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КЛИНИКАЛЫҚ ЖАҒДАЙ/КЛИНИЧЕСКИЙ СЛУЧАЙ/ CASE REPORT
Материал поступил в редакцию: 20-09-2014 Материал принят к печати: 11-10-2014 УДК: 616-002.77
Coexistent with ankylosing spondylitis and familial mediterranean fever
Ilknur Sahin1, Ilknur Albayrak2, Fatih Pektas1, Sevket Arslan1, Ali Yavuz Karahan3, Adem Kucuk1
1Necmettin Erbakan University, Konya, Turkey 2Selcuk University, Konya, Turkey 3Beyhekim State Hospital, Konya, Turkey
Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent attacks of fever and serositis. Limited data suggest that the prevalence of sacroiliitis is increased in patients with FMF. Ankylosing spondylitis (AS) is a common inflammatory rheumatic disease characterized by inflammation of the axial joints, peripheral oligoarthritis, and enthesitis. Symptoms related to back pain should be investigated thoroughly in order to diagnose AS and distinguish symptoms related to it from FMF. We presented a 46 year old man who diagnosed FMF and AS.
Key words: familial Mediterranean fever, ankylosing spondylitis, inflammation
J Clin Med Kaz 2014; 3(33): 29-31.
Address correspondence to: Adem Kucuk, MD. Division of Rheumatology, Department of Internal Medicine, Meram School of Medicine, Necmettin Erbakan University, Meram, Konya, Turkey. Email: drademk@yahoo.com, Tel: +903322237516, Fax: +903323243982
БЕХТЕРЕВ АУРУЫНЫҢ ЖЕРОРТА ТЕҢІЗІ ОТБАСЫЛЫҚ ҚЫЗБАСЫМЕН ҚОСАРЛАСЫП КЕЗДЕСУІ
Ilknur Sahin1, Ilknur Albayrak2, Fatih Pektas1, Sevket Arslan1, Ali Yavuz Karahan3, Adem Kucuk1
1 Неджметтин Эрбакан университеті, Кония, Tүркия
2Селчук университеті, Кония, Tүркия
3Бейеким мемлекеттік ауруханасы, Кония, Tүркия
Жерорта теңізі отбасылық қызбасы жедел дамитын қабынумен сипатталатын ауру. Бұл қызбаға дене қызуының қайталанбалы ұстамалары мен серозит тән. Шектелген мәліметтердің нәтижесі бойынша, Жерорта теңізі отбасылық қызбасы бар науқастарда сакроилеиттің таралуы жиі кездеседі. Бехтерев ауруы шеткі олигоартрит және энтезитпен көрініс табатын, осьтік буындардың қабынуымен сипатталатын ревматикалық ауру. Арқа тұсында болатын ауру сезіміне шағымдалатын науқастарды Бехтерев ауруын диагноздау үшін мұқият тексеру керек. Алайда, осы тұста Жерорта теңізі отбасылық қызбасына тән симптомдарды да есепке алудың маңызы зор. Біз Жерорта теңізі отбасылық қызбасы мен Бехтерев ауруы диагноздары қойылған 46-жастағы ер адамның клиникалық жағдайын ұсынып отырмыз.
Маңызды сөздер: Жерорта теңізі отбасылық қызбасы, Бехтерев ауруы, қабыну.
СЛУЧАЙ СОЧЕТАНИЯ БОЛЕЗНИ БЕХТЕРЕВА С СЕМЕЙНОЙ СРЕДИЗЕМНОМОРСКОЙ ЛИХОРАДКОЙ
Ilknur Sahin1, Ilknur Albayrak2, Fatih Pektas1, Sevket Arslan1, Ali Yavuz Karahan3, Adem Kucuk1
1 Университет Неджметтин Эрбакан, Конья, Хурция
2 Университет Селчук, Конья, Хурция
3 Государственная больница Бейеким, Конья, Хурция
Семейная средиземноморская лихорадка является воспалительным заболеванием, которая возникает внезапно. Для семейной средиземноморской лихорадки характерны периодически повторяющиеся приступы лихорадки и серозит. Ограниченные данные свидетельствуют, что распространенность сакроилеита увеличивается у пациентов с семейной средиземноморской лихорадкой. Болезнь Бехтерева - это ревматическая болезнь, которая характеризуется воспалением осевых суставов с периферическим олигоартритом и энтезитом. Пациенты с симптомами, связанными с болью в спине должны быть тщательно дообследованы для диагностики болезни Бехтерева. Здесь также важно отличить симптомы, вызванные семейной средиземноморской лихорадкой. Мы представили случай 46-летнего мужчины, которому выставлен диагноз семейная средиземноморская лихорадка и болезнь Бехтерева.
