Chronic Infantile Neurological Cutaneous and Articular (CINCA) syndrome: a review

Table 2 Results of the main studies analyzing the use of antiIL1 drugs in CINCA syndrome

Study	Type of study	Type of drug	Number of patients enrolled	Study duration (months)	Number of patients who reached primary endpoint	Number of patient with central nervous system remission^a
Goldbach-Mansky et al. 2006 [7]	Open-label	anakinra	18 CINCA	6	18/18	In 12 patients intracranial pressure, protein levels and white cells count decreased significantly
Lepore et al. 2010 [12]	Open-label	anakinra	14 CAPS: -10 CINCA - 4 MWS	36	14/14	Not evaluated
Sibley et al. 2012 [5]	Open-label	anakinra	26 CINCA	60	26/26	Significant decrease of CSF leukocyte count at 36 and 60 months (p = 0.0026 and 0.0076)
Hoffman et al. 2012 [58]	Open-label	rilonacept	101 CAPS (FCAS or MWS)	18	Mean key symptom score at week 72 reduced from 2.6 to 0	Not evaluated
Caorsi et al. 2013 [53]	Open-label	canakinumab	13 CAPS: - 7 CINCA - 4 MWS - 2 overlap CINCA/MWS	12	Complete response^b: Baseline: 5/13 Last Follow-up: 8/13	Not evaluated
Sibley et al. 2015 [55]	Open-label	canakinumab	6 CINCA	24	Full remission^c at month 6: 0/6 Inflammatory remission^d at month 6: 4/6	6 months: 0/6

^aCNS involvement: abnormal CSF leukocyte count; ^bcomplete response: absent or minimal disease activity at the global assessment with acute phase reactants within the normal range; ^cFull remission: remission of patient-reported clinical components and measures of systemic inflammation and CNS inflammation; ^dInflammatory remission: CRP ≤10 mg/L and global diary score remission

ISSN: 1750-1172