Email this article An autoinflammatory disease – PFAPA syndrome: a single-center experience
- Authors: Kozlova A.L.1, Leontieva M.E.1, Velieva P.T.1, Kalinina M.P.1, Deordieva E.A.1, Shvets O.A.1, Kuzmenko N.B.1, Mukhina A.A.1, Grachev N.S.1, Novichkova G.A.1, Shcherbina A.Y.1
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Affiliations:
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
- Issue: Vol 21, No 3 (2022)
- Pages: 56-64
- Section: ORIGINAL ARTICLES
- Submitted: 26.07.2022
- Accepted: 29.08.2022
- Published: 29.08.2022
- URL: https://hemoncim.com/jour/article/view/638
- DOI: https://doi.org/10.24287/1726-1708-2022-21-3-56-64
- ID: 638
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Abstract
The aim of this study was to analyze the clinical and laboratory data of 101 patients (61 boys, 40 girls) diagnosed with PFAPA syndrome. The age of onset of PFAPA syndrome ranged from 8 to 36 months (the median age of onset was 18 months). The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. In most cases, clinical manifestations included recurrent fevers, acute tonsillitis (in 84% of cases, the tonsils were covered with a whitish coating), and cervical lymphadenitis. More rarely, the patients had diarrhea, vomiting, myalgia, arthralgia, and rash. All of the patients met the criteria proposed by G.S. Marshall. Our management strategies for patients with PFAPA syndrome include symptomatic treatment with antipyretics, short-term glucocorticosteroid therapy, and tonsillectomy. The majority of patients have complete resolution of PFAPA symptoms by the age of 7 regardless of the type of therapy.
About the authors
A. L. Kozlova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
Author for correspondence.
Email: annamax-99@mail.ru
ORCID iD: 0000-0002-2869-6535
Anna L. Kozlova, Cand. Med. Sci., a senior researcher at the Department of Treatment Optimization for Immunodeficiencies
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationM. E. Leontieva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-0799-1025
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationP. T. Velieva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0001-9275-4261
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationM. P. Kalinina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-6839-3918
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationE. A. Deordieva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-8208-2075
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationO. A. Shvets
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0001-5347-7150
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationN. B. Kuzmenko
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-1669-8621
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationA. A. Mukhina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-3305-1694
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationN. S. Grachev
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-4451-3233
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationG. A. Novichkova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-2322-5734
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationA. Yu. Shcherbina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation
ORCID iD: 0000-0002-3113-4939
1 Samory Mashela St., Moscow 117997, Russia
Russian FederationReferences
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