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
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
- Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet. 1998;351(9103):659-64.[Crossref] [PubMed]
- Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, et al; Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005;84(1):1-11.[Crossref] [PubMed]
- Soylemezoglu O, Kandur Y, Gonen S, Düzova A, Özçakar ZB, Fidan K, et al. Familial Mediterranean fever gene mutation frequencies in a sample Turkish population. Clin Exp Rheumatol. 2016;34(6 Suppl 102):97-100.[PubMed]
- French FMF Consortium. A candidate gene for familial Mediterranean fever. Nat Genet. 1997;17(1):25-31.[Crossref] [PubMed]
- Touitou I. The spectrum of Familial Mediterranean Fever (FMF) mutations. Eur J Hum Genet. 2001;9(7):473-83.[Crossref] [PubMed]
- Lidar M, Livneh A. Familial Mediterranean fever: clinical, molecular and management advancements. Neth J Med. 2007;65(9):318-24.[PubMed]
- Lachmann HJ, Sengül B, Yavuzşen TU, Booth DR, Booth SE, Bybee A, et al. Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford). 2006;45(6):746-50.[Crossref] [PubMed]
- Ben-Zvi I, Livneh A. Chronic inflammation in FMF: markers, risk factors, outcomes and therapy. Nat Rev Rheumatol. 2011;7(2):105-12.[Crossref] [PubMed]
- Ben-Chetrit E, Levy M. Colchicine: 1998 update. Semin Arthritis Rheum. 1998;28(1):48-59.[Crossref] [PubMed]
- El Hasbani G, Jawad A, Uthman I. Update on the management of colchicine resistant Familial Mediterranean Fever (FMF). Orphanet J Rare Dis. 2019;14(1):224.[Crossref] [PubMed] [PMC]
- Cattan D. MEFV mutation carriers and diseases other than familial Mediterranean fever: proved and non-proved associations; putative biological advantage. Curr Drug Targets Inflamm Allergy. 2005;4(1):105-12.[Crossref] [PubMed]
- Ozdogan H, Arisoy N, Kasapçapur O, Sever L, Calişkan S, Tuzuner N, et al. Vasculitis in familial Mediterranean fever. J Rheumatol. 1997;24(2):323-7.[PubMed]
- Yildiz M, Adrovic A, Tasdemir E, Baba-Zada K, Aydin M, Koker O, et al. Evaluation of co-existing diseases in children with familial Mediterranean fever. Rheumatol Int. 2020;40(1):57-64.[Crossref] [PubMed]
- Özçakar ZB, Çakar N, Uncu N, Çelikel BA, Yalçinkaya F. Familial Mediterranean fever-associated diseases in children. QJM. 2017;110(5):287-90.[PubMed]
- Paç Kısaarslan A, Özdemir Çiçek S, Şahin N, Taşkın SN, Poyrazoğlu H, Gündüz Z, et al. [Diseases accompanying familial Mediterranean fever: a single-centre experience]. Bozok Med J. 2020;10(2):105-10.[Link]
- Kişla Ekinci RM, Balci S, Ufuk Altintaş D, Yilmaz M. The influence of concomitant disorders on disease severity of familial Mediterranean fever in children. Arch Rheumatol. 2017;33(3):282-7.[Crossref] [PubMed] [PMC]
- Yalçinkaya F, Ozen S, Ozçakar ZB, Aktay N, Cakar N, Düzova A, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford). 2009;48(4):395-8.[Crossref] [PubMed]
- Pras E, Livneh A, Balow JE Jr, Pras E, Kastner DL, Pras M, et al. Clinical differences between North African and Iraqi Jews with familial Mediterranean fever. Am J Med Genet. 1998;75(2):216-9.[Crossref] [PubMed]
- Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016;75(4):644-51.[Crossref] [PubMed]
- Michet CJ. Epidemiology of vasculitis. Rheum Dis Clin North Am. 1990;16(2):261-8.[PubMed]
