Clinical Features and Prognosis of Late-onset Systemic Lupus Erythematosus: Results from the 1000 Faces of Lupus Study

2009 ◽  
Vol 37 (1) ◽  
pp. 38-44 ◽  
Author(s):  
SHELIZA LALANI ◽  
JANET POPE ◽  
FAYE de LEON ◽  
CHRISTINE PESCHKEN
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Control Group ◽  
Systemic Lupus ◽  
Damage Accrual ◽  
Onset Group ◽  
Onset Systemic Lupus Erythematosus

Objective.There is controversy whether older-onset systemic lupus erythematosus (SLE) is associated with a different, more benign disease course than in younger-onset SLE. Our objective was to characterize the clinical features and prognosis of late-onset SLE in a large, multicenter cohort.Methods.We studied adult-onset lupus in the 1000 Canadian Faces of Lupus cohort (n = 1528) of whom 10.5% had onset at age ≥ 50 years versus a control group with onset at < 50 years.Results.Disease duration was different in early- and late-onset groups (15 yrs in early vs 9.3 yrs in late; p < 0.001). Caucasians were represented more in the later-onset SLE group (55.6% vs 74.5%), while Asians and Blacks were more prevalent in the younger group. Younger-onset SLE subjects fulfilled more American College of Rheumatology criteria for SLE (< 50 yrs: 5.98 ± 1.68; ≥ 50 yrs: 5.24 ± 1.44; p < 0.0001). Despite an equal prevalence of anti-dsDNA, the younger-onset group more often had positive anti-Smith autoantibody, ribonucleoprotein, and hypocomplementemia, and more nephritis, rash, and cytopenias than the older-onset group. However, disease activity and damage accrual were higher in the older-onset group. The older patients received less prednisone and immunosuppressives (current and ever-use). As expected, comorbidity was higher in the older-onset SLE group.Conclusion.This study suggests that older age-onset SLE is not benign. There may be an interaction between lupus and age in which, although there is less lupus nephritis in the elderly, more disease activity and damage are present.

scholarly journals THU0283 DISTINCT CLINICAL FEATURES OF LATE–ONSET SYSTEMIC LUPUS ERYTHEMATOSUS AMONG MALAYSIAN MULTI-ETHNIC COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 368.2-369
Author(s):  
S. S. Shaharir ◽  
M. S. Mohamed Said ◽  
S. Rajalingham ◽  
H. Mahadzir ◽  
R. Mustafar ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Age Group ◽  
Systemic Lupus ◽  
Disease Characteristics ◽  
Younger Age ◽  
Damage Accrual ◽  
Onset Group ◽  
Onset Systemic Lupus Erythematosus

