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9XPHMATC^OTOYMENO ПPQTOKOЛЛO АПО THN EPE-EnEPE ERE-EPERE-SUPPORTED PROTOCOL
ПoAuкevтplкп иеАетп órpoupYÍaQ apxeiwv EAAqvuv ao9evúv pe YlYavтoкuттaplкп apтпpÍтlбa, veфpÍтlбa öuöтпмaтlкaú epu9пмaтшбouQ Лбгаи, avтlфшöфoAlПlбlкó oûvôpo|jo, оиотфо^кп öкAпpoбepмÍa & Nóoo Aбaмavтláбп-Behcet.
Пéтpoç П. ^фпкакпс, MD, PhD
(ек pépouç тпс Омабод EpYaoÍaQ Autoóvoowv Noo^cn^ тпд EPE-EПEPE)
mediterranean journal 26
of RHEUMATOLOGY 2
E Л Л H N I К H РЕУМАТОЛОпА 2015
Eioaywyn: О eпlпoЛоöpÓQ тwv ODO^a™^ auro-ávoowv voöПpáтшv öтov e^virá x^po eктlpáтоl öe 1.08%, eÍvai uфпAóтepoQ org YuvоÍкeQ ко1 au^ávei pe тпv пА^о.1 Пpóöфото OTOixeÍa 6bíxvouv о^поп тou eninoAaopoú ain'wv тwv voöПpáтwv котá тпv те-ЛeuтоÍо бeкопevтоeтÍо от^ Hvwpéveg ШАтаед тпд ApepiMÍQ, ni9avwg Aóyw ßeAтÍwöПQ отп бláYVwöП ко1 тпv avnpei'union тouQ.2 Ta öxeтlкá eпlбпploЛoYlкá бeбopéva anó тпv EЛЛáбо eÍvai nepiopiopéva. Ze 6úo peЛéтeQ anó тп ßopeloбuтlкп EЛЛáбо п етатиоп Yia pev тo öUöтпpотlкó epu9пpaтwбп Aúrco avípxeTO oe 1.90/100,000,3 Yia 6b тo öUöтпpотlкó öкЛпpóбeppо oe 1.10/100,000.4 Zuxvóтepп пА^о epфávlöПQ Yia то ODO^a^tó epu9пpотшбп Aúro ítov то 30-49 етп ко1 Yia то ODO^a^tó öкЛпpóбeppо то 45-64 етп, п бе ovoAoyío YUvоlкшv:оvбpшv iítov 7.4:1 ко1 8.9:1, avrï-OTOixa.3,4 ria тп vóoo Aбоpоvтláбп-Behcet п öUXvóтпто eктlpáтоl oe 400/10,0005, evw Yia тп YlYOvтoкuттоplкп оpтпpÍтlбо o eninoAaopóQ оvépxeтоl oe 0.035% ото oúvoAo тou пЛп9uöpoú ко1 oe 0.08% ото áTOpa ávw тwv 50 e^v.1 Ra тo avтlфwöфoAlПlбlкó oúv6popo 6bv unápxouv ení тои поpóvтoQ бeбopévо eninoAaopoú п етаптшопд anó тпv EAAáбa. Ta ODO^a^ra auTOávo-oa voonpaTO epфavÍZouv onpavтlкп пolKlAopopфÍa ong кAlVlкéQ тouQ екбпАшое^, xapaктпpÍZovтal anó éva eктevéQ фáopa öuwoöПpoтптwv, п бе 9epaneÍa touq napouoiáZei apкeтoúQ nepiopiopoúQ wq npog тo ró-öтoQ, тпv aoфáAela ко1 тпv aпoтeAeöpaтlкóтптá тпд.
Itôxoç тпд napoúoag peAéтпQ eÍvai п кaтaYpaфп: 1. тwv бпpoYpaфlкшv xapaктпplöтlкwv тwv ao9evúv pe YlYavтoкuттaplкп ap^pÍ^a, veфpÍтlбa тou ou-öтпpaтlкoú epu9пpaтшбouQ Aúкou, avтlфwöфoAl-
пlбlкó oúv6popo, oDO^a^tó öкAпpóбeppa ко1 vóoo Aбapavтláбп-Behcet o^v EAAáбa.
