients with a distinct genetic background from those in which it was initially validated, allowing us to externally validate the DIAPS