A multi-centre, observational, prospective study was designed to evaluate the clinical usefulness of the systemic score in predicting life-threatening evolution – defined as the development of macrophage activation syndrome (MAS) and/or mortality. The intention was also to derive a more aggressive clinical patient subset. To achieve this, Ruscitti and colleagues collected data from 597 patients taking part in the GIRRCS (Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale) AOSD-study group and in the AIDA (AutoInflammatory Disease Alliance) Still's disease registry –. First developed in the 1990s,3 the prognostic value of the systemic score has previously been tested in a small number of cases.4 The new work was presented in a session on the management of Still's disease across the life-span at the 2024 congress of EULAR – The European Alliance of Rheumatology Associations.
Each patient was assessed for the systemic score, which assigns 1 point to each of 12 manifestations: fever, typical rash, pleuritis, pneumonia, pericarditis, hepatomegaly or abnormal liver function tests, splenomegaly, lymphadenopathy, leucocytosis > 15,000/mm3, sore throat, myalgia, and abdominal pain.3,4
When looking at manifestations, 100% of patients included had fever, 87.9% had joint involvement, and 66.1% had skin rash. Liver involvement was recognised in 43.5%, MAS in 13.1%, and lung disease in 6.9%. There was a mortality rate of 3.4% attributed to Still's disease.
A result of at least 7 on the systemic score proved to be a significant predictor of life-threatening evolution. To further explore this, a risk-profile assessment was performed on the clinical variables used to calculate the systemic score, and an age- and sex-adjusted multivariate logistic regression model built. This showed that liver involvement and lung disease independently predicted life-threatening evolution. The clinical characteristics of this subset of patients was then derived, revealing that those with liver involvement were significantly characterised by lymph-adenomegaly, splenomegaly, pericarditis, and pleuritis – and were also burdened by lung disease.
In the patients with lung disease, significant characteristics were sore throat, lymph-adenomegaly, splenomegaly, and liver involvement. Furthermore, those with lung disease showed a higher frequency of pericarditis, pleuritis, and abdominal pain. This subset was also burdened with a higher mortality rate.
The results suggest that the systemic score could be used as a prognostic tool in clinical practice. Additionally, liver involvement and lung disease are clinically relevant findings, and should be highlighted as key multi-organ manifestations of Still's disease, and as major predictors of life-threatening evolution.
Source
Ruscitti P, et al. The evaluation of systemic score in identifying patients with Still's disease at higher risk of life-threatening evolution; findings from GIRRCS AOSD-study group and AIDA Network Still's Disease Registry. Presented at EULAR 2024; OP0001.
Ann Rheum Dis 2024; DOI: 10.1136/annrheumdis-2024-eular.667.
References
1. Lee JJY, et al. Systemic Juvenile Idiopathic Arthritis. Pediatr Clin North Am 2018;65:691–709.
2. Giacomelli R, et al. A comprehensive review on adult onset Still's disease. J Autoimmun 2018;93:24–36.
3. Pouchot J, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991;70:118–36.
4. Ruscitti P, et al. Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Med 2016;14:194.
About EULAR
EULAR is the European umbrella organisation representing scientific societies, health professional associations and organisations for people with rheumatic and musculoskeletal diseases (RMDs). EULAR aims to reduce the impact of RMDs on individuals and society, as well as improve RMD treatments, prevention, and rehabilitation. To this end, EULAR fosters excellence in rheumatology education and research, promotes the translation of research advances into daily care, and advocates for the recognition of the needs of those living with RMDs by EU institutions.