2. тwv кАмк^ xapaктпplöтlкшv ко1 тou avoöoAoYlкoú пpoфÍA auiúv тwv ao9evúv, pe öтóxo ^v e^aYWYÍ öupпepaöpáтwv Yia ni9avéQ кAlVlкoepYaoтnplaкéQ öUöxeтÍöelQ.
3. тпд eфappoZópevпQ 9epaneÍag (avoöoкaтaöтaA-тlкá, кopтlкoelбп, ßloAoYlкoÍ пapáYOVтeQ, оупщ-ктlкá), ко9шд ко1 ni9avúv napevepYeiúv anó тп xpпöп тпд.
4. тwv ошп9е^ Zw|q (ránviopa, aAкoóA) ко1 тwv öuvvoöПpoтптшv тwv ao9evúv (кapбlaYYelaкá vo-olpa™, nep^epiraí aYYelaкп vóooq, öaкxapшбпQ бlaßптпQ, xpóvia avaroet^i^ nveupovoná9eia, кaкoп9eleQ, oöтeoпópwöп).
5. тпд етатиопд ко1 тou eninoAaopoú Aoipw^ewv (io-YeveÍg ппaтÍтlбeQ).
6. тпд epßoAlaöTlкпQ гаАифпд évavтl roBi^v^^^ кou ко1 enox^ÍQ YPÍППQ o^v пepíптwöп тпд YiYa-vтoкuттaplкпQ ap^pn^ag.
yaikó Kai m¿6o6oi: H peAe^ 9a eÍvai бlaтpптlкoú oxe-6ioopoú, шАике^^к! Ze aimív 9a evтax9oúv ao9e-veÍg:
a. pe veфpÍтlбa тou Aúкou бlaYvwö9eÍöa eÍтe enÍ тп ßáöп тпд ßloфÍaQ veфpoú, eÍтe anó тпv aveúpeöп ёк-тшопд тпд veфplкпQ AelтoupYÍaQ nou 6bv eppпveúeтal anó áAAп ama, Aei«wpaTOupÍaQ>1gr/24hr, aipaTOupÍ-ag ко1 ^aív6pwv ото oúpa ao9evúv pe ODO^a^ra epu9пpaтшбп Aúкo.
ß. pe оют^т^ öкAпpóбeppa 6ioyvwo9év ßáöel тwv ^nripÍuv ACR (1980)6 П ßáöel тwv ^m^uv ACR-EULAR (2013).7
Aé^eiQ-KAekSiá: ПoAuкevтplкп peAéтп, OYYelÍтlбeQ, veфpÍтlбо TOU Aúкou, ODO^am! öкAпpuvöП, NÓOOQ Aбоpоvтláбп-Behcet.
nOAYKENTRIKH МЕЛЕТН AHMIOYPRIAX ARXEDN EAAHNQN AI0ENQN ME RRANTOKYTTAPIKH APTHPITIAA, ЫЕФР1Т1ААIYITHMATIKOY ERY0HMATQAOYIAYKOY, АЫТФОХФОАПАКО IYNAPOMO, IYITHMATIKH IKAHPOAEPM IA & NOIO AAAMANTIAAH-BEHCET A MULTiCENTER ERiDEMiOLOGiCAL STUDY OF GiANT CELL ARTERiTiS, LUPUS NEPHRiTiS, ANTi-PHOSPHOLiPiD ANTiBODY SYNDROME, SYSTEMiC SCLEROSiS, & BEHCET'S DiSEASE iN GREECE
Y. Me avтlфwoфoAlПl5lк6 ouvSpopo SiaYvwo9ev Pa-oei twv ava9ewpnMevwv Sie9vwv Kpnrpwv тои 2006 (International Consensus Classification Criteria for definite APS).8
S. Me v6oo ASaMavTiaSn-Behcet SiaYvwo9eioa Paoei twv ava9ewpnMevwv Sie9vwv Kpnrpwv (International Criteria for Behcet's Disease).9 e. Me YiYavTOKUTTapiKii apTnpmSa Paoei twv Kpnrpwv tou ACR (1990).10
