Differences in clinical manifestations and increased severity of systemic lupus erythematosus between two groups of Hispanics: European Caucasians versus Latin American mestizos (data from the RELESSER registry)

Background Systemic lupus erythematosus (SLE) is regarded as a prototype autoimmune disease because it can serve as a means for studying differences between ethnic minorities and sex. Traditionally, all Hispanics have been bracketed within the same ethnic group, but there are differences between Hispanics from Spain and those from Latin America, not to mention other Spanish-speaking populations. Objectives This study aimed to determine the demographic and clinical characteristics, severity, activity, damage, mortality and co-morbidity of SLE in Hispanics belonging to the two ethnic groups resident in Spain, and to identify any differences. Methods This was an observational, multi-centre, retrospective study. The demographic and clinical variables of patients with SLE from 45 rheumatology units were collected. The study was conducted in accordance with Good Clinical Practice guidelines. Hispanic patients from the registry were divided into two groups: Spaniards or European Caucasians (EC) and Latin American mestizos (LAM). Comparative univariate and multivariate statistical analyses were carried out. Results A total of 3490 SLE patients were included, 90% of whom were female; 3305 (92%) EC and 185 (5%) LAM. LAM patients experienced their first lupus symptoms four years earlier than EC patients and were diagnosed and included in the registry younger, and their SLE was of a shorter duration. The time in months from the first SLE symptoms to diagnosis was longer in EC patients, as were the follow-up periods. LAM patients exhibited higher prevalence rates of myositis, haemolytic anaemia and nephritis, but there were no differences in histological type or serositis. Anti-Sm, anti-Ro and anti-RNP antibodies were more frequently found in LAM patients. LAM patients also had higher levels of disease activity, severity and hospital admissions. However, there were no differences in damage index, mortality or co-morbidity index. In the multivariate analysis, after adjusting for confounders, in several models the odds ratio (95% confidence interval) for a Katz severity index >3 in LAM patients was 1.45 (1.038–2.026; p = 0.02). This difference did not extend to activity levels (i.e. SLEDAI >3; 0.98 (0.30–1.66)). Conclusion SLE in Hispanic EC patients showed clinical differences compared to Hispanic LAM patients. The latter more frequently suffered nephritis and higher severity indices. This study shows that where lupus is concerned, not all Hispanics are equal.

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Predictors of renal damage in systemic lupus erythematous patients: data from a multiethnic, multinational Latin American lupus cohort (GLADEL)

RMD Open ◽

10.1136/rmdopen-2020-001299 ◽

2020 ◽

Vol 6 (3) ◽

pp. e001299

Author(s):

Cristina Reátegui-Sokolova ◽

Manuel F Ugarte-Gil ◽

Guillermina B Harvey ◽

Daniel Wojdyla ◽

Guillermo J Pons-Estel ◽

...

Keyword(s):

Risk Factors ◽

Systemic Lupus Erythematosus ◽

Latin American ◽

Lupus Erythematosus ◽

Renal Damage ◽

Cox Regression ◽

Damage Index ◽

Early Response ◽

Male Gender ◽

Systemic Lupus

AimA decrease in proteinuria has been considered protective from renal damage in lupus nephritis (LN), but a cut-off point has yet to be established. The aim of this study was to identify the predictors of renal damage in patients with LN and to determine the best cut-off point for a decrease in proteinuria.MethodsWe included patients with LN defined clinically or histologically. Possible predictors of renal damage at the time of LN diagnosis were examined: proteinuria, low complement, anti-double-stranded DNA antibodies, red cell casts, creatinine level, hypertension, renal activity (assessed by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)), prednisone dose, immunosuppressive drugs and antimalarial use. Sociodemographic variables were included at baseline. Proteinuria was assessed at baseline and at 12 months, to determine if early response (proteinuria <0.8 g/day within 12 months since LN diagnosis) is protective of renal damage occurrence. Renal damage was defined as an increase of one or more points in the renal domain of The Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index (SDI). Cox regression models using a backward selection method were performed.ResultsFive hundred and two patients with systemic lupus erythematosus patients were included; 120 patients (23.9%) accrued renal damage during their follow-up. Early response to treatment (HR=0.58), antimalarial use (HR=0.54) and a high SES (HR=0.25) were protective of renal damage occurrence, whereas male gender (HR=1.83), hypertension (HR=1.86) and the renal component of the SLEDAI (HR=2.02) were risk factors for its occurrence.ConclusionsEarly response, antimalarial use and high SES were protective of renal damage, while male gender, hypertension and higher renal activity were risk factors for its occurrence in patients with LN.