Background:Systemic Lupus Erythematosus (SLE) commonly affects young women in their reproductive age group. However, there is an increase prevalence of late-onset SLE, parallel to the higher life expectancies among general populations worldwide. It has been reported that up to 25% SLE populations have a later onset of disease and their disease expression and course may be different.Objectives:To determine the clinical features and outcomes of late-onset SLE patients in a multi-ethnic Malaysian cohort.Methods:Medical records of SLE patients who attended regular follow-up clinics in Universiti Kebangsaan Malaysia Medical Centre (UKMMC) from 2011 until June 2019 were reviewed. Late-onset SLE was defined as the onset of SLE symptoms or diagnosis after the age of 50 years old. Information on their socio-demographics and disease characteristics were obtained from the clinical records. Disease damage was assessed using the SLICC/ACR (Systemic Lupus International Collaborating Clinics/American College of Rheumatology) Damage Index (SDI) scores. The disease characteristics and autoantibody profiles were compared between late-onset and younger onset patients. Damage accrual at disease onset and at 5 years was obtained and compared between the two groups.Results:A total of 429 patients were included and majority of them were Malays (n= 225, 52.4%) followed by Chinese (n=180, 42), Indian (n=21, 4.9%) and others (n=3,0.7%). This multi-ethnic SLE cohort was consisted of predominantyly female patients (n=372,86.7%) with disease duration of 9.9 years ± 6.8 years. A total of 13.8% (n=59) had late onset SLE with mean onset of disease at 58.1 ± 6.3 years while younger group was 27.2 ± 9.4 years. The commonest system involvement among the late-onset group was haematological manifestation (69.5%).Compared to the younger-onset SLE, late-onset SLE occurred significantly higher among the Chinese (66.1%) as compared to Malay (32.3%), Indians and other ethnics (1.7%), p<0.01. Patients with late-onset SLE also had significantly less musculoskeletal (37.3% vs 62.4%) and renal (23.7% vs 71.1%), p<0.001 and tend to have less muco-cutanoues manifestations (28.8 vs 42.4%, p=0.06). Meanwhile, pulmonary involvement was more common among the late onset SLE patients (11.9% vs 0.8%, p<0.001). Extractable nuclear antigen (ENA) results were available in 197 patients and patients with late-onset SLE had significantly higher rate of anti-RO positive (63% vs 3.9%), p=0.01. Otherwise, no significant difference in the other autoantibodies expressions including anti-La, anti-Sm, anti-RNP, anti-ribosomal P and anti-phospholipid antibodies. Patients with late-onset SLE tend to have more damage accrual at 5 years as compared to the younger age group (p=0.07). The mortality in the late onset group was 13.6% (n=8) as compared to 2.7% (n=10) in the younger age group, p=0.01. Majority of the cause of death in the later onset SLE was infection (87.5%) while in the younger age group was infection and active disease (90%).Conclusion:Late onset SLE occurs more commonly among Chinese ethnics in Malaysia and Malaysian SLE patients with late onset of the disease have distinct clinical manifestations. Damage accrual at 5 years tend to be higher in the late-onset group and the mortality is significantly higher with the major cause of death is infection. The different disease expression and outcome in late onset SLE suggest different factors in influencing the disease course and hence further studies including their genetic profiles are warranted.References:[1]Paula I. Burgos; Graciela S. Alarcón. Late-onset Lupus: Facts and Fiction. Future Rheumatol. 2008;3(4):351-356.[2]S Stefanidou, C Gerodimos, A Benos et al. Clinical expression and course in patients with late onset systemic lupus erythematosus. Hippokratia. 2013; 17(2): 153–156.Acknowledgments:This research was supported by the “Fundamental Research Grant Scheme (FRGS/1/2018/SKK02/UKM/03/1)” by Ministry of Education MalaysiaDisclosure of Interests:Syahrul Sazliyana Shaharir: None declared, Mohd Shahrir Mohamed Said: None declared, Sakthiswary Rajalingham Speakers bureau: Pfizer (500USD), Hazlina Mahadzir: None declared, Ruslinda Mustafar: None declared, Asrul Abdul Wahab: None declared

Differences in Clinical Features and Mortality between Childhood-onset and Adult-onset Systemic Lupus Erythematosus: A Prospective Single-center Study

2016 ◽  
Vol 43 (8) ◽  
pp. 1490-1497 ◽  
Author(s):  
Young Bin Joo ◽  
So-Yeon Park ◽  
Soyoung Won ◽  
Sang-Cheol Bae
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Hemolytic Anemia ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Adult Onset ◽  
Single Center ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

Objective.To compare clinical features and mortality between childhood-onset systemic lupus erythematosus (cSLE) and adult-onset SLE (aSLE) in a prospective single-center cohort.Methods.A total of 1112 patients with SLE (133 cSLE and 979 aSLE) were enrolled and followed from 1998 to 2012. The 2 groups were compared regarding American College of Rheumatology (ACR) classification criteria for SLE, autoantibodies, disease activity measured by the Adjusted Mean SLE Disease Activity Index (AMS), damage measured by the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI), and medication. The standardized mortality ratio (SMR) was calculated. Predictors of mortality in SLE were evaluated using Cox proportional hazard models.Results.After a mean followup of 7.6 years, patients with cSLE had a higher number of cumulative ACR criteria and a higher AMS (p < 0.001 each), but there was no difference in SDI (p = 0.797). Immunosuppressants were used more frequently by patients with cSLE (p < 0.001). The SMR of cSLE was 18.8 (95% CI 8.6–35.6), significantly higher than that of aSLE (2.9, 95% CI 2.1–3.9). We found cSLE to be an independent predictor of mortality (HR 3.6, p = 0.008). Moreover, presence of hemolytic anemia (7.2, p = 0.034) and antiphospholipid antibody (aPL; 3.8, p = 0.041) increased the magnitude of risk of early mortality more in the patients with cSLE than in those with aSLE.Conclusion.The clinical course of cSLE as measured by number of clinical manifestations and disease activity is worse than that of aSLE. Also, cSLE patients with hemolytic anemia and aPL are at greater risk of death than patients with aSLE who have those features.