Ze SiaoTHMa ev6Q ¿touq 9a кaтaYpaфoйv SiaSoxiKa 6Aoi oi ao9eveiQ Me tiq пpoavaфep9eíoeQ SiaYvwoeiQ nou enioKenTovTai Ta ouMMeTexovra KevTpa (naveni-oTHMiaKEQ kAivikeq TpiTC>Pa9Miwv voooKoMeiwv). npiv Tnv evTa^n oTn MeAeTn, Ka9e ao9ev|Q 9a npenei va uпoYpaфel evTuno пAnpoфopnм¿vnQ ouYKaTa9eonQ. H avwvuMia twv ao9evwv nou ouMMeTexouv e£aoфa-AiZeTai Me Tnv KaTaxwpnon twv SeSoMevwv touq Me Ta apxiKa tou ovoмaтeпwvuмoй touq, Ta onoia oe ouv-SuaoM6 Me to ¿toq YevvnonQ Kai tov kwSik6 tou ке-vTpou пapaкoЛoй9пoпQ 9a oxnMaTiZouv ¿va MovaSiK6 KwSiK6 ava ao9ev|. H кaтaYpaфп twv SeSoMevwv 9a YiveTai eiTe Me тг| ouM^ipwon ev6Q пpoтuпwм¿vou evrtrau пou 9a araoTeAAeTai oth YPaMMaTeia thq MeAeTHQ, eiTe Me arau9eiaQ eioaYWYi twv oToixeiwv oe nAeKTpoviKii Paon SeSoMevwv, пou 9a eivai пpo-oPaoiMH Meow ev6Q aoфaЛoйQ 5la5lктuaкoй т6пou. ZTa KevTpa tou 9a ouMMeTaoxouv, 9a aпooтaЛoйv avaAuTiKEQ oSnYieQ oxeTiKa Me тп ouM^ipwon twv S^opwv пe5íwv TnQ ф6pмaQ кaтaYpaфпQ, woTe va eraTeux9ei KaTa to SuvaT6v oMoioYeveia oTn ouAAoy! twv SeSoMevwv. MeTa пapo5o TpieTiaQ uпapxel пр6-pЛeфn va eпavaЛnф9eí n кaтaYpaфп twv SeSoMevwv twv ao9evwv пpoкelм¿vou va e£ax9oйv Kai пpooптl-Ka ouM^paoMaTa aп6 тп MeAeTn. ria Tnv avaAuon twv oToixeiwv 9a eфapмoo9oйv Me9oSoi пeplYpaфl-K|Q oTaTioTlKIQ.
Проабокымеуа апотеЛеамата: H MeAeTn пou 9a xpnMaToSoTn9ei a™ Tnv EAAnviKi PeuMaToAoYiKi ETaipeia oкoпeйel Yia прштп фopa oTnv EAAaSa va ouYKevTpwoei SeSoMeva aп6 ¿va пoAй MeYaAo SeiYMa ao9evwv Me veфpíтl5a tou Aйкou, ouoTnMaTiK6 oKAn-p6SepMa, avтlфwoфoAlПl5lк6 oйv5poмo, YiYavToKuT-TapiK| apTnpiTiSa Kai v6oo ASaMavTiaSn-Behcet, Me Tnv пpooптlкп va SieuKpivioei Tnv eraSnMioAoYia Kai tiq KAiviKeQ e^avoeiQ twv voonMaTwv auTwv otov eAAnviK6 xwpo, va кaтaYpaфel tiq eфapмoZ6мeveQ 9epaпeíeQ Kai to Pa9M6 ouмм6pфwoпQ touq Me tiq Sie9veiQ Kai eAAnviKeQ oSnYieQ Kai va пeplYpaфel tiq ouxv6TepeQ era^o^ Kai ouvvoonp6TnTeQ twv ou-oTnMaTiKwv auToavoowv voonMaTwv.