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Association between organ damage and mortality in systemic lupus erythematosus: a systematic review and meta-analysis

BMJ Open ◽

10.1136/bmjopen-2019-031850 ◽

2020 ◽

Vol 10 (5) ◽

pp. e031850 ◽

Cited By ~ 1

Author(s):

Irene B Murimi-Worstell ◽

Dora H Lin ◽

Henk Nab ◽

Hong J Kan ◽

Oluwadamilola Onasanya ◽

...

Keyword(s):

Systematic Review ◽

Systemic Lupus Erythematosus ◽

Latin American ◽

Lupus Erythematosus ◽

Meta Analysis ◽

Damage Index ◽

Organ Damage ◽

Cochrane Library ◽

Systemic Lupus ◽

Cross Sectional

ObjectiveAt least half of patients with systemic lupus erythematosus (SLE) develop organ damage as a consequence of autoimmune disease or long-term therapeutic steroid use. This study synthesised evidence on the association between organ damage and mortality in patients with SLE.DesignSystematic review and meta-analysis.MethodsElectronic searches were performed in PubMed, Embase, Cochrane Library and Latin American and Caribbean Health Sciences Literature for observational (cohort, case-control and cross-sectional) studies published between January 2000 and February 2017. Included studies reported HRs or ORs on the association between organ damage (measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score) and mortality. Study quality was assessed using the modified Newcastle-Ottawa assessment. Pooled HRs were obtained using the DerSimonian and Laird random-effects model. Heterogeneity was assessed using the Cochrane Q (Q) and I2 statistics.ResultsThe search yielded 10 420 articles, from which 21 longitudinal studies were selected. Most studies (85%) were of high quality. For 10 studies evaluating organ damage (SDI) as a continuous variable and reporting HR as a measure of association, a 1-unit increase in SDI was associated with increased mortality; pooled HR was 1.34 (95% CI: 1.24 to 1.44, p<0.001; Q p=0.027, I2=52.1%). Exclusion of one potential outlying study reduced heterogeneity with minimal impact on pooled HR (1.33 (95% CI: 1.25 to 1.42), p<0.001, Q p=0.087, I2=42.0%). The 11 remaining studies, although they could not be aggregated because of their varying patient populations and analyses, consistently demonstrated that greater SDI was associated with increased mortality.ConclusionsOrgan damage in SLE is consistently associated with increased mortality across studies from various countries. Modifying the disease course with effective therapies and steroid-sparing regimens may reduce organ damage, improve outcomes and decrease mortality for patients with SLE.

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Hematological manifestations of systemic lupus erythematosus in the early stage: association with other symptoms of the disease and possible prognostic value

Rheumatology Science and Practice ◽

10.14412/1995-4484-2019-166-170 ◽

2019 ◽

Vol 57 (2) ◽

pp. 166-170

Author(s):

A. V. Petrov ◽

V. A. Beloglazov ◽

D. V. Shadyro ◽

A. R. Gafarova ◽

A. A. Petrov

Keyword(s):

Systemic Lupus Erythematosus ◽

Central Nervous System ◽

Nervous System ◽

Lupus Erythematosus ◽

Cumulative Incidence ◽

Early Stage ◽

Damage Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Hematological Manifestations

Objective: to assess the relationship between hematological manifestations of systemic lupus erythematosus (SLE) in the early stage of the disease and development of other syndromes and symptoms of the disease, as well as the nature and severity of internal organs damage during the subsequent five-year period.Subjects and methods. The analysis of data of examination of 89 patients with SLE during the five-year period was carried out. The frequency of clinical manifestations of SLE, the level of antinuclear and antiphospholipid antibodies, SLICC/ACR damage index (SDI) depending on the presence of hematological manifestations of SLE in the onset of the disease including leukopenia (LP), thrombocytopenia (TP) and autoimmune hemolytic anemia (AGA) were studied.Results and discussion. In the onset of SLE LP was observed in 21.3%, TP – in 26.9%, AGA – in 8.9% of patients. The presence of LP was associated with an increase of antibodies to SSA frequency, and TP – with more frequent detection of antibodies to Ro-52, cardiolipin and β-2-glycoprotein (p<0.05). Patients with TP in the onset of SLE compared with patients without hematological manifestations, had an increase (p<0.05) of the cumulative incidence of nephritis (83.3 and 42.9%), central nervous system lesions (70.8 and 26.5%), vasculitis (45.8 and 10.2%) and Libman-Sachs endocarditis (20.8 and 6.1%, respectively), which was accompanied by an increase of SDI values (median was 2.09 [2, 1.82; 2.21] and 1.12 [0.81; 1.32], p<0.05). In patients with LP, the cumulative incidence of pneumonitis and Sjogren's syndrome was increased in the onset of SLE compared with patients without hematological manifestations (15.8 vs 6.1% and 15.7 vs 2.0%, respectively, p<0.05).Conclusion. Presence of TP in the onset of SLE is a predictor of kidneys, central nervous system, peripheral vessels and heart valves damage during the next 5 years.

