Abstract
ANAKINRA TREATMENT IN RECURRENT PERICARDITIS: SINGLE CENTER EXPERIENCE
Full text
Background: Recurrent pericarditis (RP), however the etiology is unknown in the majority, may be observed in autoinflammatory diseases such as familial Mediterranean fever (FMF) and tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS). Colchicine has long been used to treat pericarditis related to FMF as well as patients with idiopathic recurrent pericarditis (IRP) (1). Alternative treatments have been reported for cases with colchicine resistant RP.Objectives:
Objectives: The aim is to present our data regarding anakinra treatment in recurrent pericarditis either related to FMF or idiopathic, who are resistant to colchicine.
Methods: Patients who had recieved anakinra with a diagnosis of recurrent pericarditis either idiopathic or secondary to FMF followed in our autoinflammatory disease center between 2014-2018 are evaluated retrospectively. From patients’ files, demographic and clinical features, response to other treatment approaches such as NSAID, corticosteroid, colchicine, were evaluated. All patients have been genetically screened for monogenic autoinflammatory diseases (MEFV, TRAPS, MVK, NLRP3, NOD2). Patients who had at least 3 attacks were administered anakinra 100 mg/day. Therapeutic efficacy, as well as side effect profile of anakinra is also assessed.
Results: There were 5 patients (3 male and 2 female) with the diagnosis of RP, 1 was related to FMF and 4 were idiopathic. The mean age of the group was 28±8 (range 20-40). All patients diagnosed with IRP were negative for autoinflammatory genetic screening, while a MEFV variant (K695R het.) was detected in the FMF patient. Median duration of follow-up was 30 months (range 11-129). In
table 1
, demographic and clinical features are given. The median number of recurrence was 6 before anakinra treatment. No episode of pericarditis was observed in any of the patients after the initiation of anakinra. The response to anakinra persisted even after the dose was reduced to 100 mg/alternate day in 3 patients, however in 2, recurrence of pericarditis was observed and anakinra was escalated to initial dose. It was possible to discontinue corticosteroid treatment in all patients. Currently all patients continue anakinra treatment. No side effect including injection site reaction, has been observed by now.
Table 1
Demographic features and treatment response during anakinra therapy
Patient ID #
Age
Sex
Diagnosis
Duration of pericarditis follow-up (mo)
Prior medications
Number of recurrences before anakinra
Anakinra treatment duration (mo)
Time to corticosteroid discontinuation (mo)
Number of recurrences after daily dose of anakinra
1*
23
M
IRP
129
Colchicine, NSAIDs, CS, HCQ
6
52
9
No
2
32
F
IRP
128
Colchicine, NSAIDs
7
4
NA
No
3*
40
F
IRP
21
Colchicine, CS
6
8
1
No
4
20
M
IRP
11
Colchicine, CS
3
8
2
No
5
25
M
FMF
30
Colchicine, CS
5
15
1
No
F: Female, M: Male, CS: Corticosteroid, HCQ: Hydroxychloroquine, NA: Not applicable
*Dose tapering was unsuccessful in these patients
Conclusion: Anakinra seems to be a safe and effective treatment approach for colchicine resistant recurrent pericarditis. However recurrence may occur during dose tapering.
REFERENCES:
[1] Adler Y, et al. Colchicine treatment for recurrent pericarditis. A decade of experience. Circulation. 19982;97(21):2183-5.
Disclosure of Interests: None declared
DOI: 10.1136/annrheumdis-2019-eular.6562Citation: Ann Rheum Dis, volume 78, supplement 2, year 2019, page A1004Session: Other orphan diseases
(Scientific Abstracts)
1 organization