THU0530 IDIOPATHIC RECURRENT PERICARDITIS: CLINICAL FINDINGS AND TREATMENT APPROACH
Authors: Camilla Celani, Silvia Federici, Anna Tulone, Brigitte Bader-Meunier, Virginia Messia, Manuela Pardeo, Claudia Bracaglia, Pierre Quartier, Fabrizio De Benedetti, Antonella Insalaco
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Recurrent pericarditis affects 15-30% of patients with acute pericarditis. The etiology is poorly understood, with about 80% being idiopathic. Several treatment options are available for recurrences, including NSAIDs, colchicine, glucocorticoids and IL-1 inhibitors (i.e. Anakinra). Standardized guidelines for the management of these patients are still lacking <h3>Objectives:</h3> To analyze clinical findings and treatment approach in a cohort of pediatric patients with recurrent pericarditis <h3>Methods:</h3> Patients with at least two episodes of idiopathic pericarditis, followed at two Pediatric Rheumatology centers between 2006 and 2018, were included <h3>Results:</h3> A total of 42 patients (18 males) were included. Mean age at disease onset was 11.8 years (range 4-17). Chest pain and fever were the presenting symptoms in all patients. In 47% pleural effusion was detected. Laboratory tests showed increased white blood cell count (mean 14.509/mm3), C-reactive protein (mean 18.01 mg/dl) and erythrocyte sedimentation rate (mean 39 mm/h) in all patients. The first episode was variably treated: 18/42 (43%) received NSAIDs alone, 5/42 (11.9%), colchicine alone or associated to NSAIDs and 3/42 patients (7%) received antibiotics alone. 16/42 (38%), not responsive to NSAIDs or colchicine, received glucocorticoides. Patients who received glucocorticoids at the first episode relapsed earlier (median time of 2.1 months range 10 days-5 months), than patients treated with NSAIDs (6.6 months range 10 days -24 months) or with colchicine (5 months range 10 days-5 months) (p&lt;0.05). In our study, initial treatment of the first episode did not affect the number of subsequent flares. To evaluate treatment strategy at relapses, we divided our study population in two groups: Group 1 (20 pts) in which recurrence was treated with NSAIDs, colchicine or glucocorticoid (alone or combined); group 2 (22 patients) in which anakinra was started. Among patients belonging group 2, 9 received anakinra at first relapse, 7 at the second, 2 at the third and 2 at the fourth. Anakinra treatment was followed by a prompt resolution of symptoms and inflammatory signs within 2 days. During daily treatment with full dose anakinra, no relapses were reported over a median of 13.3 months (range 5-24 months). In 13 out of 22 patients, anakinra was gradually tapered reducing the days of administration during the week. Four of these patients relapsed. The mean time from the start of anakinra to tapering was 17±4 months (range 14-23 months) in the 4 patients who experienced a relapse versus 14±4 months (range 7-21 months) in patients who did not flare, with no statistical difference. Among the 22 patients belonging to group 2 anakinra was finally discontinued in 11 after a mean time of 23.4 months (range 12-36). Among these, 8 relapsed after anakinra withdrawal including 2 of the 4 patients already relapsed during tapering). Only 3 patients didn't present any relapse (up to 20.3 months of follow-up). All patients who relapsed responded quickly to the reintroduction of anakinra <h3>Conclusion:</h3> Our study confirms the lack of a standardized treatment approach in patients with recurrent pericarditis. Patients treated with glucocorticoid at first episode relapse before than those treated with other drugs. Anakinra is an effective treatment; however, tapering/discontinuation of the drug lead to relapses in several cases. Further experience on larger population is needed to define the best treatment duration and approach to withdrawal of IL-1 inhibitor <h3>Disclosure of Interests:</h3> Camilla Celani: None declared, Silvia Federici: None declared, Anna Tulone: None declared, Brigitte Bader-Meunier: None declared, Virginia Messia: None declared, Manuela Pardeo: None declared, Claudia Bracaglia: None declared, Pierre Quartier Consultant for: AbbVie, Chugai-Roche, lilly, Novartis, Novimmune, Sanofi, and SOBI, Consultant for: AbbVie, Chugai-Roche, Lilly, Novartis, Novimmune, Sanofi, and SOBI, Speakers bureau: AbbVie, BMS, Chugai-Roche, Novartis, Pfizer, and SOBI, Speakers bureau: AbbVie, BMS, Chugai-Roche, Novartis, Pfizer, and SOBI, Fabrizio De Benedetti Grant/research support from: Abbvie, SOBI, Novimmune, Roche, Novartis, Sanofi, Pfizer, Antonella Insalaco: None declared