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Juvenile- and adult-onset systemic lupus erythematosus: a comparative study in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER)

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24; on behalf of the RELESSER Study Group of the Spanish Society of Rheumatology (SER) and the Study Group of Systemic Autoimmune Diseases of the SER (EAS-SER).

  1. Department of Rheumatology, Hospitalet-Sant Joan Despí Moisès Broggi University General Hospital, Hospitalet Llobregat, Spain. vtorrente@hsjdbcn.org
  2. Department of Rheumatology, Parc de Salut Mar-IMIM, Barcelona, Spain.
  3. Department of Rheumatology, Doctor Negrín University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain.
  4. Research Unit, Spanish Society of Rheumatology (SER), Madrid, Spain.
  5. Rheumatology Department, Gregorio Marañón University Hospital, Madrid, Spain.
  6. Rheumatology Department, Doce de Octubre University Hospital, Madrid, Spain.
  7. Rheumatology Department, Sierrallana Hospital, Torrelavega, Spain.
  8. Rheumatology Department, Germans Trías i Pujol University Hospital, Badalona, Spain.
  9. Rheumatology Unit, Clinica POVISA, Vigo, Spain.
  10. Department of Rheumatology, Navarra Hospital, Navarra, Pamplona, Spain.
  11. Department of Rheumatology, Príncipe de Asturias University Hospital, Madrid, Spain.
  12. Department of Rheumatology, Salamanca Clinic University Hospital, Salamanca, Spain.
  13. Department of Rheumatology Department, Complexo Hospitalario Universitario de Ourense, Spain.
  14. Department of Rheumatology, León Hospital, Spain.
  15. Department of Rheumatology, Marqués de Valdecilla University Hospital, Santander, Spain.
  16. Rheumatology Department, Puerta de Hierro-Majadahonda Hospital, Madrid, Spain.
  17. Department of Rheumatology, Basurto Hospital, Spain.
  18. Rheumatology Department, Hospital Insular of Gran Canaria, Gran Canaria, Las Palmas de Gran Canaria, Spain.
  19. Rheumatology Department, Tenerife Clinic Hospital, Santa Cruz de Tenerife, Spain.
  20. Department of Rheumatology, Virgen Macarena Hospital, Sevilla, Spain.
  21. Department of Rheumatology, Miguel Servet University Hospital, Zaragoza, Spain.
  22. Department of Rheumatology, Virgen de la Arrixaca University Hospital, Murcia, Spain.
  23. Department of Rheumatology, La Paz University Hospital, Madrid, Spain.
  24. Department of Rheumatology, Complexo Hospitalario Universitario de Vigo, Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain.

CER10275 Submission on line
2017 Vol.35, N°6 - PI 1047, PF 1055
Paediatric Rheumatology

Rheumatology Article
Rheumatology Article

 

Abstract

OBJECTIVES:
We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database.
METHODS:
Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years.
RESULTS:
We reviewed 3,428 aSLE patients (89.6% women) and 484 jSLE patients (89.8% girls), 93% Caucasian (both groups). Mean age at diagnosis was 38.1±14 and 16.6±6.3 years (p<0.001) and mean age at the end of follow-up was 48.8±14.3 and 31.5±30 years (p<0.001), respectively. jSLE showed significantly more clinical (including lymphadenopathy, fever, malar rash, mucosal ulcers, pericarditis, pleuritis, Raynaud’s phenomenon, lupus nephritis, recurrent nephritis, histologic nephritis changes, thrombocytopenia, haemolytic anaemia, thrombotic thrombocytopenic purpura, seizures, lupus headache and organic brain syndrome) and immunological (a-dsDNA and a-Sm antibodies, hypocomplementaemia) involvement than did aSLE, except for secondary Sjögren’s syndrome, a-Ro antibodies, fibromyalgia and osteoporosis. jSLE also showed more SLE family history, longer diagnosis delay, higher SLEDAI and Katz scores, but lower Charlson scores than aSLE. Several specific domains were more frequently involved in SLICC/ACR DI in jSLE. jSLE patients more frequently underwent all SLE-related treatment and procedures, as well as dialysis and kidney transplantations.
CONCLUSIONS:
jSLE shares many clinical and serological features with aSLE. However, jSLE patients typically manifested more activity, severity, cumulative damage in certain areas, than their aSLE counterparts.

PMID: 28628467 [PubMed]

Received: 24/01/2017 - Accepted : 03/04/2017 - In Press: 12/06/2017 - Published: 12/12/2017