Turkiye Klinikleri Journal of Pediatrics

.: ORIGINAL RESEARCH
Çocukluk Çağı Ailevi Akdeniz Ateşi Hastalarına Eşlik Edebilen Hastalıklar
Coexistent Diseases with Familial Mediterranean Fever in Childhood
Deniz GEZGİN YILDIRIMa, Oğuz SÖYLEMEZOĞLUb
aGazi Üniversitesi Tıp Fakültesi, Çocuk Romatolojisi BD, Ankara, TÜRKİYE
bGazi Üniversitesi Tıp Fakültesi, Çocuk Nefrolojisi BD, Ankara, TÜRKİYE
Turkiye Klinikleri J Pediatr. 2021;30(1):8-14
doi: 10.5336/pediatr.2020-78451
Article Language: TR
Full Text
ÖZET
 Amaç: Ailevi Akdeniz ateşi (AAA) hastalığı, tekrarlayan ateş ve serözit atakları ile karakterize otoinflamatuar bir hastalıktır. Artmış inflamasyon atakları, ek hastalıkların ortaya çıkmasına katkıda bulunabilir. Çalışmamızın amacı, pediatrik AAA hastalarına eşlik edebilen diğer hastalıkların görülme sıklığını araştırmaktır. Gereç ve Yöntemler: Sekiz yüz elli sekiz AAA hastası içinde, eşlik eden ek hastalığı olan 90 hastanın tıbbi bilgileri, geriye dönük olarak medikal dosyalarından incelendi. Hastaların güncel yaşı, AAA tanı yaşı, cinsiyeti, klinik özellikleri, tedavileri, aile öyküsü, Mediterranean fever gen analizi, Pras aktivite skoru, eşlik eden hastalıkları ve laboratuvar tetkikleri kayıt edildi. Bulgular: Sekiz yüz elli sekiz AAA hastasının arasından 90 (%10,5) hastada eşlik eden ek hastalık tespit edildi. Yirmi yedi (%30) hastada immünglobulin A vasküliti (Henoch-Schönlein purpurası), 15 (%17) hastada periyodik ateş, aftöz stomatit, farenjit ve lenfadenit hastalığı, 12 (%13) hastada juvenil idiyopatik artrit, 12 (%13) hastada inflamatuar bağırsak hastalığı (İBH), 7 (%8) hastada AAA hastalığına ikincil sakroileit, 4 (%5) hastada Behçet hastalığı, 3 (%3) hastada astım, 3 (%3) hastada poliarteritis nodoza, 2 (%2,5) hastada Kawasaki hastalığı, 2 (%2,5) hastada kronik tekrarlayan multifokal osteomiyelit, 1 (%1) hastada üveit, 1 (%1) hastada ürtikeryal vaskülit ve 1 (%1) hastada multipl skleroz hastalığı vardı. Yirmi sekiz (%31) hastada M694V homozigot mutasyon var iken, 30 (%33) hastada M694V heterozigot mutasyon tespit edildi. Sonuç: Otoinflamatuar bir hastalık olan AAA hastalığına vaskülitler, kronik artrit ve İBH gibi diğer otoinflamatuar ve otoimmün hastalıklar sık eşlik etmektedir. AAA hastalarının takibinde gelişebilecek ek hastalıklar açısından klinisyenler, ayırıcı tanıda bu hastalıklar açısından da dikkatli olmalıdırlar.

Anahtar Kelimeler: Ailevi akdeniz ateşi; inflamasyon; komorbidite
ABSTRACT
 Objective: Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by recurrent fever and serositis. Increased inflammation could contribute to occur concomitant diseases. The aims of this study are to investigate the incidence of concomitant diseases with FMF. Material and Methods: The medical records of 90 patients with coexistence diseases among 858 FMF patients were investigated retrospectively from their medical files. The age of onset, gender, clinical features, treatments, family history, Mediterranean fever gene analysis, Pras activity score, concomitant diseases and laboratory studies of patients were recorded. Results: Among 858 FMF patients, additional coexistence disease was identified in 90 (10.5%) patients. Immunoglobulin A vasculitis (Henoch-Schönlein purpura) was occurred in twenty seven (30%) patients, periodic fever, aphthous stomatitis, pharyngitis and lymphadenitis disease in 15 (17%), juvenile idiopathic arthritis in 12 (13%), inflammatory bowel disease (IBD) in 12 (13%), sacroiliitis associated with FMF in 7 (8%), Behçet's disease in 4 (5%), asthma in 3 (3%), polyarteritis nodosa in 3 (3%), Kawasaki disease in 2 (2.5%), chronic recurrent multifocal osteomyelitis in 2 (2.5%), uveitis in 1 (1%), urticartial vasculitis in 1 (1%) and multiple sclerosis in 1 (1%) patient. Twenty-eight (31%) patients had M694V homozygous mutation, while 30 (33%) patients had M694V heterozygous mutation. Conclusion: FMF which is an autoinflammatory disease is often accompanied with other autoinflammatory and autoimmune diseases such as vasculitis, chronic arthritis and IBD. Clinicians should be aware of in differential diagnosis of additional diseases that may develop in follow-up of FMF patients.

Keywords: Familial mediterranean fever; inflammation; comorbidity
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