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

Abstract

<jats:sec><jats:title>Objectives</jats:title><jats:p> This study aimed to compare the clinical features, damage accrual, and survival of patients with familial and sporadic systemic lupus erythematosus (SLE). </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> 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. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> 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). </jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p> 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. </jats:p></jats:sec>

Polyautoimmunity in systemic lupus erythematosus patients: New insights from a cross-sectional study

“…Studies in patients diagnosed with SLE and PolyA in different latitudes allowed us to evaluate the behavior in other populations, including Latin American patients. A multicenter cohort “Grupo Latino-Americano de estudio del Lupus” by its acronym in spanish (GLADEL) performed in Latin America, found significant differences between Latins, African Americans, mixed race or mestizos, and caucasian regarding kidney disease, pericarditis, polyadenopathies, severity of disease measured through disease scales, and different outcomes in patients with SLE [ 10 , 11 ].…”