Skip to main content

Systemic Score Predicts Still's Disease Severe Complications

A multinational study of adults with Still's disease  found that calculating their systemic score at baseline significantly predicted the patients risk for life-threatening outcomes (MAS or death).

Two cohorts of adult-onset Still's disease (AOSD) included the GIRRCS (Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale) AOSD-study group and AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry patients. The life-threatening outcomes were defined as mortality, and/or macrophage activation syndrome (MAS), a secondary hemophagocytic lymphohistiocytosis.

There were a total of 597 Still's disease patients , with a mean age of 36.6±17.3 years (male 44%).  The Systemic Score (of Pouchot et al) was defined based on the presence of 12 manifestations (one point each): fever,  typical rash, pleuritis, pneumonia, pericarditis,  hepatomegaly or abnormal liver function tests,  splenomegaly, lymphadenopathy, leukocytosis > 15000/mm3, sore throat, myalgia, and abdominal pain (maximum score: 12 points).

The systemic score significantly predicted:

  • life-threatening outcomes (OR: 1.24, 95%CI:1.07–1.42; p=0.004). 
  • systemic score ≥7 was more predictive of life-threatening evolution (OR: 3.36, 95%CI:1.81–6.25; p<0.001). 

Use of this tool demonstrated the importance of liver involvement (OR: 1.68, 95%CI:1.48–2.67; p=0.031) and lung disease (OR: 2.12, 95%CI:1.14–4.49; p=0.042) on serious outcomes.

The clinical utility of the systemic score was demonstrated in evaluating of Still's disease patients are at higher risk of life-threatening evolution.

stillsNow cTA


If you are a health practitioner, you may to comment.

Due to the nature of these comment forums, only health practitioners are allowed to comment at this time.

The author has no conflicts of interest to disclose related to this subject