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Epidemiology and burden of systemic lupus erythematosus in a Southern European population: data from the community-based lupus registry of Crete, Greece
  1. Irini Gergianaki1,2,
  2. Antonis Fanouriakis3,
  3. Argyro Repa1,
  4. Michalis Tzanakakis1,
  5. Christina Adamichou1,
  6. Alexandra Pompieri1,
  7. Giorgis Spirou1,
  8. Antonios Bertsias4,
  9. Eleni Kabouraki1,
  10. Ioannis Tzanakis5,
  11. Leda Chatzi4,6,7,
  12. Prodromos Sidiropoulos1,2,
  13. Dimitrios T Boumpas2,3,8,9,10,
  14. George K Bertsias1,2
  1. 1Department of Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Heraklion, Greece
  2. 2Laboratory of Autoimmunity and Inflammation, Institute of Molecular Biology and Biotechnology, FORTH, Heraklion, Greece
  3. 34th Department of Medicine, Attikon University Hospital, National and Kapodistrian University of Athens Medical School, Athens, Greece
  4. 4Department of Social Medicine, University of Crete School of Medicine, Heraklion, Greece
  5. 5Department of Nephrology, General Hospital of Chania, Chania, Greece
  6. 6Department of Preventive Medicine, Keck School of Medicine, University of Southern California, Los Angeles, California, USA
  7. 7Department of Genetics and Cell Biology, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands
  8. 8Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece
  9. 9Medical School, University of Cyprus, Nicosia, Cyprus
  10. 10Biomedical Research Foundation of the Academy of Athens, Athens, Greece
  1. Correspondence to Dimitrios T Boumpas, Laboratory of Autoimmunity and Inflammation, Institute of Molecular Biology and Biotechnology, FORTH, Heraklion, Greece; boumpasd{at}


Objectives Several population-based studies on systemic lupus erythematosus (SLE) have been reported, yet community-based, individual-case validated, comprehensive reports are missing. We studied the SLE epidemiology and burden on the island of Crete during 1999–2013.

Methods Multisource case-finding included patients ≥15 years old. Cases were ascertained by the ACR 1997, SLICC 2012 criteria and rheumatologist diagnosis, and validated through synthesis of medical charts, administrative and patient-generated data.

Results Overall age-adjusted/sex-adjusted incidence was 7.4 (95% CI 6.8 to 7.9) per 100 000 persons/year, with stabilising trends in women but increasing in men, and average (±SD) age of diagnosis at 43 (±15) years. Adjusted and crude prevalence (December 2013) was 123.4 (113.9 to 132.9) and 143 (133 to 154)/105 (165/105 in urban vs 123/105 in rural regions, p<0.001), respectively. Age-adjusted/sex-adjusted nephritis incidence was 0.6 (0.4 to 0.8) with stable trends, whereas that of neuropsychiatric SLE was 0.5 (0.4 to 0.7) per 100 000 persons/year and increasing. Although half of prevalent cases had mild manifestations, 30.5% developed organ damage after 7.2 (±6.6) years of disease duration, with the neuropsychiatric domain most frequently afflicted, and 4.4% of patients with nephritis developed end-stage renal disease. The ACR 1997 and SLICC 2012 classification criteria showed high concordance (87%), yet physician-based diagnosis occurred earlier than criteria-based in about 20% of cases.

Conclusions By the use of a comprehensive methodology, we describe the full spectrum of SLE from the community to tertiary care, with almost half of the cases having mild disease, yet with significant damage accrual. SLE is not rare, affects predominantly middle-aged women and is increasingly recognised in men. Neuropsychiatric disease is an emerging frontier in lupus prevention and care.

  • epidemiology
  • incidence
  • prevalence
  • lupus nephritis
  • neuropsychiatric lupus

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  • DTB and GKB contributed equally.

  • Contributors IG conducted the study, performed research and face interviews; AF, AR, CA and AP examined and interviewed patients; MT and IT provided data on lupus nephritis patients; GS established and maintained the SLE database; EK arranged patients visits and delivered questionnaires; LC supervised the methodology; AB performed the statistical analysis; PS examined patients and supervised the study; GKB and DTB conceived the study and supervised the study; GKB and IG drafted the manuscript.

  • Funding This work was implemented by the Greek General Secretariat of Research and Technology ‘Aristeia’ action of the Operational Programme ‘Education and Lifelong Learning’, and was co-funded by the European Social Fund (ESF) and National Resources (Aristeia I 2344 to DTB).

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval University Hospital of Iraklio.

  • Provenance and peer review Not commissioned; externally peer reviewed.