Session Information
Date: Tuesday, October 23, 2018
Title: Systemic Lupus Erythematosus – Clinical Poster III: Treatment
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: GLADEL (Grupo Latino Americano De Estudio de Lupus) has previously shown that a 14.1% of its patients have relatives with an autoimmune disease (1). The present study was conducted to contrast the clinical features, damage accrual and survival of GLADEL patients with familial and sporadic SLE.
Methods: Familial SLE was defined as patients with a first degree relative with SLE (parents, siblings and offspring); these relatives were interviewed in person or by telephone and examined, if warranted. Patients with second and third degrees relatives with SLE were excluded from these analyses. All other patients were considered as having sporadic lupus. The sociodemographic, clinical, immunological, treatment characteristics plus disease activity, damage and mortality, were compared between the two patient groups using Wilcoxon test and Chi-square tests for continuous and categorical variables, respectively. Odds ratios (OR) and 95% confidence intervals (CI) were estimated. Time-to-damage and mortality were examined with Cox multivariable regressions [Hazard ratios (HR) and 95% CI] in which variables previously found to be associated were also included. Statistical significance was set at p ≤ 0.05; All analyses were performed using the SAS software, version 9.4 (SAS Institute, Cary, NC, USA).
Results: The cohort consisted of 1176 patients; 66 (5.6%) of them had familial and 1110, sporadic lupus. The majority of patients in both groups were women, of comparable age and all ethnic groups were represented. None of the sociodemographic variables examined were significantly associated with familial SLE. As shown in Table 1, there were some differences in terms of the clinical variables with discoid lupus [22.7% versus 12.9%; p = 0.024; OR 1.97 (1.08- 3.60)] and neurologic disorder [(45.4% versus 33.5%; p = 0.047; OR 1.65 (1.00- 2.73)] being significantly associated with familial SLE. In contrast, pericarditis was negatively associated with familial SLE [7.6% versus 19.1%; p = 0.01; OR 0.35 (0.14- 0.87)]. The SLEDAI and SDI were similar in both groups; in the Cox analyses, familial lupus was not significantly associated with damage accrual (HR=1.00; 0.53– 1.89; p = 0.99) or higher mortality (HR 1.01; 0.24– 4.31; p =0.99).
Conclusion: Familial SLE was associated with the presence of neurologic manifestations and of discoid lupus and with the absence of pericarditis, which has not been previously reported; however, no differences in hard endpoints such as disease activity, damage accrual and/or mortality were observed when compared with sporadic SLE.
1. Alarcon-Segovia D, et al. Familial aggregation of systemic lupus erythematosus, rheumatoid arthritis, and other autoimmune diseases in 1,177 lupus patients from the GLADEL cohort. Arthritis Rheum. 2005;52(4):1138-47.
|
Table 1. Characteristics of GLADEL patients associated with familial lupus versus all other GLADEL patients |
||||
|
Variable, (n, %) |
Familial lupus (n=66) |
Sporadic lupus |
p-value |
OR (CI95%) |
|
Malar rash |
44 (66.6%) |
714 (64.3%) |
0.699 |
|
|
Discoid lupus |
15 (22.7%) |
144 (12.9%) |
0.024 |
1.97 (1.08- 3.60) |
|
Photosensitivity |
38 (57.6%) |
666 (60.0%) |
0.696 |
|
|
Oral ulcers |
30 (45.4%) |
494 (44.5%) |
0.880 |
|
|
Arthritis |
53 (80.3%) |
920 (82.9%) |
0.590 |
|
|
Pleuritis |
16 (24.2%) |
288 (25.9%) |
0.759 |
|
|
Pericarditis |
5 (7.6%) |
212 (19.1%) |
0.019 |
0.35 (0.14- 0.87) |
|
Neurological disorder |
30 (45.4%) |
372 (33.5%) |
0.047 |
1.65 (1.00- 2.73) |
|
Renal disorder |
40 (60.6%) |
641 (57.7%) |
0.648 |
|
|
Haematological disorder |
52 (78.8%) |
873 (78.6%) |
0.979 |
|
|
Inmunological disorder (*135) |
50 (83.3%) |
795 (81.0%) |
0.659 |
|
|
ANA |
62 (93.9%) |
1061 (95.7%) |
0.531 |
|
|
Raynaud’s phenomenon |
21 (31.8%) |
347 (31.3%) |
0.924 |
|
|
Anti-dsDNA antibodies (*7) |
44 (66.7%) |
650 (58.5%) |
0.193 |
|
|
Anti-SSA/Ro (*110) |
15 (22.7%) |
274 (24.7%) |
0.719 |
|
|
Anti-SSB/La (*117) |
11 (16.7%) |
155 (13.9%) |
0,5401 |
|
|
Antiphospholipid antibodies (*74) |
26 (39.4%) |
383 (34.5%) |
0,4178 |
|
|
Hypocomplementemia (*30) |
39 (59.1%) |
642 (57,84%) |
0,8412 |
|
|
Mortality in the follow-up (n, %) |
4 (6.0%) |
73 (6,58%) |
0,8692 |
|
|
SLEDAI at cohort entry (*147) (median, IQR) |
9 (13.0) |
8 (11.0) |
0.692 |
|
|
SDI accrual (median, IQR) |
1.0 (2.0) |
1.0 (2.0) |
0.859 |
|
|
* Missing data; OR (odd ratio); IQR (Interquartile range); ANA (Anti-nuclear antibodies); SLEDAI (Systemic Lupus Erythematosus Disease Activity Index); SDI (SLICC/ACR Damage Index) |
||||
To cite this abstract in AMA style:
Quintana R, Pons-Estel GJ, Roberts K, Sacnun M, Serrano RM, Nieto R, Conti S, Gervasoni V, Catoggio LJ, Soriano ER, Scolnik M, Garcia M, Alvarellos A, Saurit V, Berbotto G, Sato E, Costallat L, Borba E, Bonfa E, Xavier RM, de Oliveira e Silva AC, Molina J, Iglesias-Gamarra A, Guibert-Toledano M, Reyes GA, Massardo L, Neira OJ, Cardiel MH, Barile L, Amigo MC, Silveira LH, Garcia de la Torre I, Acevedo-Vasquez E, Ugarte-Gil M, Alfaro-Lozano J, Segami I, Chacón-Díaz R, Esteva Spinetti MH, Gómez-Puerta JA, Alarcón GS, Pons-Estel BA. Clinical Features, Damage Accrual and Survival in Patients with Familial Systemic Lupus Erythematosus (SLE): Data from a Multiethnic, Multinational Latin American Lupus Cohort [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/clinical-features-damage-accrual-and-survival-in-patients-with-familial-systemic-lupus-erythematosus-sle-data-from-a-multiethnic-multinational-latin-american-lupus-cohort/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-damage-accrual-and-survival-in-patients-with-familial-systemic-lupus-erythematosus-sle-data-from-a-multiethnic-multinational-latin-american-lupus-cohort/