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Disease Activity Scoring in Adult-onset Still's disease

Jack Cush, MD

Still’s disease in adults (AOSD) or children (sJIA) can have dramatic symptom severity, making it easy to gauge disease activity and response to therapy, especially at the outset. However, a validated measure of disease activity has not been agreed (for clinical trial and treatment assessments). A new study compares two such activity measuresin a large cohort of Still’s patients.

Researchers scored the disease severity of 174 AOSD patients, classifying them as either high or low disease severity states using the modified Systemic Manifestation Score (mSMS) and the mPouchot scores.

They found that both mSMS and mPouchot score  positively correlated with ESR (both P < 0.001), CRP (both P < 0.0001), and serum ferritin (both P < 0.0001). Moreover, both mSMS and mPouchot score are significantly associated with liver dysfunction and high IL-18 (both P < 0.0001) and IL-6 (both P < 0.01) levels in AOSD patients.

Using the area under curve (AUC) value, the mSMS was significantly less than of mPouchot score (0.71 for mSMS, 0.81 for mPouchot score, P < 0.0001), suggesting a slightly higher sensitivity for the sSMS (compared with mPouchot) (75.64% vs 74.36%) but a lower specificity (55.06% vs 76.40%). High disease severity of AOSD was better identfied by the mPouchot score.

These measures are compared the the Systemic Disease Inflammatory Index (SDII and the Systemic Feature Score (SFS) on the Still’s Disease Activity Calculator page of

These scores can accurate depict disease severity of AOSD (and sJIA) and may prove useful in assessing outcomes in clinical trials.  Moreover it may be helpful to have such a score to identify those with high disease severity and to assess such outcomes or approaches for those with high disease activity.


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