ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2659

Clinical Features, Damage Accrual and Survival in Patients with Familial Systemic Lupus Erythematosus (SLE): Data from a Multiethnic, Multinational Latin American Lupus Cohort

Rosana Quintana1,2, Guillermo J. Pons-Estel3,4, Karen Roberts1, Mónica Sacnun5, Rosa Maria Serrano2, Romina Nieto1, Silvana Conti1, Viviana Gervasoni1, Luis J. Catoggio6, Enrique R Soriano7, Marina Scolnik7, Mercedes Garcia8, Alejandro Alvarellos9, Verónica Saurit10, Guillermo Berbotto11, Emilia Sato12, Lilian Costallat13, Eduardo Borba14, Eloisa Bonfa15, Ricardo M. Xavier16, Ana Carolina de Oliveira e Silva17, JF Molina18, Antonio Iglesias-Gamarra19, Marlene Guibert-Toledano20, Gil A. Reyes20, Loreto Massardo21, Oscar J Neira22, Mario H Cardiel23, Leonor Barile24, Mary Carmen Amigo25, Luis H. Silveira26, Ignacio Garcia de la Torre27, Eduardo Acevedo-Vasquez28, Manuel Ugarte-Gil29, Jose Alfaro-Lozano30, Ines Segami31, Rosa Chacón-Díaz32, Maria H Esteva Spinetti33, Jose A Gómez-Puerta34, Graciela S. Alarcón35 and Bernardo A Pons-Estel2, 1Hospital Provincial de Rosario, Rosario, Argentina, 2Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Rosario, Argentina, 3Rheumatology, Hospital Provincial de Rosario, Rosario, Argentina, 4Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario. Argentina, Rosario, Argentina, 5Rheumatology Section, Hospital Provincial de Rosario, Rosario, Argentina, 6Rheumatology Unit, Internal Medicine Service. Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 7Rheumatology Unit, Internal Medicine Service. Hospital Italiano Buenos Aires. Argentina, Buenos Aires, Argentina, 8Rheumatology, HIGA General San Martin La Plata, La Plata, Argentina, 9Rheumatology, Rheumatology Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, 10Hospital Privado Centro Médico de Córdoba, Cordoba, Argentina, 11Hospital Escuela Eva Perón, Granadero Baigorria, Argentina, Granadero Baigorria, Argentina, 12Rheumatology Division, Universidade Federal de Sao Paulo, Sao Paulo, Brazil, 13Universidade Estadual da Campinas, Campinas, Brazil, 14Faculdade de Medicina, Hospital das Clínicas. Universidade de São Paulo, São Paulo, Brazil, 15Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil, 16Universidade Federal do Rio Grande do Sul Porto Alegre, Rio Grande do Sul, Brazil, 17Faculdade de Medicina, Universidade Federal de Goias, Goiania, Brazil, 18Reumalab, Medellín, Colombia, 19Clinical Development, Nordic Bioscience, Herlev, Denmark, 20Centro de Investigaciones Médico-Quirúrgicas (CIMEQ), Havana, Cuba, 21GLADEL, Santiago, Chile, 22Hospital del Salvador, Facultad de Medicina, Universidad de Chile, Santiago, Chile, 23Centro de Investigación Clínica de Morelia, Morelia, Mexico, 24Hospital Ángeles del Pedregal, Mexico, Mexico, 25Rheumatology, Centro Medico ABC, Mexico, Mexico, 26Rheumatology, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico City DF, Mexico, 27Hospital General de Occidente, Guadalajara, Mexico, 28Hospital Guillermo Almenara Irigoyen. EsSalud, Lima, Peru, 29Rheumatology, Universidad Científica del Sur, Lima, Peru, 30Rheumatology, Hospital Guillermo Almenara Irigoyen. EsSalud, Lima, Peru, 31Hospital Edgardo Rebagliati Martins. EsSalud, Lima, Peru, 32Servicio de Reumatología, Hospital Universitario de Caracas, Centro Nacional de Enfermedades Reumáticas, Caracas, Venezuela, 33Hospital Central de San Cristóbal, San Cristobal, Venezuela (Bolivarian Republic of), 34Rheumatology Department, Hospital Clínic, Barcelona, Spain, 35Universidad Peruana Cayetano Heredia, Lima, Peru

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords:

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
(n=1110)

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)

Disclosure: R. Quintana, None; G. J. Pons-Estel, None; K. Roberts, None; M. Sacnun, None; R. M. Serrano, None; R. Nieto, None; S. Conti, None; V. Gervasoni, None; L. J. Catoggio, None; E. R. Soriano, AbbVie, Bristol-Myers Squibb, GSK, Janssen, Novartis, Pfizer Inc, Roche, UCB, 2,AbbVie, Bristol-Myers Squibb, Eli Lilly, GSK, Janssen, Novartis, Pfizer Inc, Roche, Sanofi, UCB, 5,AbbVie, Bristol-Myers Squibb, Eli Lilly, Janssen, Novartis, Pfizer Inc, Roche, Sandoz, UCB, 8; M. Scolnik, None; M. Garcia, None; A. Alvarellos, None; V. Saurit, None; G. Berbotto, None; E. Sato, None; L. Costallat, None; E. Borba, None; E. Bonfa, Fundação de Amparo à Pesquisa do Estado de São Paulo, 2,Conselho Nacional de Desenvolvimento Científico e Tecnológico, 2; R. M. Xavier, None; A. C. de Oliveira e Silva, None; J. Molina, None; A. Iglesias-Gamarra, None; M. Guibert-Toledano, None; G. A. Reyes, None; L. Massardo, None; O. J. Neira, None; M. H. Cardiel, Gilead, Pfizer Inc, Roche, 2,Eli Lilly, Pfizer Inc, 5,Eli Lilly, Pfizer Inc, 8; L. Barile, None; M. C. Amigo, None; L. H. Silveira, None; I. Garcia de la Torre, None; E. Acevedo-Vasquez, None; M. Ugarte-Gil, None; J. Alfaro-Lozano, None; I. Segami, None; R. Chacón-Díaz, None; M. H. Esteva Spinetti, None; J. A. Gómez-Puerta, None; G. S. Alarcón, None; B. A. Pons-Estel, None.

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 .

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