BIBAIOrPAQIA
1 Andrianakos A, Trontzas P, Christoyannis F, et al. Prevalence of rheumatic diseases in Greece: A cross-sectional population based epidemiological study. The ESORDIG Study. J Rheumatol 2003; 30: 1589-601.
2 Peschken C, Hitchon C. Rising prevalence of systemic autoimmune rheumatic disease: increased awareness, increased disease or increased survival? Arthritis Res Ther 2012; 14: A20.
3 Alamanos Y, Voulgari PV, Siozos C, et al. Epidemiology of systemic lupus erythematosus in northwest Greece 1982-2001. J Rheumatol 2003; 30: 731-5.
4 Alamanos Y, Tsifetaki N, Voulgari PV, et al. Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. Semin Arthritis Rheum 2005; 34: 714-20.
5 Konstantopoulos K, Kanta E, Papadopoulos V, et al. Mediterranean fever gene mutations in greek patients with Behcet's disease. West Indian Med J 2012; 61: 28-31.
6 Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980; 23: 581-90.
7 Van Den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 2013; 65: 2737-47.
8 Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4: 295-306.
9 Davatchi F, Assaad-Khalil S, Calamia KT, HYPERLINK "http:// www.ncbi.nlm.nih.gov/pubmed/?term=Crook%20JE" Crook JE, Sadeghi-Abdollahi B, HYPERLINK "http://www.ncbi. nlm.nih.gov/pubmed/?term=Schirmer%20M" Schirmer M, et al. The International Criteria for Behget's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatology Venereol 2014; 28: 338-47.
10 Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33: 1122-8.
A multicenter epidemiological study of giant cell arteritis, lupus nephritis, anti-phospholipid antibody syndrome, systemic sclerosis, & Behcet's disease in Greece.
Petros P. Sfikakis, MD, PhD, on behalf of the ERE Systemic Autoimmune Diseases Study Group)
mediterranean journal 26
of RHEUMATOLOGY 2
EAAHNIKH PEYMATOAOriA 2015
Introduction: Prevalence of systemic autoimmune diseases in Greece has been estimated as high as 1.08%. It is higher among women and rises with age.1 Recent evidence from a north American study shows an increase in the prevalence of these conditions during the last fifteen years, possibly attributable to an improvement in their diagnosis and treatment2. The relevant epidemiologic data from Greece is limited. In two studies from the northwestern part of the country, the incidence of systemic lupus erythematosus and systemic sclerosis was 1.90/100,0003 and 1.10/100,000,4 respectively. The peak age of onset was 30-49 years for systemic lupus erythematosus and 45-64 years for systemic sclerosis, while the female to male ratio was 7.4:1 and 8.9:1, respectively.3,4 The incidence of Behcet's disease is estimated to be nearly 400/10,0005. As far as giant cell arteritis is concerned, the prevalence amounts to
0.035. in the total population and 0.08% in the population above 50 years of age.1 There are no epidemiologic data from Greece regarding antiphospholipid antibody syndrome. Systemic autoimmune diseases have a highly variable clinical presentation, they are characterized by a wide spectrum of comorbidities and their treatment is fraught with several limitations in terms of cost, safety and efficacy.
Aim of the present study is to record:
1. The demographics of patients with giant cell arteritis, lupus nephritis, anti-phospholipid antibody syndrome, systemic sclerosis and Behcet's disease in Greece.
2. The clinical characteristics and the serological profile of these patients with the intention of revealing possible clinico-laboratory correlations
3. The implemented treatment (immunosuppressives, corticosteroids, biologics, anticoagulants) and pos-
sible side effects from its use.
4. Lifestyle (smoking, alcohol) and patient comorbid-ities (cardiovascular diseases, peripheral vascular disease, diabetes mellitus, chronic obstructive pulmonary disease, malignancies, osteoporosis).
