Protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever in children: case-based r
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Rheumatology International https://doi.org/10.1007/s00296-020-04696-0
INTERNATIONAL
CASE BASED REVIEW
Protracted febrile myalgia syndrome as the first manifestation of familial Mediterranean fever in children: case‑based review Kübra Öztürk1 · Mustafa Çakan2 Received: 13 July 2020 / Accepted: 27 August 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Protracted febrile myalgia syndrome (PFMS) is one of the rare conditions characterized by long standing severe myalgia and fever in familial Mediterranean fever (FMF) patients. Laboratory studies show elevated values of inflammatory markers and normal creatinine phosphokinase (CPK) levels. Rarely, PFMS may be the first manifestation of FMF and this makes the diagnosis of FMF and PFMS challenging. The aim of this report was to describe case series of PFMS patients without previous diagnosis of FMF and discuss with other pediatric PFMS cases described in the literature. Six patients with PFMS as the first manifestation of FMF from two Pediatric Rheumatology clinics were presented. The male: female ratio was 1:1. The median (min–max) age at diagnosis was 7.05 (5.5–15.5) years. All patients had severe myalgia. No fever was detected in two patients. Only one patient had rash. Markedly elevated acute phase reactants were observed in all patients. CPK levels were normal in all patients. Also, we searched the PubMed/MEDLINE, Google Scholar, Web of Science and Scopus databases from inception to May 2020, using the keywords ‘familial Mediterranean fever, protracted febrile myalgia, child’ and 52 pediatric PFMS were found. In the literature, three patients did not have fever, and nineteen patients were not previously diagnosed with FMF as our patients. As a conclusion, PFMS may be the first manifestation of FMF. It should be suspected in cases with prolonged severe myalgia with or without fever, and high acute phase reactants. Keywords Familial Mediterranean fever · Myalgia · Child
Introduction Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by recurrent attacks of fever, abdominal pain, chest pain, arthralgia, and myalgia resolving within 1–3 days [1]. Protracted febrile myalgia syndrome (PFMS) in FMF patients was first described by Langevitz et al. in 1994 as a vasculitic condition characterized by severe myalgia, fever, abdominal pain, diarrhea, and arthralgia/arthritis episodes lasting for 4–6 weeks [2]. Laboratory studies typically show markedly elevated values of inflammatory * Kübra Öztürk [email protected] Mustafa Çakan [email protected] 1
Gaziantep Cengiz Gökçek Children Hospital, Clinic of Pediatric Rheumatology, 15 Temmuz Mahallesi, 148063 Cadde, No 4, 27010 Sehitkamil, Gaziantep, Turkey
Şanlıurfa Research and Training Hospital, Clinic of Pediatric Rheumatology, Yenice Mah, 63250 Şanlıurfa, Turkey
2
markers. Creatinine phosphokinase (CPK) levels are always normal. Affected patients have consistently been shown to carry one or two mutations in the pyrin-encoding the MEditerranean FeV
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