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

Gil Amarilyo¹, Deborah Rothman², Kalpana Manthiram³, Suzanne Li⁴, Liora Harel⁵, Kathryn Edwards⁶, Gary Marshall⁷, Simona Nativ⁸, Kathleen Haines⁹, Geraldina Lionetti¹⁰, Julie Cherian¹¹, Yongdong Zhao¹², Patricia DeLaMora¹³, Grant Syverson¹⁴, Ian Michelow¹⁵, Yuriy Stepanovskiy¹⁶, Akaluck Thatayatikom¹⁷, Cagri Yildirim-Toruner¹⁸, Shoghik Akoghlanian¹⁹, Lori Tucker²⁰, Katalin Koranyi¹⁹, Hemaltha Srinivasalu²¹, Fatma Dedeoglu²² and Sivia Lapidus⁸, ¹Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Petach Tikva, Israel, ²Albany Medical Center, Albany, NY, ³National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁴Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack Meridian Health, Hackensack, NJ, ⁵Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, ⁶Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN, ⁷Norton Children's Hospital, University of Louisville, Louisville, KY, ⁸Pediatric Rheumatology, Goryeb Children's Hospital, Morristown, NJ, ⁹Joseph M Sanzari Children’s Hospital, Hackensack Meridian Health, Hackensack, NJ, ¹⁰Pediatric Rheumatology, UCSF Benioff Children's Hospital Oakland, Oakland, CA, ¹¹Department of Pediatrics, Stony Brook University Hospital, Stony Brook, NY, ¹²Seattle Children's Hospital, Department of Pediatric Rheumatology, University of Washington, Seattle, WA, ¹³Weill Cornell Medical College, New York, NY, ¹⁴Pediatric Rheumatology, Medical College of Wisconsin, Wauwatosa, WI, ¹⁵Center for International Health Research, Alpert Medical School of Brown University, Providence, RI, ¹⁶Shupyk National Academy of Postgraduate Education, Kyiv, Ukraine, ¹⁷UF Health Shands Hospital, Gainesville, FL, ¹⁸Rheumatology, Nationwide Children's Hospital, Columbus, OH, ¹⁹Nationwide Children's Hospital, Columbus, OH, ²⁰Pediatric Rheum/Rm K4-120, BC Childrens Hospital, Vancouver, BC, Canada, ²¹Children's National Medical Center, Washington, DC, ²²Division of Immunology, Rheumatology Program, Boston Children's Hospital, Harvard Medical School, Boston, MA

Meeting: 2017 Pediatric Rheumatology Symposium

Keywords: Fever

Session Information

Date: Thursday, May 18, 2017

Title: Clinical and Therapeutic Poster Session

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.

Antipyretic arm,Cimetidine 10-20mg/kg/dose twice a day ,Prophylaxis arm,Colchicine 0.5-1.25mg/day ,Fever interval ≤ 14 days,Incomplete response*,* Incomplete response defined as recurrence of fever within 12-24 hours after treatment with prednisone Recommended time trial for medication efficacy is 3 months or 3 febrile episodes before changing to another arm.

Figure 1

Disclosure: G. Amarilyo, 2,5; D. Rothman, None; K. Manthiram, None; S. Li, None; L. Harel, 2,5; K. Edwards, None; G. Marshall, None; S. Nativ, 5; K. Haines, None; G. Lionetti, None; J. Cherian, None; Y. Zhao, 2; P. DeLaMora, None; G. Syverson, None; I. Michelow, None; Y. Stepanovskiy, None; A. Thatayatikom, None; C. Yildirim-Toruner, None; S. Akoghlanian, None; L. Tucker, None; K. Koranyi, None; H. Srinivasalu, None; F. Dedeoglu, None; S. Lapidus, 5.

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 .

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