Familial Mediterranean fever (FMF) and Sjögren’s syndrome: A case responding to azathioprine treatment
Abstract
SUMMARY
Sjögren’s syndrome (SS) is a chronic, autoimmune, systemic exocrinopathy and extraglandular manifestations can be seen in the course of the disease. Vasculitic skin lesions can rarely be seen in patients. Familial Mediterranean fever (FMF) is a familial, recurrent, autoinflammatory disease which is seen mostly in young people and characterized by self-limiting attacks responding to colchicine treatment. However, resistance to colchicine might be seen in some cases. Coexistence of FMF and SS is rare and the use of azathioprine (AZA) for the treatment of resistant FMF is yet controversial. Herein, we aim to share the post-treatment positive findings of a colchicine-resistant patient diagnosed with FMF and SS comorbiding vasculitis treated with AZA.
Keywords: azathioprine, resistant-FMF, Sjögren’s syndrome, vasculitis
ÖZET
Sjögren sendromu (SS), kronik, otoimmün, sistemik ekzokrinopatidir ve ekstarglandüler bulgular hastalığın seyrinde görülebilir. Bazı hastalarda nadirende olsa vaskülitik cilt lezyonları görülebilmektedir. Ailevi Akdeniz ateşi (AAA), ailevi otoinflamatuvar bir hastalık olup daha çok genç yaştaki bireylerde tekrarlayan, kendi kendini sınırlayan ataklarla karakterize ve tedavisinde daha çok kolşisin ile yanıt alınan bir hastalıktır. Fakat bazı vakalarda kolşisine direnç olmaktadır. AAA ve SS birlikteği nadir olup özellikle dirençli-AAA tedavisinde azatioprin (AZA) kullanımı da henüz tartışmalıdır. Biz burada AAA ve vaskülitin eşlik ettiği SS’lu AZA ile tedavi edilen kolşisin dirençli hastada ortaya çıkan anlamlı yanıtı sunmak istedik.
Anahtar sözcükler: Azatiyoprin, dirençli-AAA, Sjögren sendromu, vaskülit
References
- Vitali C, Bombardieri S, Jonsson R. Sjögren’s syndrome: A revised version of the European criteria proposed European Consensus Group. Ann American- Rheum Dis 2002; 61: 544-58.
- Fox RI. Sjögren’s syndrome. Lan- cet 2005; 366: 321-31.
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren’s syn- drome new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64: 347-54.
- Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet 1998; 351: 659-64.
- Tanaka M, Migita K, Miyashita T, Maeda Y, Nakamura M, Komori A, Ishibashi H, Eguchi K, Kikuchi M, Hirayama K, Yasunami M. Co- existence of familial Mediterrane- an fever and Sjögren’s syndrome in a Japanese patient. Clin Exp Rheumatol 2007; 25: 792.
- Sayarlioglu H, Sayarlioglu M, Dogan E, Soyoral Y. Successful treatment of ne- phrotic syndrome due to FMF am- yloidosis with azathioprine: Report of three Turkish cases. Rheumatol Int 2006; 27: 197-9. R,
- Korkmaz C. Immunosuppressive treatment of AA amyloidosis of familial Mediterranean fever. Clin Exp Rheumatol 2005; 23: 107-8.
- Erten S, Erzurum C, Altunoglu A. Three family members with famil- ial Mediteranean fever carrying the M694V mutation showed different clinical presentation. Intern Med 2012; 51: 1765-8.
- Aksu K, Keser G. Coexistence of vasculitides with familial Mediter- anean fever. Rheumatol Int 2011; 31: 1263-74.
- Akalın T, Demirag MD, Tezcan ME, Ozturk MA. Scleritis and sudden hearing loss associated with familial Mediterranean fever. Clin Exp Rheumatol 2010; 28: 103-4.
- Alpay N, Sumnu A, Caliskan Y, Yazici H, Turkmen A, Gul A. Ef- ficacy of anakinra treatment in a patient with colchicine-resistant familial Rheumatol Int 2012; 32: 3277-9.
- Livneh A, Langevitz P, Zemer D. The changing face of familial Mediterranean fever. Semin Ar- thritis Rheum 1996; 26: 612-27.
- Cerquaglia C, Diaco M, Nucera G, La Regina M, Montalto M, Manna R. Pharmacological and clinical basis of treatment of familial Med- iterranean fever (FMF) with col- chicine or analogues: An update. Curr Drug Targets Inflamm Aller- gy 2005; 4: 117-24.
