Clinical features, damage accrual, and survival in patients with familial systemic lupus erythematosus: data from a multi-ethnic, multinational Latin American lupus cohort

Rosana Quintana, Guillermo J. Pons-Estel, Karen Roberts, Mónica Sacnún, Rosa Serrano, Romina Nieto, Silvana Conti, Viviana Gervasoni, Luis J. Catoggio, Enrique R. Soriano, Marina Scolnik, Mercedes A. García, Alejandro Alvarellos, Verónica Saurit, Guillermo A. Berbotto, Emilia I. Sato, Lilian T.Lavras Costallat, Eduardo Ferreira Borba Neto, Eloisa Bonfa, Ricardo M. XavierAna Carolina de Oliveira e Silva Montandon, José Fernando Molina-Restrepo, Antonio Iglesias-Gamarra, Marlene Guibert-Toledano, Gil Alberto Reyes-Llerena, Loreto Massardo, Oscar J. Neira, Mario H. Cardiel, Leonor A. Barile-Fabris, Mary Carmen Amigo, Luis H. Silveira, Ignacio García De La Torre, Eduardo M. Acevedo-Vásquez, Manuel F. Ugarte-Gil, José Luis Alfaro-Lozano, María Inés Segami, Rosa Chacón-Díaz, María H. Esteva-Spinetti, José A. Gomez-Puerta, Graciela S. Alarcón, Bernardo A. Pons-Estel

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1 Scopus citations


Objectives: This study aimed to compare the clinical features, damage accrual, and survival of patients with familial and sporadic systemic lupus erythematosus (SLE). Methods: A multi-ethnic, multinational Latin American SLE cohort was studied. Familial lupus was defined as patients with a first-degree SLE relative; these relatives were interviewed in person or by telephone. Clinical variables, disease activity, damage, and mortality were compared. Odds ratios (OR) and 95% confidence intervals (CI) were estimated. Hazard ratios (HR) were calculated using Cox proportional hazard adjusted for potential confounders for time to damage and mortality. Results: A total of 66 (5.6%) patients had familial lupus, and 1110 (94.4%) had sporadic lupus. Both groups were predominantly female, of comparable age, and of similar ethnic distribution. Discoid lupus (OR = 1.97; 95% CI 1.08–3.60) and neurologic disorder (OR = 1.65; 95% CI 1.00–2.73) were significantly associated with familial SLE; pericarditis was negatively associated (OR = 0.35; 95% CI 0.14–0.87). The SLE Disease Activity Index and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) were similar in both groups, although the neuropsychiatric (45.4% vs. 33.5%; p = 0.04) and musculoskeletal (6.1% vs. 1.9%; p = 0.02) domains of the SDI were more frequent in familial lupus. They were not retained in the Cox models (by domains). Familial lupus was not significantly associated with damage accrual (HR = 0.69; 95% CI 0.30–1.55) or mortality (HR = 1.23; 95% CI 0.26–4.81). Conclusion: Familial SLE is not characterized by a more severe form of disease than sporadic lupus. We also observed that familial SLE has a higher frequency of discoid lupus and neurologic manifestations and a lower frequency of pericarditis.

Original languageEnglish
Pages (from-to)1140-1145
Number of pages6
Issue number9
StatePublished - 1 Aug 2020


  • Systemic lupus erythematosus
  • damage accrual
  • disease activity
  • familial lupus
  • mortality
  • sporadic lupus


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