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Mediterr J Rheumatol 2015; 26(2): 76-79
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A Multicenter Epidemiological Study of Giant Cell Arteritis, Lupus Nephritis, Anti-Phospholipid Antibody Syndrome, Systemic Sclerosis, & Behcet Disease in Greece
Petros P. Sfikakis
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(on behalf of the ERE Systemic Autoimmune Diseases Study Group)
Abstract

This paper has no abstract.

Keywords: Epidemiological study, vasculitis, lupus nephritis, Behcet’s Disease
Full Text

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 possible side effects from its use.
4. Lifestyle (smoking, alcohol) and patient comorbidities (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 erythematosus.

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

For a 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 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: Thestudy, 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-phospholipid 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
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  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.
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  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, Crook JE, Sadeghi-Abdollahi B, Schirmer M, et al. The International Criteria for Behçet’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.