Recurrent Pericarditis: A Challenge in Autoinflammatory Disease Clinic and the Role of Anakinra

Blas Betancourt¹, Amanda Ombrello², Ananta Subedi³, Patrycja M. Hoffmann² and Daniel L. Kastner⁴, ¹National Institute of Arthritis, Musculoskeletal and Skin Disease (NIAMS), Bethesda, MD, ²NHGRI, National Institutes of Health, Bethesda, MD, ³National Institutes of Health, NIAMS, Bethesda, MD, ⁴Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Autoinflammatory Disease and anakinra

Session Information

Date: Monday, October 22, 2018

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster II: Interstitial Lung Disease, Still's Disease, FMF, Polychondritis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Recurrent pericarditis is a common complication following an acute episode of pericarditis affecting about 15-30% of the cases. The etiology of this condition is unknown in most of the patients but it is thought that an autoinflammatory process has an important role in the pathogenesis. Anakinra is an interleukin-1 receptor antagonist that reduces systemic inflammatory responses. The aim of our study was to describe the clinical characteristics and response to anakinra in patients with recurrent pericarditis.

Methods: We retrospectively reviewed patients referred to our clinic with the diagnosis of recurrent pericarditis who were resistant to conventional therapy.

Results: A total of 11 patients were included. The majority were females (7 [64%]) with a median age at presentation of 20 years. Nine patients were diagnosed with recurrent idiopathic pericarditis, one case was found to have familial Mediterranean fever and another patient had a history of systemic lupus erythematosus. Autoimmune workup was negative in all of the cases except in the patient with a history of lupus. Standard doses of nonsteroidal anti-inflammatory drugs, colchicine, and steroids were used in all of the cases. Good response to steroids was noted in all patients (except in the case of lupus) but it was not possible to taper glucocorticoids completely because of recurrence of the pericarditis. Other immunosuppressive medications used were methotrexate, hydroxychloroquine, infliximab, azathioprine, and mycophenolate mofetil. Anakinra, doses between 100-300 mg daily, was associated with clinical response in most of the patients (complete response in 5 patients, partial response in 4 patients, and no response in 1 patient). One patient did not have a follow-up evaluation after anakinra treatment. Anakinra was well tolerated and the main adverse side effect was local reactions.

Conclusion: Anakinra is a safe and effective drug for the management of treatment-resistant recurrent pericarditis.

Disclosure: B. Betancourt, None; A. Ombrello, None; A. Subedi, None; P. M. Hoffmann, None; D. L. Kastner, None.

To cite this abstract in AMA style:

Betancourt B, Ombrello A, Subedi A, Hoffmann PM, Kastner DL. Recurrent Pericarditis: A Challenge in Autoinflammatory Disease Clinic and the Role of Anakinra [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/recurrent-pericarditis-a-challenge-in-autoinflammatory-disease-clinic-and-the-role-of-anakinra/. Accessed .

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