AB0636 Late-onset systemic lupus erythematosus: Clinical features, course and prognosis

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 675.4-675
Author(s):  
A. Tomic Lucic ◽  
R. Petrovic ◽  
M. Radak Perovic ◽  
D. Milovanovic ◽  
M. Veselinovic ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

scholarly journals Th cytokine profile in childhood-onset systemic lupus erythematosus

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wei Quan ◽  
Jingnan An ◽  
Gang Li ◽  
Guanghui Qian ◽  
Meifang Jin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Healthy Control Group ◽  
Control Group ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Healthy Control ◽  
Ifn Γ ◽  
Onset Systemic Lupus Erythematosus

Abstract Background Childhood-onset systemic lupus erythematosus (cSLE) is a kind of chronic inflammatory disease characterized by a highly abnormal immune system. This study aimed to detect the serum levels of Th (T helper) cytokines (IL-2, IL-4, IL-5, IL-6, IL-9, IL-10, IL-13, IL-17A, IL-17F, IL-21, IL-22, IFN-γ and TNF-α) in cSLE and healthy controls, and then to elucidate their association with clinical manifestations, disease activity and laboratory parameters. In order to provide clues for early diagnosis and timely intervention treatment of cSLE patients. Methods A total of 33 children with cSLE and 30 healthy children were enrolled in this study. Children in the cSLE group were classified into the inactive or active cSLE group according to their SLE disease activity index 2000 (SLEDAI-2 K) score. Th cytokine profiles in the peripheral blood were detected and analysed. Results Levels of IL-2, IL-10 and IL-21 in the cSLE group were significantly higher than those in the healthy control group (P < 0.05, P < 0.01 and P < 0.01, respectively). Expression of IL-2, IL-10 and IL-21 in the active cSLE group was significantly higher than that in the healthy control group (P < 0.05, P < 0.01 and P < 0.05, respectively), but that of IL-22 expression was markedly lower in the active cSLE group than in the healthy control group (P < 0.001). IL-21 in the inactive SLE group was significantly higher than that in the healthy control group (P < 0.05), and levels of IL-2 and IL-10 in the active cSLE group were significantly higher than those in the inactive cSLE group (P < 0.01 and P < 0.05). In-depth analysis showed that after excluding age, gender and drug interference, the levels of IL-2 (P < 0.05), IL-6 (P < 0.05) and IL-10 (P < 0.05) were still positively correlated with SLEDAI-2 K scores. However, the levels of IL-6 (P < 0.05) and IFN- γ (P < 0.05) were still negatively correlated with CD4+/CD8+, and the concentration of IL-6 (P < 0.05) was still positively correlated with the occurrence of nephritis. Conclusion This study provides a theoretical basis for the discovery of effective methods to regulate imbalance in T lymphocyte subsets in cSLE, which may lead to new approaches for the diagnosis of cSLE.

scholarly journals POS0725 CLINICAL AND IMMUNOLOGICAL CHARACTERISTICS OF PATIENTS WITH JUVENILE-, ADULT- AND LATE-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 612.2-613
Author(s):  
O. Iaremenko ◽  
D. Koliadenko ◽  
I. Matiyashchuk
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Disease Onset ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Autoantibody Profile ◽  
Onset Systemic Lupus Erythematosus

