Therapeutic approach in Colchicine-resistant Recurrent pEricarditis in children: an open-label randomized trial comparing Anakinra vs sTEroids

INTERVENTION: Product Name: Kineret 100 mg/0.67 ml solution for injection in pre‐filled syringe.,Product Code: PRD6844805,Pharmaceutical Form: SOLUTION FOR INJECTION,Other descriptive name: ,Strength: Anakinra 150mg,Product Name: PREDNISONE,Product Code: SUB10020MIG,Pharmaceutical Form: TABLETS,Other descriptive name: ,Strength: 5mg,Product Name: PREDNISONE,Product Code: SUB10020MIG,Pharmaceutical Form: TABLETS,Other descriptive name: ,Strength: 25mg CONDITION: MedDRA version: 20.0Level: PTClassification code: 10034484Term: PericarditisSystem Organ Class: 100000004849 Recurrent pericarditis, idiopathic or secondary to invasive cardiac procedures, not responsive to colchicine and NSAIDs after the first relapse. ; MedDRA version: 20.0Level: PTClassification code: 10034484Term: PericarditisSystem Organ Class: 100000004849 Therapeutic area: Diseases [C] ‐ Cardiovascular Diseases [C14]‐Diseases [C] ‐ Immune System Diseases [C20] PRIMARY OUTCOME: Main Objective:Main objective of this study is to demonstrate that anakinra is more effective than steroids to control the disease and prevent further flares in paediatric (from 8 months to < 18 years) patients with RP not responding to first line treatment with NSAIDS and colchicine at the proper dosage, or in case of colchicine intolerance. Primary end point(s):Complete response of disease flare at day 7,Lack of relapse at month 3 Secondary Objective:In the study we aim to collect the RNA to perform trascriptomic study in the patients enrolled in the study at different timepoints (at baseline, at day 14, 28 and, in responder patient, at the time of the relapse or at last visit of the study). Through a correlation between the clinical, laboratory parameters and the response to treatment with the trascriptome analysis, we aim to identify transcripts associated to a higher probability of response to treatment and long‐term remission off‐therapy. SECONDARY OUTCOME: Secondary end point(s):Behavior of acute phase reactants during 6 months of observation Secondary end point(s):Number of day of hospitalization Secondary end point(s):Number of flares at follow up Secondary end point(s):Quality of life Secondary end point(s):Safety (registration of side effects occurred during the whole period of the study) Secondary end point(s):Time to achieve a complete control of the flare Secondary end point(s):Time to flare after treatment tapering Secondary end point(s):Transcriptomic study INCLUSION CRITERIA: Male and female patients, from 8 months to less than 18 years,Parent or legal guardian written informed consent and child assent, if appropriate, are required before any assessment is performed,Diagnosis of relapse of pericarditis in a patient with previous diagnosis of acute pericarditis (idiopathic or secondary to invasive cardiac procedures),Inadequate response or intolerance to non‐steroidal anti‐inflammatory drugs or colchicine