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Symptom-based stratification of patients with Sjögren’s disease
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Introdução: A Doença de Sjögren (DSj) é uma patologia reumática crónica e autoimune caracterizada por sintomas de secura, fadiga e dor. Tratando-se de uma doença sistémica, pode afetar qualquer sistema de órgãos, e esta importante heterogeneidade fenotípica constitui um desafio na abordagem a estes doentes. Com este estudo, pretendemos investigar uma população de doentes com DSj, agrupados com base nos seus sintomas, de modo a analisar as suas características específicas. Métodos: Incluímos doentes do estudo prospetivo Observational Lisbon Sjögren’s Syndrome Prospective (OLISSIPO) e utilizámos a Newcastle Sjögren's Stratification Tool (NSST), de modo a categorizar os doentes em quatro clusters diferentes: low-symptom burden (LSB), high symptom burden (HSB), pain dominant with fatigue (PDF) and dryness dominant with fatigue (DDF). Por fim, procedemos à comparação de características clínicas, laboratoriais, patológicas e de imagem entre estes grupos. Resultados: Distribuímos 319 pacientes (94,4% do sexo feminino, idade média de 58,5±13,6 anos) pelos quatro grupos: LSB (n=39), HSB (n=95), PDF (n=155) e DDF (n=30). Observámos menor fluxo salivar não estimulado nos grupos DDF (1,3 ml/15min) e HSB (1,8 ml/15min), e maior uso de pilocarpina (37% no HSB e 33% no DDF). O grupo DDF apresentou os títulos mais elevados de anticorpos anti-La/SSB (1084 IU/l), e o grupo PDF os níveis mais altos de proteína C-reativa (0,39 mg/dl). A fibromialgia e a depressão foram mais prevalentes em HSB e menos em LSB e o impacto na qualidade de vida aparenta ser também maior em HSB e menor em LSB. Conclusão: A heterogeneidade da Doença de Sjögren é evidenciada pelos quatro clusters de doentes com diferentes sintomas e gravidade da doença. O cluster DDF mostrou maior disfunção das glândulas salivares, títulos mais elevados de anticorpos anti-La/SSB e maior necessidade de secretagogos. O cluster HSB apresentou o maior impacto na qualidade de vida, em contraste com o grupo LSB. O cluster PDF pode ser um grupo intermédio com um fenótipo mais "inflamatório".
Introduction: Sjögren's disease (SjD) is a chronic autoimmune rheumatic condition typically characterized by xerophthalmia, xerostomia, fatigue, and pain. As a systemic disease, it can affect virtually any organ system, resulting in a wide range of symptoms. In fact, this important phenotypical heterogeneity represents a major obstacle when approaching these patients. Herein, we aimed to study a SjD population based on symptom clusters, detailing and comparing their characteristics. Methods: We included patients from the Observational Lisbon Sjögren’s Syndrome Prospective Observational (OLISSIPO) cohort. We employed the Newcastle Sjögren's Stratification Tool (NSST) to categorize patients into four different symptom-based clusters: low-symptom burden (LSB), high symptom burden (HSB), pain dominant with fatigue (PDF) and dryness dominant with fatigue (DDF). We then compared patients in these clusters across clinical, laboratory, pathological, and imaging features. 7 Results: We allocated 319 patients (94.4% female, mean age 58.5±13.6 years ) into the four symptom-based clusters: LSB (n=39, 12.2%), HSB (n=95, 29.8%), PDF (n=155, 48.6%) and DDF (n=30, 9.4). Significant differences were observed across these groups revealing lower unstimulated salivary flow in the DDF (1.3 ml/15min) and HSB (1.8 ml/15min) groups, alongside with higher pilocarpine usage (37% in HSB and 33% in DDF). The DDF group had higher anti-La/SSB antibody titers (1084 IU/l), while the PDF group showed the highest CRP levels (0.39 mg/dl). Fibromyalgia, depression and decreased quality of life were more commonly observed in the HSB cluster and were less frequent in the LSB group. 8 Conclusion: Heterogeneity of SjD is evidenced by four clusters of patients with different symptoms and disease burden. The DDF cluster showed higher salivary gland dysfunction, anti-La/SSB titers and need for secretagogues, the HSB cluster had the worst disease impact, which contrasted with the LSB group. The PDF cluster may be an intermediate group with a more “inflammatory” endotype.
Introduction: Sjögren's disease (SjD) is a chronic autoimmune rheumatic condition typically characterized by xerophthalmia, xerostomia, fatigue, and pain. As a systemic disease, it can affect virtually any organ system, resulting in a wide range of symptoms. In fact, this important phenotypical heterogeneity represents a major obstacle when approaching these patients. Herein, we aimed to study a SjD population based on symptom clusters, detailing and comparing their characteristics. Methods: We included patients from the Observational Lisbon Sjögren’s Syndrome Prospective Observational (OLISSIPO) cohort. We employed the Newcastle Sjögren's Stratification Tool (NSST) to categorize patients into four different symptom-based clusters: low-symptom burden (LSB), high symptom burden (HSB), pain dominant with fatigue (PDF) and dryness dominant with fatigue (DDF). We then compared patients in these clusters across clinical, laboratory, pathological, and imaging features. 7 Results: We allocated 319 patients (94.4% female, mean age 58.5±13.6 years ) into the four symptom-based clusters: LSB (n=39, 12.2%), HSB (n=95, 29.8%), PDF (n=155, 48.6%) and DDF (n=30, 9.4). Significant differences were observed across these groups revealing lower unstimulated salivary flow in the DDF (1.3 ml/15min) and HSB (1.8 ml/15min) groups, alongside with higher pilocarpine usage (37% in HSB and 33% in DDF). The DDF group had higher anti-La/SSB antibody titers (1084 IU/l), while the PDF group showed the highest CRP levels (0.39 mg/dl). Fibromyalgia, depression and decreased quality of life were more commonly observed in the HSB cluster and were less frequent in the LSB group. 8 Conclusion: Heterogeneity of SjD is evidenced by four clusters of patients with different symptoms and disease burden. The DDF cluster showed higher salivary gland dysfunction, anti-La/SSB titers and need for secretagogues, the HSB cluster had the worst disease impact, which contrasted with the LSB group. The PDF cluster may be an intermediate group with a more “inflammatory” endotype.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Palavras-chave
Doença de Sjögren Estratificação com base em sintomas Classificação da doença Heterogeneidade de doentes Reumatologia