How the adult criss works in pediatric jssc patients - results from the juvenile scleroderma inception cohort

Introduction: The Composite Response Index in Systemic Sclerosis
(CRISS) was developed by Dinesh Khanna as a response measure in
patients with adult systemic sclerosis. CRISS aims to capture the
complexity of systemic sclerosis and to provide a sensitive measure for
change in disease activity. The CRISS score is based on a two-step approach. First, significant disease worsening or new-onset organ damage
is defined as non-responsiveness. In patients who did not fulfill the criteria of part one, a probability of improvement is calculated for each
patient based the Rodnan Skin Score (mRSS), percent predicted forced
vital capacity (FVC%), patient and physician global assessments (PGA),
and the Health Assessment Questionnaire Disability Index (HAQ-DI). A
probability of 0.6 or higher indicates improvement.
Objectives: to validate the CRISS in a prospectively followed cohort
of patients with juvenile systemic sclerosis (jSSc)
Methods: Data from the prospective international inception cohort
for jSSc was used to validate the CRISS. Patients with an available 12-
months follow-up were included in the analyses. Clinically improvement was defined by the anchor question about improvement
(much better or little better versus almost the same, little worse or
much worse) in patients overall health due to scleroderma since the
last visit provided by the treating physician.
Results: Forty seven jSSc patients were included in the analysis.
74.2% had diffuse subtype. The physician rated the disease as
improved in 34 patients (72.3%) since the last visit. No patient had a
renal crisis or new onset of left ventricular failure during the 12-
months follow-up. Three patients (3.4%) each had a new onset or
worsening of lung fibrosis and new onset of pulmonary arterial
hypertension. In total, 6 patients resulted in a rating of not improved
based on the CRISS in part I. The mRSSS, FVC%, CHAQ and PGA significantly improved during the 12-months follow-up in patients who
were rated as improved. The predicted probability based on the CRIS
S algorithm resulted in an area under curve of 0.77 predicting the anchor question of improvement. In summary, 33 (70.0%) patients were
correctly classified by the adult CRISS score resulting in an overall
area under curve of 0.7.
Conclusion: The CRISS score was evaluated in a pediatric jSSc cohort
for the first time. It showed a good performance. However, it seems
that the formula of part II of the CRISS score needs a calibration to
pediatric jSSc patients.