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Therapy of autoinflammatory diseases: a review of the literature


The evidence for the therapy of autoinflammatory diseases is limited. There are few randomized controlled trials (RCTs) or cohort studies.


To provide an up to date literature review about the response to treatment in autoinflammatory diseases.


A literature search using Medline and Embase was performed regarding the treatment of Blau’s syndrome, Behçet’s disease, CAPS, CRMO, DIRA, FMF, MKD, NLRP12-mediated periodic fever, PAPA, PFAPA and TRAPS.


RCTs provide evidence for rilonacept and canakinumab in CAPS, colchicine in FMF and (adeno)tonsillectomy in PFAPA syndrome. For Behçet disease, RCTs have been conducted on the effect of several drugs, including colchicine, azathioprine, cyclosporine A, interferon alfa, etanercept, sucralfate suspension and pimecrolimus. Descriptive studies suggest that NSAIDs and corticosteroids are highly effective in respectively CRMO and PFAPA and moderately effective in the other diseases. Etanercept and anakinra appear to induce a complete or partial response in most patients with MKD, TRAPS, PAPA and colchicine-resistant FMF. Anakinra appears to induce a complete response in the majority of the CAPS and DIRA patients, but seems to be less effective in NLRP12-mediated periodic fever. Complete remission by infliximab is described in cases with Blau’s syndrome, CRMO, PAPA, refractory FMF and Behçet disease, but the effect of infliximab in TRAPS patients seems to be disappointing.


Reported findings were compared to the results of the Eurofever registry. These combined results could serve as a base for therapeutic guidelines and identify candidate drugs for future therapeutic trials.

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Correspondence to NM ter Haar.

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This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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ter Haar, N., Frenkel, J. Therapy of autoinflammatory diseases: a review of the literature. Pediatr Rheumatol 9 (Suppl 1), P15 (2011).

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