Background:Systemic lupus erythematosus (SLE) predominantly develops in women of child-bearing age. However, nearly 20% of cases present during childhood, generally after puberty (juvenile-onset SLE, JSLE). On the other hand, 10-20% of patients develop SLE after the age of 45-50 years (late-onset SLE, LSLE) [1]. It is known that age at disease onset can influence the clinical presentation and course of SLE, but the findings are not always consistent across the studies [2].Objectives:The aim of this study was to evaluate the spectrum of clinical manifestations and autoantibody profile in patients with SLE in the central region of Ukraine regarding age at onset.Methods:The study included 258 SLE patients before starting an adequate therapy, comprising 225 females (87.2%) and 33 males (12.8%). The median age at SLE onset was 28 (20-39) years. The patients were classified into 3 groups: I – age at SLE onset ≤18 years (JSLE; n=52; 20.2%), II – SLE onset at age 19-44 years (adult-onset SLE, ASLE; n=161; 62.4%), III – age at disease onset ≥45 years (LSLE; n=45; 17.4%). The clinical and demographic data, SLE Disease Activity Index (SLEDAI), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and autoantibody profile were analyzed. Quantitative and categorical data were compared using Kruskal-Wallis test and chi-square test, respectively.Results:There was a difference in prevalence of malar rash between the groups (p=0.022): it was more common in JSLE (40.4%) and ASLE (34.4%) than in LSLE patients (15.6%; p=0.04 and 0.05, respectively). Similar distribution was found for renal involvement: JSLE and ASLE patients presented higher rates of nephritis (55.8% and 49.4%, respectively) than LSLE patients (23.8%; p=0.012 and 0.014, respectively). But the groups did not differ significantly with regard to nephrotic syndrome (p=0.224). ASLE was associated with more frequent alopecia (38.8%) comparing with JSLE (19.2%; p=0.04). Moreover, ASLE patients also had the highest frequency of lymphadenopathy (56.3%) whereas in LSLE it was observed only in 25.0% of patients (p=0.001). Serositis was more common in LSLE (54.5%) and ASLE (43.8%) than in JSLE (23.1%; p=0.011 and 0.034, respectively). Although secondary Sjögren’s syndrome was more frequently observed in ASLE (7.6%) and LSLE (7.3%) than in JSLE (0.0%), the difference did not achieve statistical significance (p=0.157). Also, no differences were observed in the occurence of arthritis, pulmonary and neurological manifestations, constitutional symptoms, SLEDAI score among the groups. Median CRP level in LSLE was significantly higher (14.0 (1.1-46.4) mg/L) than in JSLE (0.7 (0.0-12.0) mg/L) (p<0.05). But all groups did not differ significantly with regard to ESR levels. When differences in antinuclear antibodies were analyzed, we disclosed that the frequency of anti-dsDNA positive results was significantly higher in JSLE (68.6%) and ASLE (70.1%) patients when compared with that found in LSLE patients (31.3%) (p=0.016 and 0.001, respectively). There were no significant differences between groups with regard to positivity for other antibodies (anti-Sm, -Ro, -La, -RNP, antiphospholipid antibodies).Conclusion:JSLE and ASLE patients are more likely to have malar rash, nephritis and anti-dsDNA positivity. Alopecia and lymphadenopathy are most frequent in ASLE patients. JSLE are far less likely to have serositis than any other group. Patients with LSLE demonstrate comparatively low frequency of major organ involvement, but they have higher levels of CRP.References:[1]Ambrose N., et al. Differences in disease phenotype and severity in SLE across age groups. Lupus. 2016;25(14):1542-1550.[2]Livingston B., et al. Differences in autoantibody profiles and disease activity and damage scores between childhood- and adult-onset systemic lupus erythematosus: a meta-analysis. Seminars in Arthritis and Rheumatism. 2012;42(3):271-280.Disclosure of Interests:None declared

scholarly journals FRI0183 DISTINCTIVE TRAITS OF MYOCARDIAL INFLAMMATION IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A MULTICENTRE STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 675.2-675
Author(s):  
G. A. Ramirez ◽  
M. Gerosa ◽  
G. De Luca ◽  
L. Beretta ◽  
S. Sala ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Early Onset ◽  
Disease Duration ◽  
Late Onset ◽  
Myocardial Inflammation ◽  
Systemic Lupus ◽  
Research Support

