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

Quintana R.; Pons-Estel G.J.; Roberts K.; Sacnún M.; Serrano R.; Nieto R.; Conti S.; Gervasoni V.; Catoggio L.J.; Soriano E.R.; Scolnik M.; García M.A.; Alvarellos A.; Saurit V.; Berbotto G.A.; Sato E.I.; Costallat L.T.L.; Neto E.F.B.; Bonfa E.; Xavier R.M.; de Oliveira e Silva Montandon A.C.; Molina-Restrepo J.F.; Iglesias-Gamarra A.; Guibert-Toledano M.; Reyes-Llerena G.A.; Massardo L.; Neira O.J.; Cardiel M.H.; Barile-Fabris L.A.; Amigo M.-C.; Silveira L.H.; Torre I.G.D.L.; Acevedo-Vásquez E.M.; Ugarte-Gil M.F.; Alfaro-Lozano J.L.; Segami M.I.; Chacón-Díaz R.; Esteva-Spinetti M.H.; Gomez-Puerta J.A.; Alarcón G.S.; Pons-Estel B.A. · Lupus (2020) · DOI: 10.1177/0961203320935184

Resumen

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. © The Author(s) 2020.

Instituciones: Universidad Peruana Cayetano Heredia; Universidad Científica del Sur