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Factors predictive of serious infections over time in systemic lupus erythematosus patients: data from a multi-ethnic, multi-national, Latin American lupus cohort

Lupus ◽

10.1177/0961203319860579 ◽

2019 ◽

Vol 28 (9) ◽

pp. 1101-1110 ◽

Cited By ~ 14

Author(s):

V R Pimentel-Quiroz ◽

M F Ugarte-Gil ◽

GB Harvey ◽

D Wojdyla ◽

G J Pons-Estel ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Latin American ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Hazard Ratio ◽

Systemic Lupus ◽

Serious Infections ◽

Damage Accrual ◽

Over Time

Aim The aim of this study was to identify factors predictive of serious infections over time in patients with systemic lupus erythematosus (SLE). Methods A multi-ethnic, multi-national Latin American SLE cohort was studied. Serious infection was defined as one that required hospitalization, occurred during a hospitalization or led to death. Potential predictors included were sociodemographic factors, clinical manifestations (per organ involved, lymphopenia and leukopenia, independently) and previous infections at baseline. Disease activity (SLEDAI), damage (SLICC/ACR Damage Index), non-serious infections, glucocorticoids, antimalarials (users and non-users), and immunosuppressive drugs use; the last six variables were examined as time-dependent covariates. Cox regression models were used to evaluate the predictors of serious infections using a backward elimination procedure. Univariable and multivariable analyses were performed. Results Of the 1243 patients included, 1116 (89.8%) were female. The median (interquartile range) age at diagnosis and follow-up time were 27 (20–37) years and 47.8 (17.9–68.6) months, respectively. The incidence rate of serious infections was 3.8 cases per 100 person-years. Antimalarial use (hazard ratio: 0.69; 95% confidence interval (CI): 0.48–0.99; p = 0.0440) was protective, while doses of prednisone >15 and ≤60 mg/day (hazard ratio: 4.18; 95 %CI: 1.69–10.31; p = 0.0019) and >60 mg/day (hazard ratio: 4.71; 95% CI: 1.35–16.49; p = 0.0153), use of methylprednisolone pulses (hazard ratio: 1.53; 95% CI: 1.10–2.13; p = 0.0124), increase in disease activity (hazard ratio: 1.03; 95% CI: 1.01–1.04; p = 0.0016) and damage accrual (hazard ratio: 1.22; 95% CI: 1.11–1.34; p < 0.0001) were predictive factors of serious infections. Conclusions Over time, prednisone doses higher than 15 mg/day, use of methylprednisolone pulses, increase in disease activity and damage accrual were predictive of infections, whereas antimalarial use was protective against them in SLE patients.

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IgM Anti-ß2Glycoprotein I Is Protective Against Lupus Nephritis and Renal Damage in Systemic Lupus Erythematosus

The Journal of Rheumatology ◽

10.3899/jrheum.100650 ◽

2010 ◽

Vol 38 (3) ◽

pp. 450-453 ◽

Cited By ~ 32

Author(s):

TARANEH MEHRANI ◽

MICHELLE PETRI

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Protective Effect ◽

Lupus Erythematosus ◽

Renal Damage ◽

Damage Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

American College Of Rheumatology ◽

Glycoprotein I

Objective.Antibodies to ß2glycoprotein I (IgG and IgM isotypes) have recently been added to the laboratory criteria of the revised antiphospholipid syndrome classification criteria. We investigated whether IgM anti-ß2-glycoprotein I (anti-ß2-GPI) is associated with clinical manifestations of systemic lupus erythematosus (SLE).Methods.Anti-ß2-GPI was measured in 796 patients with SLE (93% women, 53% white, 38% African American, mean age 45 yrs). IgM anti-ß2-GPI (> 20 phospholipid units) was found in 16%. Associations were determined with clinical manifestations of SLE and with components of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index.Results.As expected, IgM anti-ß2-GPI was highly associated with both the lupus anticoagulant and with anticardiolipin. It was associated with transient ischemic attack (OR 2.64, p = 0.04), but not significantly with venous or arterial thrombosis. IgM anti-ß2-GPI was protective against lupus nephritis (OR 0.54, p = 0.049), renal damage (p = 0.019), and hypertension (OR 0.58, p = 0.008). This protective effect remained after adjustment for ethnicity.Conclusion.In SLE, IgM anti-ß2-GPI is not associated with thrombosis but is protective against lupus nephritis and renal damage. “Natural” autoantibodies of the IgM isotype may have a protective effect.