- Tekin M, Yalçinkaya F, Tümer N, Cakar N, Koçak H, Ozkaya N, et al. Familial Mediterranean fever--renal involvement by diseases other than amyloid. Nephrol Dial Transplant. 1999;14(2):475-9.[Crossref] [PubMed]
- Ozen S, Ben-Chetrit E, Bakkaloglu A, Gur H, Tinaztepe K, Calguneri M, et al. Polyarteritis nodosa in patients with Familial Mediterranean Fever (FMF): a concomitant disease or a feature of FMF? Semin Arthritis Rheum. 2001;30(4):281-7.[Crossref] [PubMed]
- Yalçinkaya F, Ozçakar ZB, Kasapçopur O, Oztürk A, Akar N, Bakkaloğlu A, et al. Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa. J Pediatr. 2007;151(6):675-8.[Crossref] [PubMed]
- Ozçakar ZB, Yalçinkaya F, Cakar N, Acar B, Kasapçopur O, Ugüten D, et al. MEFV mutations modify the clinical presentation of Henoch-Schönlein purpura. J Rheumatol. 2008;35(12):2427-9.[Crossref] [PubMed]
- Schwartz T, Langevitz P, Zemer D, Gazit E, Pras M, Livneh A. Behçet's disease in Familial Mediterranean fever: characterization of the association between the two diseases. Semin Arthritis Rheum. 2000;29(5):286-95.[Crossref] [PubMed]
- Atas N, Armagan B, Bodakci E, Satis H, Sari A, Bilge NSY, et al. Familial Mediterranean fever is associated with a wide spectrum of inflammatory disorders: results from a large cohort study. Rheumatol Int. 2020;40(1):41-8.[Crossref] [PubMed]
- Gaggiano C, Rigante D, Sota J, Grosso S, Cantarini L. Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clin Rheumatol. 2019;38(1):11-7.[Crossref] [PubMed]
- Berkun Y, Levy R, Hurwitz A, Meir-Harel M, Lidar M, Livneh A, et al. The familial Mediterranean fever gene as a modifier of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome. Semin Arthritis Rheum. 2011;40(5):467-72.[Crossref] [PubMed]
- Yener GO, Tekin ZE, Girişgen İ, Çetin EN, Akdağ B, Yüksel S. Juvenile idiopathic arthritis in a center in the Western Anatolia region in Turkey. Turk Pediatri Ars. 2020;55(2):157-65.[PubMed] [PMC]
- Aydin F, Özçakar ZB, Çakar N, Çelikel E, Uncu N, Çelikel Acar B, et al. Sacroiliitis in children with familial Mediterranean fever. J Clin Rheumatol. 2019;25(2):69-73.[Crossref] [PubMed]
- Yıldırım DG, Fidan HK, Gönen S, Söylemezoğlu O. Sacroiliitis associated with familial Mediterranean fever in childhood: a case series and review of literature. Turk J Pediatr. 2020;62(2):175-81.[Crossref] [PubMed]
- Cattan D, Notarnicola C, Molinari N, Touitou I. Inflammatory bowel disease in non-Ashkenazi Jews with familial Mediterranean fever. Lancet. 2000;355(9201):378-9. Erratum in: Lancet 2000;355(9208):1020.[Crossref] [PubMed]
- Lee JJY, Stimec J, Cameron B, Laxer RM. Concurrent chronic recurrent multifocal osteomyelitis and familial Mediterranean fever. A case report. Clin Exp Rheumatol. 2018;36(6 Suppl 115):151.[PubMed]
- Ozdogan H, Ugurlu S, Uygunoglu U, Tutuncu M, Gul A, Akman G, et al. The efficacy of anti- IL-1 treatment in three patients with coexisting familial Mediterranean fever and multiple sclerosis. Mult Scler Relat Disord. 2020;45:102332.[Crossref] [PubMed]
- Barut K, Guler M, Sezen M, Kasapçopur O. Inceased frequency of psoriasis in the families of the children with familial Mediterranean fever. Clin Exp Rheumatol. 2016;34(6 Suppl 102):S137.[PubMed]
- Erden A, Batu ED, Seyhoğlu E, Sari A, Sönmez HE, Armagan B, et al. Increased psoriasis frequency in patients with familial Mediterranean fever. Ups J Med Sci. 2018;123(1):57-61.[Crossref] [PubMed] [PMC]
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