Ключевые слова: семейная средиземноморская лихорадка, болезнь Бехтерева, воспаление.
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INTRODUCTION
Complaints of joint disease is seen in 70%-75% of Familial Mediterranean fever (FMF) patients. These joints are usually lower limbs such as hips or knees. Insomuch that arthritis may be the first sign in one-third of FMF patients. The arthritis of FMF usually presents as recurrent and self limited acute attacks. Protracted arthritis is seen only 5% of patients. Increased risk of sacroiliitis in FMF patients is controversial.
FMF is an autosomal-recessive disease generally seen in Jewish, Armenian, Turkish, and Middle Eastern Arab
populations. The disease is caused by MEFV gene mutation which is located on the short arm of chromosome 16 and encodes an immunoregulatory protein known as pyrin or marenostrin. This gene may change the inflammatory response leading to inflammatory diseases. Thus it may contribute to the development of AS. The M694V polymorphism of the MEFV gene is more likely to be associated with arthritis and may be with sacroiliitis [1]. We presented a 46 year old man who diagnosed FMF and ankylosing spondylitis (AS) simultaneously.
CASE
46 year old man who is under colchicine threatment for 20 years due to FMF, was also having complaints related to backache, showing characteristic of inflamatory diseases in last 3 years. Patient was also suffering from peripheral
joint swelling. Regarding the inflamatory characterised backache sacroiliac magnetic rezonans imaging (MRI) planned. Findings related bilateral sacroiliitis were seen in MRI results (figure 1).
Patient is found HLA B27 positive in adition to heterozygous M694V positive results. In the light of findings patient was considered as having both FMF and
DISCUSSION
FMF is an autosomal-recessive disease characterised by typical acute attacks of serositis, arthritis or skin rash. Joint disease occurs in approximately 75% of patients and generally resolves within a few days without damage. Spondyloarthropathy (SpA), usually associated with a lack of HLA-B27, is seen rarely in FMF patients. In FMF patients, SpA usually involves unilateral and bilateral sacroiliitis with inflammatory low back pain and recurrent enthesitis
CONCLUSION
The exact relationship between FMF and AS remains obscure. However, SpA was proposed as one of the causes of the possible joint involvement in FMF and the pathogenetic relationship between these two conditions remains unknown. Recent data suggest that pro-inflammatory cytokines including IL-10, IL-12, IL-17, IL-18 contribute to the cytokine network in the inflammatory cascade of FMF and indicates a relationship between chemokines and
AS. Indomethacin and sulfasalazine were added to ongoing colchicine regime.
[2]. Some cases with coincidence of AS and FMF who had a positive HLA-B27 were presented as case reports in the literature. They were diagnosed as coincidence of FMF and AS [3]. As the frequency of M694V is higher among FMF patients with radiographic sacroiliitis than those without this disorder, MEFV gene variations may be the potential pathogenic link between FMF and AS.
subclinical inflammation. A sustained inflammatory reaction is observed in the disease course, and cytokine levels such as IL-1, IL-6 and TNF-alpha have been shown to increase during and between the attacks. As these cytokines are also known to contribute to the pathogenesis of AS, a possible connection between these two diseases can be considered to exist. There is no clear data on the relationship between the attacks of peripheral arthritis and SpA, and the cytokine
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network, as well as the gene expression profile in patients with FMF.
We believe that due to highened risk of sacroiliitis
among FMF patients, ankylosing spondylitis should not be overlooked among FMF patients and inflamatory back pain should be investigated thoroughly.
REFERENCES
1. Kastner DL. Intermittent and Periodic Arthritic Syndromes In: Koopman WJ. (ed) Arthritis and Allied Conditions. Philadelphia: Lippincott Williams & Wilkins 2001; 1400-13.
2. Langevitz P, Livneh A, Zemer D, et al. Seronegative spondiloarthropathy in familial Mediterranean fever. Semin Arthritis Rheum 1997; 27(2): 67-72.
3. Incel NA, Sarajoglu M, Erdem HR. Seronegative spondyloarthropathy of familial Mediterranean fever. Rheumatol Int 2003;23:41-3.
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