- Ozgocmen S, Akgul O. Anti-TNF agents in familial Mediterranean fever: Report of three cases and review of the literature. Mod Rheumatol 2011; 21: 684-90.
- Fujikawa K, Migita K, Tsukada T, Umeda M, Nonaka F, Kawakami A, Eguchi K. Interleukin-6 target- ing therapy in familial Mediterra- nean fever. Clin Exp Rheumatol 2013; 31: 150-1.
Abstract
Sjögren sendromu (SS), kronik, otoimmün, sistemik ekzokrinopatidir ve ekstarglandüler bulgular hastalığın seyrinde görülebilir. Bazı hastalarda nadirende olsa vaskülitik cilt lezyonları görülebilmektedir. Ailevi Akdeniz ateşi (AAA), ailevi otoinflamatuvar bir hastalık olup daha çok genç yaştaki bireylerde tekrarlayan, kendi kendini sınırlayan ataklarla karakterize ve tedavisinde daha çok kolşisin ile yanıt alınan bir hastalıktır. Fakat bazı vakalarda kolşisine direnç olmaktadır. AAA ve SS birlikteği nadir olup özellikle dirençli-AAA tedavisinde azatioprin (AZA) kullanımı da henüz tartışmalıdır. Biz burada AAA ve vaskülitin eşlik ettiği SS’lu AZA ile tedavi edilen kolşisin dirençli hastada ortaya çıkan anlamlı yanıtı sunmak istedik
Keywords
References
- Vitali C, Bombardieri S, Jonsson R. Sjögren’s syndrome: A revised version of the European criteria proposed European Consensus Group. Ann American- Rheum Dis 2002; 61: 544-58.
- Fox RI. Sjögren’s syndrome. Lan- cet 2005; 366: 321-31.
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren’s syn- drome new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64: 347-54.
- Ben-Chetrit E, Levy M. Familial Mediterranean fever. Lancet 1998; 351: 659-64.
- Tanaka M, Migita K, Miyashita T, Maeda Y, Nakamura M, Komori A, Ishibashi H, Eguchi K, Kikuchi M, Hirayama K, Yasunami M. Co- existence of familial Mediterrane- an fever and Sjögren’s syndrome in a Japanese patient. Clin Exp Rheumatol 2007; 25: 792.
- Sayarlioglu H, Sayarlioglu M, Dogan E, Soyoral Y. Successful treatment of ne- phrotic syndrome due to FMF am- yloidosis with azathioprine: Report of three Turkish cases. Rheumatol Int 2006; 27: 197-9. R,
- Korkmaz C. Immunosuppressive treatment of AA amyloidosis of familial Mediterranean fever. Clin Exp Rheumatol 2005; 23: 107-8.
- Erten S, Erzurum C, Altunoglu A. Three family members with famil- ial Mediteranean fever carrying the M694V mutation showed different clinical presentation. Intern Med 2012; 51: 1765-8.
- Aksu K, Keser G. Coexistence of vasculitides with familial Mediter- anean fever. Rheumatol Int 2011; 31: 1263-74.
- Akalın T, Demirag MD, Tezcan ME, Ozturk MA. Scleritis and sudden hearing loss associated with familial Mediterranean fever. Clin Exp Rheumatol 2010; 28: 103-4.
- Alpay N, Sumnu A, Caliskan Y, Yazici H, Turkmen A, Gul A. Ef- ficacy of anakinra treatment in a patient with colchicine-resistant familial Rheumatol Int 2012; 32: 3277-9.
- Livneh A, Langevitz P, Zemer D. The changing face of familial Mediterranean fever. Semin Ar- thritis Rheum 1996; 26: 612-27.
- Cerquaglia C, Diaco M, Nucera G, La Regina M, Montalto M, Manna R. Pharmacological and clinical basis of treatment of familial Med- iterranean fever (FMF) with col- chicine or analogues: An update. Curr Drug Targets Inflamm Aller- gy 2005; 4: 117-24.
- Ozgocmen S, Akgul O. Anti-TNF agents in familial Mediterranean fever: Report of three cases and review of the literature. Mod Rheumatol 2011; 21: 684-90.
- Fujikawa K, Migita K, Tsukada T, Umeda M, Nonaka F, Kawakami A, Eguchi K. Interleukin-6 target- ing therapy in familial Mediterra- nean fever. Clin Exp Rheumatol 2013; 31: 150-1.
Details
| Primary Language | English |
|---|---|
| Journal Section | Case Reports |
| Authors | |
| Publication Date | September 18, 2015 |
| Published in Issue | Year 2015 |