Familial Mediterranean fever with Prolonged Febrile Myalgias Syndrome: Clinical, Laboratory and Demographic Characteristics of 9 Patients

Yıl 2018, , 227 - 232, 30.09.2018

Alaaddin Yorulmaz , Hikmet Akbulut , Şükrü Arslan

https://doi.org/10.16899/gopctd.456887

Öz

Objective: The
aim of this study is to retrospectively review a group of patients with
prolonged febrile myalgias syndrome in patients with FMF and to share their
clinical experience.

Materials and Methods:
Nine patients with prolonged febrile myalgias syndrome were identified from the
electronic database of a Selçuk University Medical Faculty Pediatric Emergency
Department and Pediatric Rheumatology Polyclinic (September 2016-May 2018). Their
clinical and laboratory data were collected. Data such as age, sex,
accompanying symptoms of FMF, previous follow-up of FMF, duration of follow-up
after diagnosis, hospital stay, number of days before symptoms, complete blood
count and acute phase reactants were recorded. Pulse corticosteroid therapy was
administered at a dose of 10mg/kg.

Results In
the study, 7 of 9 patients (77.8%) were females and 2 (22.2%) were male. The
ages of our patients ranged from 39 months to 192 months and the mean median
was 145 months. Only 2 (22.2%) of the patients had previously been diagnosed
and received colchicine treatment. The first sign of FMF in 7 of patients
(77.78%) was PFMS. When genetic analysis of the patients was examined, M694V
homozygous was detected in 4 patients (44.44%). Acute phase reactors were at
high level in all patients. Creatine kinase levels were within normal limits in
all patients. The patients received intravenous methylprednisolone. In all
patients, complete recovery was seen after treatment.

Conclusion:
PFMS should be considered in the presence of high fever, acute phase reactant
height, and long-standing myalgia that does not meet FMF criteria.

Anahtar Kelimeler

Familial Mediterranean fever; protracted febrile myalgia; child

Uzamış Febril Myalji Sendromu ile başvuran Ailesel Akdeniz ateşi: 9 Hastanın Klinik, Laboratuvar ve Demografik Özellikleri

Öz

Amaç: Bu
çalışmanın amacı AAA’li hastalarda uzamış febril myalji sendromlu bir grup
hastayı geriye dönük olarak incelemek ve klinik tecrübemizi paylaşmaktır.

Gereç ve Yöntemler:
UFMS'li dokuz hasta Selçuk Üniversitesi Tıp Fakültesi Pediatri Acil Servisi ve
Pediatrik Romatoloji Polikliniği (Eylül 2016- Mayıs 2018) elektronik veri
tabanından tanımlandı. Klinik ve laboratuvar verileri toplandı. Hastaların
yaşı, cinsiyet, eşlik ettiği semptomlar, AAA’un eski tanı ise takip süresi,
tanı konulduktan sonraki takip süresi, hastanede yatış süresi, semptomların kaç
gün öncesinden başladığı, tam kan sayımı, akut faz reaktanları gibi veriler
kaydedildi.

Bulgular: Çalışmada 9 hastanın 7'si (% 77.8)
kadın, 2'si (% 22.2) erkekti. Hastalarımızın yaşları 39 ay ile 192 ay
arasında değişmekte olup median ortalaması 145 ay idi. Hastaların sadece 2’sine
(%22,2) daha önce tanı konulmuş olup kolşisin tedavisi almaktaydı. Hastaların
7’sinde (%77,78) AAA’inin ilk belirtisi FMS idi. Hastaların genetik analizine
bakıldığında 4 hastada (%44,44) M694V homozigot tespit edildi. Tüm hastalarda
akut faz reaktanları yüksek seviyede idi. Kreatin kinaz düzeyi tüm hastalarda
normal sınırlardaydı. Pulse kortikosteroid tedavisi, 10 mg / kg dozunda
uygulandı. Tüm hastalarda tedavi sonrası tam iyileşme görüldü.

Sonuç: AAA
kriterlerini karşılamayan, yüksek ateşle birlikte akut faz reaktanı
yüksekliğinin olduğu ve uzun süren myalji varlığında UFMS düşünülmelidir.

Anahtar Kelimeler

Ailesel Akdeniz ateşi; Uzamış febril myalji; Çocuk

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