Background:Myocarditis is an infrequent but potentially life-threatening inflammatory disorder and might be part of the spectrum of systemic lupus erythematosus (SLE). Little is known about the clinical and histologic features of myocarditis in SLE, especially compared to other forms of myocarditis.Objectives:to test for potential distinctive traits among myocarditis in SLE (MyoSLE), SLE without myocarditis (OnlySLE) and myocarditis without SLE (OnlyMyo)Methods:Patients with MyoSLE were identified from three centres and compared with 231 cross-sectionally enrolled patients with OnlySLE and 87 patients with OnlyMyo. MyoSLE patients were split into two groups based on myocarditis onset within (early onset) vs after (late onset) the first year from SLE diagnosis. OnlySLE patients were dichotomised in the same way based on disease duration at time of enrolment. Demographics and general clinical features were collected retrospectively. SLE disease activity index 2000 (SLEDAI-2K), SLE International Collaborating Clinics/American College of Rheumatology damage index (SDI), clinical and laboratory features were collected at time of myocarditis onset in MyoSLE and at enrolment in OnlySLE. Quantitative data are expressed as median [interquartile range].Results:Fourteen MyoSLE patients were identified, 50% with early onset. Women were equally frequent among MyoSLE (71%) and OnlySLE patients (87%) and less frequent in the OnlyMyo group (43%; p<0.001). Age was comparable among groups. Clinical features at presentation, including left ventricular ejection fraction, were similar between MyoSLE and OnlyMyo, although the former had higher levels of pro-brain natriuretic peptide (1.1 [0.4-1.8] vs 0.1 [0.1-0.5] ng/ml; p=0.004). Patients with MyoSLE also had a lower frequency of left ventricle lateral wall involvement (36 vs 68%; p=0.035) and of oedema (20 vs 71%; p=0.036) and necrosis (0 vs 64%; p=0.009) at biopsy. Antiphospholipid antibodies (aPL) were more frequent in MyoSLE (57%) compared to both OnlyMyo (16%; p=0.003) and OnlySLE (28%; p=0.031). Compared to OnlySLE, patients with MyoSLE also had a higher prevalence of aPL-syndrome (APS: 36 vs 7%; p=0.003), neuropsychiatric (NPSLE: 43 vs 19%; p=0.039) and gastrointestinal manifestations (21 vs 5%; p=0.045). Early and late onset patients had similar demographics and clinical features and did not differ from patients with OnlySLE with similar disease duration in terms of SLEDAI-2K and SDI. Late onset MyoSLE patients had a higher prevalence of NPSLE (57 vs 18%; p=0.026) and APS (57 vs 7%; p=0.001) and higher C-reactive protein levels (6 [2-12] vs 1[0-4] mg/l; p=0.024) compared to OnlySLE patients with the same disease duration.Conclusion:Demographics of patients with MyoSLE are more similar to patients with OnlySLE than to OnlyMyo patients. MyoSLE might have distinct histological and pathogenic features compared to OnlyMyo. Patients with MyoSLE show similar patterns of disease activity and accrued damage at time of myocarditis onset compared to patients with OnlySLE with the same disease duration but might diverge later on in SLE course. aPL are frequent in MyoSLE and might both contribute to the pathogenesis of myocardial inflammation and account for the high prevalence of NPSLE and APS, especially in late onset cases.References:[1]Gartshteyn Y et al., Lupus, 2020[2]Thomas G et al., J Rheumatol, 2017[3]Peretto G et al., Int J Cardiol, 2019[4]McDonnell T et al., Blood Rev, 2019Disclosure of Interests:Giuseppe Alvise Ramirez: None declared, Maria Gerosa: None declared, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Lorenzo Beretta Grant/research support from: Pfizer, Simone Sala: None declared, Giovanni Peretto: None declared, Luca Moroni: None declared, Francesca Mastropaolo: None declared, adriana cariddi: None declared, Silvia Sartorelli: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Enrica Bozzolo: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

Late-onset systemic lupus erythematosus: clinical features, course, and prognosis

2013 ◽  
Vol 32 (7) ◽  
pp. 1053-1058 ◽  
Author(s):  
Aleksandra Tomic-Lucic ◽  
Radmila Petrovic ◽  
Marija Radak-Perovic ◽  
Dragan Milovanovic ◽  
Jasmina Milovanovic ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Late Onset ◽  
Systemic Lupus ◽  
Onset Systemic Lupus Erythematosus

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

Lupus ◽  
2020 ◽  
Vol 29 (9) ◽  
pp. 1140-1145
Author(s):  
Rosana Quintana ◽  
Guillermo J Pons-Estel ◽  
Karen Roberts ◽  
Mónica Sacnún ◽  
Rosa Serrano ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Clinical Features ◽  
Latin American ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Damage Index ◽  
Systemic Lupus ◽  
Damage Accrual ◽  
Familial Lupus

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.

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