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

Year 2018, , 227 - 232, 30.09.2018

Alaaddin Yorulmaz , Hikmet Akbulut , Şükrü Arslan

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

Abstract

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.

Keywords

Familial Mediterranean fever; protracted febrile myalgia; child

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

Abstract

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.

Keywords

Ailesel Akdeniz ateşi; Uzamış febril myalji; Çocuk

References

• KAYNAKLAR1. Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet 1998; 351: 659-664.
• 2. Livneh A, Pras M, Padeh S, et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997; 40: 1879-1885.
• 3. Sohar E, Gafhi J, Pras M, et al. Familial Mediterranean fever: A survey of 470 cases and review of the literature. Am J Med 1967; 43: 227-253.
• 4. Eliakim M, Levy M, Ehrenfeld M. Recurrent polyserositis. Amsterdam- New York-Oxford: Elsevier/North-Holland Biomedical Press; 1981:15.
• 5. Yilmaz E, Ozen S, Balci B, Duzova A, Topaloglu R, Besbas N, et al. Mutation frequency of Familial Mediterranean fever and evidence for a high carrier rate in the Turkish population. Eur J Hum Genet 2001; 9: 553-555.
• 6. Dinc A, Pay S, Turan M, Caglar K, Can C. Prevalence of Familial Mediterranean fever in young Turkish men. Clin Exp Rheumatol 2000; 18: 787-788.
• 7. Majeed HA, Al-Qudah AK, Qubain H, et al. The clinical patterns of myalgia in children with familial Mediterranean fever. Semin Arthritis Rheum. 2000; 30:138-143.
• 8. Touitou I. The spectrum of familial Mediterranean fever (FMF) mutations. Eur J Hum Genet. 2001; 9: 473-483.
• 9. Tekin M, Yalcinkaya F, Tumer N, et al. Clinical, laboratory and molecular characteristics of children with familial Mediterranean fever-associated vasculitis. Acta Paediatr 2000; 89: 177-182.
• 10. Ozdogan H, Arisoy N, Kasapcopur O, et al. Vasculitis in familial Mediterranean fever. J Rheumotol 1997; 24: 323-327.
• 11. Langevitz P, Zemer D, Livneh A, et al. Protracted febrile myalgia in patients with familial Mediterranean fever. J Rheumatol 1994; 21: 1708-1709.
• 12. Kaplan E, Mukamel M, Barash J, Brik R, Padeh S, Berkun Y, et al. Protracted febrile myalgia in children and young adults with familial Mediterranean fever: analysis of 15 patients and suggested criteria for working diagnosis. Clin Exp Rheumatol 2007; 25: 114-117
• 13. Mauldin J, Cameron HD, Jeanotte D, Solomon G, Jarvis JN. Chronic arthritis in children and adolescents in two Indian health service user populations. BMC Musculoskeletal Disorders 2004; 5: 1-7.
• 14. Davies K, Copeman A. The spectrum of paediatric and adolescent rheumatology. Best Pract Res Clin Rheumatol 2006; 20: 179-200.
• 15. Yalçinkaya F. Ozen S. Ozçakar ZB ve ark. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford) 2009; 48: 395-398.
• 16. Odabas AR, Cetinkaya R, Selcuk Y, et al. Familial Mediterranean fever. South Med 2002; 95: 1400-1403.
• 17. Koşan C, Ailevi Akdeniz Ateşi’ ne Tanısal Yaklaşım. Atatürk Üniversitesi Tıp Dergisi 2003; 35: 1-6
• 18. Öztürk C, Sütçüoğlu S, Özer E. Uzamış febril miyalji ile prezente olan ailevi Akdeniz ateşi olgusu. İzmir Behçet Uz Çocuk Hast. Dergisi 2013; 3: 63-66
• 19. Peru H, Karagül C, Karagöl HİE. Uzamış febril miyalji ile ortaya çıkan ailesel Akdeniz ateşi olgusu. Ankara Üniversitesi Tıp Fakültesi Mecmuası 2008; 61: 93-95.
• 20. Öztürk C, Halıcıoğlu O, Coker I ve ark. Association of clinical and genetical features in FMF with focus on MEFV strip assay sensitivity in 452 children from western Anatolia, Turkey. Clin Rheumatol 2012; 31: 493-501.
• 21. Schwabe AD, Peters RS. Familial Mediterranean fever in Armenians. Analysis of 100 cases. Medicine 1974; 53: 453-462.
• 22. Majeed HA, Rawashdeh M, el-Shanti H, Qubain H, Khuri-Bulos N, Shahin HM. Familial Mediterranean fever in children: the expanded clinical profile. Q J Med 92: 309-318.
• 23. Livneh A, Langevitz P. Diagnostic and treatment concerns in familial Mediterranean fever. Baillieres Best Pract Res Clin Rheumatol 2000; 14: 477-498
• 24. Odabas AR, Cetinkaya R, Selcuk Y, et al. Severe and prolonged febrile myalgia in familial Mediterranean fever. Scand J Rheumatol 2000; 29: 394-395.
• 25. Ertekin V, Selimoglu MA, Alp H, Yılmaz N. Familial Mediterranean fever protracted febrile myalgia in children: report of two cases. Rheumatol Int 2005; 25: 398-400.
• 26. Soylu A, Kasap B, Türkmen M, Salyam GS, Kavukcu S. Febrile Myalgia Syndrome in Familial Mediterranean Fever. J Clin Rheumatol 2006; 12: 93-6.
• 27. Majeed HA, El-Khateeb M, El-Shanti, H, Rabaiha ZA, et al. The spectrum of familial mediterranean fever gene mutations in Arabs: Report of a large series. Semin Artritis Rheum 2005; 34: 813-818.
• 28. Sıdı G, Shınar Y, Lıvneh A, Langevıtz P, Pras M, Pras E: Protracted febrile myalgia of familial Mediterranean fever. Mutation analysis and clinical correlations. Scand J Rheumatol 2000; 29: 174-176.
• 29. Zaks N, Shınar Y, Padesh et al.: Analysis of the three most common MEFV mutations in 412 patients with familial Mediterranean fever. Isr Med Assoc J 2003; 5: 585-588.
• 30. Schlesinger M, Kopolovic J, Viskoper RJ, Ron N. A case of familial Mediterranean fever with cutaneous vasculitis and immune complex nephritis: Light, electron, and immunofluorescent study of renal biopsy. Am. J. Clin. Pathol. 1983; 80: 511-514.
• 31. Duru S, Civilibal M, Karakoyun M, Payasli M, Elevli M. Protracted febrile myalgia in two children with familial Mediterranean fever. Pediatr Int 2010; 52: 137-140.
• 32. Eran R, Gil A, Yoel L, Efraim B, Ori G, Jacob A, et al. Protracted febrile myalgia syndrome treated with pulse of corticosteroids. Semin Arthritis Rheum.2018 Jun;47: 897-899.