Abstract Background/Aims Idiopathic recurrent pericarditis (IRP) is a relapsing remitting autoinflammatory condition that causes significant morbidity. It is a rare subset of pericarditis, characterised by chest pain, debility, fever and systemic inflammation with raised inflammatory markers. Recent guidance recommends first-line treatment with non-steroidal anti-inflammatory drugs (NSAIDS) and colchicine. Glucocorticoids may be needed, but are associated with significant side-effects and increased relapse risk on discontinuation. A small proportion of people have IRP refractory to first-line therapy, and guidelines recommend IL-1 antagonists such as anakinra, currently off tariff in the UK. We present a case of recurrent, refractory pericarditis joint managed by cardiology and rheumatology in which anakinra was effective and steroid sparing. Methods An 18-year-old male, normally fit and well, presented emergently with sudden onset chest pain, dyspnoea and pyrexia. His CRP was 215mg/L, with ST elevation on multiple electrocardiographic leads. The echocardiogram showed global pericardial effusion. He was treated with ibuprofen 400mg tds, colchicine 500mg bd and discharged. He returned 5 days later, with ongoing chest pain, pyrexia and a CRP of 268 mg/L. Adhesions and septations within the pericardial space were found on repeat echocardiography. VATS pericardial biopsy and drainage demonstrated negative cultures and histology in keeping with inflammation. Rheumatology screen was negative. He was discharged on a weaning course of prednisolone 40mg daily. Within a few days he re-presented with flushing, insomnia, sweating, increased appetite and mood changes that settled with a rapid wean of steroids. A third admission 13 weeks from initial presentation was characterised by chest pain and a CRP of 106mg/L. A successful off tariff funding request for a trial of anakinra 100mg daily resulted in no relapses or steroid requirement. Results IRP is diagnosed where other causes of pericarditis are excluded and recurring pericarditis is confirmed on ECG and cardiac imaging associated with systemic inflammatory response (almost always fever and raised CRP). Anakinra is effective in the treatment of IRP, recommended by international guidelines but unfunded in the UK and unfamiliar to cardiologists. Diagnosis of IRP requires a high index of suspicion and management requires collaboration between cardiology and rheumatology. In our case the correct first-line medications (NSAIDS and colchicine) were started, but at suboptimal doses and glucocorticoids were commenced at a higher dose than needed causing intolerable side effects. Rheumatologists have expertise in rational use of these medicines and can exclude autoimmune causes of pericarditis. 6 months of anakinra costs £6720, significantly less than the cost of recurrent admissions. Conclusion We demonstrate the need for co-operation between cardiology and rheumatology in the management of pericarditis, especially in identifying the rare subset with IRP. Coordination between cardiology and rheumatology in this inflammatory affair improves early treatment, diagnosis and outcomes. Disclosure J.M.L. Belcher: None. A. Al-Mohammad: None. O. Watson: None. S. Khalid: None. R.S. Tattersall: None.
Belcher et al. (Wed,) studied this question.