Idiopathic recurrent pericarditis as an immune-mediated disease: current insights into pathogenesis and emerging treatment options

Abstract

Idiopathic recurrent pericarditis affects 30–50% of patients with a previous attack of pericarditis. The etiopathogenesis is incompletely understood and most cases remain idiopathic with a presumed immune-mediated pathogenesis. The mainstay of therapy is aspirin or a nonsteroidal anti-inflammatory drug plus colchicine and the possible adjunct of a low-to-moderate dose of a corticosteroid in more difficult cases. Colchicine as an adjunct to anti-inflammatory therapy reduces by 50% the subsequent recurrent rate. For true refractory cases with failure of standard combination therapies, new and emerging options especially include human intravenous immunoglobulins and biological agents (i.e., anakinra). The outcome of idiopathic recurrent pericarditis is good with a negligible risk of developing constrictive pericarditis. Thus, it is important to reassure patients on their prognosis, explaining the nature of the disease and the likely course. Moreover, therapeutic choices should include less toxic agents and favor cheaper drugs whenever possible.

Keywords::

• pathogenesis
• recurrent pericarditis
• therapy

Financial & competing interests disclosure

Institutional funding was received by Acarpia (Madeira, Portugal) and FARGIM (Catania, Italy) for completion of clinical trials on colchicine for the treatment and prevention of pericarditis. All these studies were investigator initiated and spontaneous studies within the Italian Healthcare System. The sponsors had no role in the planning of the study as well as in managing data or writing of the papers including the present invited review. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Idiopathic recurrent pericarditis affects 30–50% of patients with a previous attack of pericarditis.

• The etiopathogenesis is incompletely understood and most cases remain idiopathic with a presumed immune-mediated pathogenesis.

• The mainstay of therapy is aspirin or an NSAID plus colchicine and the possible adjunct of a low to moderate dose of a corticosteroid to achieve a better control of symptoms.

• Evaluation of C-reactive protein allows to confirm the diagnosis and to monitor the activity of the disease.

• High doses of corticosteroids are associated with more severe side effects and recurrences.

• Colchicine as an adjunct to anti-inflammatory therapy reduces by 50% the subsequent recurrent rate.

• For true refractory cases with failure of standard combination therapies, new and emerging options include human intravenous immunoglobulins and biological agents (i.e., anakinra).

• The outcome of idiopathic recurrent pericarditis is good with a negligible risk of developing constrictive pericarditis during follow-up.

• It is important to reassure patients on their prognosis, explaining the nature of the disease and the likely course.

• Therapeutic choices should take into account the overall good outcome of the disease, including less toxic agents.