Artikel
Clinical features and treatment of idiopathic recurrent acute pleuro-pericarditis
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Veröffentlicht: | 5. Februar 2019 |
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Gliederung
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Background: Idiopathic recurrent acute pleuro-pericarditis (IRAP) is an increasingly recognized autoinflammatory disease comprising post-pericardiotomy-syndrome, recurrent pericarditis and post-myocardial-infarction-syndrome. Different autoimmune mechanisms were discussed in the past. Recently, IRAP is considered as an autoinflammatory disease. Therapeutic options comprise colchicine, prednisolone and interleukin (IL)-1beta blocking agents. The aim is to investigate whether idiopathic and post-interventional pleuro-pericarditis represent a clinical spectrum and to identify treatment options.
Methods: This study analyses demographic, clinical and laboratory features of post-interventional and idiopathic pleuro-pericarditis and adult onset Still’s disease (AOSD) as a reference. Patients with infectious disease, connective tissue disease, chronic heart failure, renal failure and other non-exsudative effusions were excluded from this analysis. Patients with IRAP were treated with colchicine, prednisolone and IL1β blocking agents.
Results: Between 2005 and 2017, 66 cases of idiopathic and post-interventional pleuro-pericarditis were identified and compared to 83 cases of AOSD. Clinical and laboratory features suggest that idiopathic and post-interventional pleuro-pericarditis represent a clinical spectrum which is identical with IRAP. Prednisolone was started with 25mg to 125mg and tapered to less than 7mg or discontinued if not effective. 47 of 66 patients (71%) were treated with prednisolone and 10/47 (21%) were in remission with no need of any further therapeutically escalation. Colchicine was given to 44/66 patients (67%) and 29/44 (66%) were in complete remission. Four of 66 patients (6%) did not respond or had contraindications against colchicine or prednisolone and were treated with anakinra. Of these patients, 4/4 (100%) were in remission. During the follow-up period of 20 patient*months 2 of 4 patients maintained the remission with anakinra every 2nd day and two patients discontinued anakinra and remained in remission.
Conclusion:
1. Post-pericardectomy-syndrome, post-myocardial-infarction-syndrome and idiopa-thic recurrent pericarditis represent a clinical spectrum of autoinflammatory diseases.
2. Treatment options comprise colchicine as a first-line therapy, prednisolone and anti-IL1 blocking agents.