Session Type: Abstract Submissions
Session Time: 5:30PM-7:00PM
Background/Purpose: PFAPA is the most common recurrent fever condition in children. A recent survey showed heterogeneity in physicians’ management strategies. In order to evaluate the effectiveness of different treatments for PFAPA, standardized treatment plans are needed. The PFAPA Work Group of CARRA (Childhood Arthritis and Rheumatology Research Alliance) developed CTPs for PFAPA that will lead to improved characterization of a well-defined cohort and optimal treatment. We aimed to develop consensus treatment plans (CTPs) for periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome for future evaluation of treatment responses in PFAPA.
Methods: A core group of PFAPA experts including pediatric rheumatologists, infectious disease specialists and immunologists comprised the CARRA PFAPA Workgroup. After a literature review was conducted, a survey was distributed to pediatric rheumatology and infectious diseases physicians who treat PFAPA patients to query their management strategies. The CARRA PFAPA workgroup met monthly via teleconferences and in-person meetings from 2014-2016 to develop the CTPs. A modified nominal group technique was employed to create the proposed CTPs. Patient characteristics for enrollment were established through expert consensus.
Results: Based on these data, 4 preliminary CTP arms were created: anti-pyretic treatment, corticosteroids, prophylaxis arm and surgical management (Figure 1). This approach was approved by the majority of the PFAPA subcommittee.
Conclusion: CTPs with four treatment arms were developed for PFAPA. Coupled with data collection on patient outcomes at defined intervals, these CTPs will generate comparative effectiveness data in an observational setting to determine optimal treatment of PFAPA after the larger CARRA group is assessed for their willingness to follow at least one of the regimens in these CTPs.
To cite this abstract in AMA style:Amarilyo G, Rothman D, Manthiram K, Li S, Harel L, Edwards K, Marshall G, Nativ S, Haines K, Lionetti G, Cherian J, Zhao Y, DeLaMora P, Syverson G, Michelow I, Stepanovskiy Y, Thatayatikom A, Yildirim-Toruner C, Akoghlanian S, Tucker L, Koranyi K, Srinivasalu H, Dedeoglu F, Lapidus S. Preliminary Consensus Treatment Plans for Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis: The Foundation for Capturing Treatment Responses in PFAPA from the CARRA PFAPA Subcommittee [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 4). https://acrabstracts.org/abstract/preliminary-consensus-treatment-plans-for-periodic-fever-aphthous-stomatitis-pharyngitis-and-adenitis-the-foundation-for-capturing-treatment-responses-in-pfapa-from-the-carra-pfapa-subcommittee/. Accessed November 13, 2019.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/preliminary-consensus-treatment-plans-for-periodic-fever-aphthous-stomatitis-pharyngitis-and-adenitis-the-foundation-for-capturing-treatment-responses-in-pfapa-from-the-carra-pfapa-subcommittee/