5. Incidence and prevalence of infections (viral hepatitis B and C).
6. Vaccination coverage against pneumococcus and influenza virus in patients with giant cell arteritis.
Materials and Methods: The study has a cross-sectional, multicenter design and will include patients with:
a. Lupus nephritis diagnosed either by renal biopsy or by the presence of renal function decline (not attributable to any other cause), proteinuria>1gr/24hr, hematuria and casts in the urine of patients with systemic lupus ery-thematosus.
b. Systemic sclerosis according to the ACR (1980)6 or the ACR-EULAR (2013)7 criteria.
c. Antiphospholipid antibody syndrome, based on the revised International Consensus Classification Criteria for Definite APS (2006).8
d. Behcet's disease according to the revised International Criteria for Behcet's Disease9
e. Giant cell arteritis on the basis of the 1990 ACR criteria.10
Fora period of one year, all patients seen in the participating centers (tertiary care university clinics) bearing one of the above diagnoses will be sequentially recorded. All participants are required to sign an informed consent form prior to enrollment. Patient anonymity will be ensured by replacing all patient identifiers by a unique code produced by the combination of patient initials, year of birth and center code. Patient data can be filled in using a simple registry form, available both in print and
Keywords: Epidemiological study, vasculitis, lupus nephritis, Behcet's Disease.
nOAYKENTRIKH MEAETH AHMIOYPRIAX APXEIQN EAAHNQN AX0ENQN ME RRANTOKYTTAPIKH APTHPITIAA, NEOPITIAAIYXTHMATIKOY EPY0HMATQAOYI AYKOY, ANTIOQXOOAOAIKO XYNAPOMO, XYXTHMATIKH IKAHPOAEPM IA & NOIO AAAMANTIAAH-BEHCET A MULTiCENTER ERiDEMiOLOGiCAL STUDY OF GiANT CELL ARTERiTiS, LUPUS NEPHRiTiS, ANTi-PHOSPHOLiPiD ANTiBODY SYNDROME, SYSTEMiC SCLEROSiS, & BEHCET'S DiSEASE iN GREECE
through a secure website. Detailed instructions on how to complete the forms will be sent to all participating centers in order to achieve the maximum level of homogeneity in data collection. Provision has been made for a repeat data recording after 3 years, in order to allow prospective follow up of the patients. Descriptive statistics methods will be applied for data analysis.
Anticipated benefits: The study, which will be funded by the Hellenic Rheumatology Society, for the first time aims to record data from a large number of Greek patients with giant cell arteritis, lupus nephritis, anti-phos-pholipid antibody syndrome, systemic sclerosis and Behcet's disease in the purpose of clarifying the epidemiology, clinical features, complications, comorbidities and treatment of these diseases.
REFERENCES
1 Andrianakos A, Trontzas P, Christoyannis F, Kaskani E, Nikolia Z, Tavaniotou E, et al. Prevalence of rheumatic diseases in Greece: A cross-sectional population based epidemiological study. The ESORDIG Study. J Rheumatol 2003; 30: 1589-601.
2 Peschken C, Hitchon C. Rising prevalence of systemic autoimmune rheumatic disease: increased awareness, increased disease or increased survival? Arthritis Res Ther 2012; 14: A20.
3 Alamanos Y, Voulgari PV, Siozos C, et al. Epidemiology of systemic lupus erythematosus in northwest Greece 1982-2001. J Rheumatol 2003; 30: 731-5.
4 Alamanos Y, Tsifetaki N, Voulgari PV, Siozos C, Tsamandouraki K, Alexiou GA, et al. Epidemiology of systemic sclerosis in northwest Greece 1981 to 2002. Semin Arthritis Rheum 2005; 34: 714-20.
5 Konstantopoulos K, Kanta E, Papadopoulos V, Kaklamanis P, Hatzinikolaou M, Kalotychou V, et al. Mediterranean fever gene mutations in greek patients with Behcet's disease. West Indian Med J 2012; 61: 28-31.
6 Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980; 23: 581-90.
7 Van Den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative. Arthritis Rheum 2013; 65: 2737-47.
8 Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cerve-ra R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4: 295-306.
9 Davatchi F, Assaad-Khalil S, Calamia KT, Crook JE, Sadeghi-Abdollahi B, HYPERLINK "http://www.ncbi.nlm.nih.gov/ pubmed/?term=Schirmer%20M" Schirmer M, et al. The International Criteria for Behget's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatology Venereol 2014; 28: 338-47.
10 Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Ca-labrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33: 1122-8.
