Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry

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Tipo de produção
article
Data de publicação
2023
DOI
Scopus
Web of Science
PubMed
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Título da Revista
ISSN da Revista
Título do Volume
Editora
SPRINGER-VERLAG ITALIA SRL
Autores
TRIGGIANESE, Paola
VITALE, Antonio
LOPALCO, Giuseppe
CICCIA, Francesco
AL-MAGHLOUTH, Ibrahim
RUSCITTI, Piero
SFIKAKIS, Petros Paul
IANNONE, Florenzo
Citação
INTERNAL AND EMERGENCY MEDICINE, v.18, n.8, p.2231-2243, 2023
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data.
Palavras-chave
Arthritis, Autoinflammatory diseases, Diagnosis, MAS, Prognosis
URI
https://observatorio.fm.usp.br/handle/OPI/59198
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Coleções
Artigos e Materiais de Revistas Científicas - HC/ICHC