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SAT0179 ADHERENCE TO HYDROXYCHLOROQUINE INFLUENCES THE INCIDENCE OF ORGAN DAMAGE DURING FOLLOW-UP IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1656 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1031.1-1031

Author(s):

J. H. Kang ◽

S. E. Choi ◽

H. Xu ◽

D. J. Park ◽

S. S. Lee

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Damage Index ◽

Clinical Manifestations ◽

Medication Possession Ratio ◽

Organ Damage ◽

Systemic Lupus ◽

Laboratory Findings ◽

The Impact

Background:Objectives:Hydroxychloroquine (HCQ) is a cornerstone drug in patients with systemic lupus erythematosus (SLE), decreasing the risk of flares and comorbidities and improving survival. This study investigated the effects of HCQ adherence on clinical manifestations, disease activity, and organ damage in Korean patients with SLE.Methods:Data on 299 SLE patients were obtained from the Korean Lupus Network registry. Demographic variables, clinical manifestations, laboratory findings, PGA, and SLEDAI-2000 and SLICC damage index scores were recorded at the time of enrollment and repeated annually for 4 consecutive years. Patients were divided into two groups according to the level of HCQ adherence. Adherence was defined by the medication possession ratio and dichotomized as ≤ 80% vs. > 80%. Univariate and multivariate analyses were performed to assess the impact of adherence to HCQ on clinical outcomes.Results:Of the 299 patients, 31 (10.4%) showed poor drug adherence during the follow-up period. Patients with poor HCQ adherence were older (P=0.011), less insured (P=0.024), experienced lower employment (P=0.033), and had a higher rate of comorbidities such as hypertension (P=0.048) and depression (P<0.001). The non-adherent group had higher mean and changed SLICC damage index scores than the adherent group across all 4 years. In the multivariate analysis, HCQ non-adherence was significantly associated with older age (OR, 1.043; 95% CI, 1.006–1.081; P=0.021), depression (OR, 11.98; 95% CI, 1.099–130.6; P=0.042), and an annual increase in the SLICC damage index score (OR, 2.275; 95% CI, 1.369–3.779; P=0.002).Conclusion:HCQ adherence might be influenced by age and depressive mood. Additionally, the poor adherence to HCQ in SLE patients was correlated with higher cumulative organ damage. Therefore, patients with SLE should be educated to take HCQ appropriately to improve their clinical outcome in clinical practice.Disclosure of Interests:None declared

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The problem of fatigue in patients with systemic lupus erythematosus according to the data on a Russian RENAISSANCE cohort

Modern Rheumatology Journal ◽

10.14412/1996-7012-2020-4-23-30 ◽

2020 ◽

Vol 14 (4) ◽

pp. 23-30

Author(s):

E. A. Aseeva ◽

S. K. Solovyev ◽

N. Yu. Nikishina ◽

G. M. Koilubaeva ◽

T. A. Lisitsyna ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Activity Index ◽

Damage Index ◽

Clinical Manifestations ◽

Common Symptom ◽

Systemic Lupus ◽

Wide Range ◽

Antibody Levels

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of clinical manifestations. Numerous observations and surveys of patients have shown that the most common symptom of SLE is fatigue complaints in 51 to 90% of patients.Objective: to determine the significance of fatigue in the general health status of RENAISSANCE cohort patients with SLE who were hospitalized in the Clinic, V.A. Nasonova Research Institute of Rheumatology.Patients and methods. The investigation included SLE patients aged 18 years and older who met the 2012 SLICC criteria. The standard examination accepted in the management of patients with SLE was made. Disease activity was determined by SLEDAI-2K; irreversible lesions in various organs were identified using the SLICC damage index. The SF-36 and the LupusQoL questionnaires were used to assess health-related quality of life (HRQOL) and the FACIT-Fatigue scale was applied to measure fatigue.Results and discussion. The investigation enrolled 328 patients, mainly women (91%); the mean age was 34.4±11.5 years; the duration of the disease was 106.3±97.9 months. In this group, moderate and high disease activities (SLEDAI-2K scores of 6–10 and 11–19, respectively) were observed at approximately the same frequency. At the time of inclusion, more than half (56.5%) of the patients already had various irreversible organ lesions. At Visit 1, the FACIT-Fatigue scale showed that fatigue was present in 148 (45%) of the 328 patients. According to the presence of fatigue, the patients were divided into two groups. Group 1 included 148 patients with fatigue; Group 2 consisted of 180 patients without fatigue. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and anti-DNA antibody levels were significantly higher in the fatigue group (p=0.01 and p=0.02, respectively); the patients also had decreased HRQOL according to 7 LupusQol domains (p<0.001). The patients with fatigue were significantly more likely to receive intravenous glucocorticoids and rituximab. At 12 months after the start of treatment, the patients with fatigue were found to have a statistically significant reduction in disease activity, as well as normalization of anti-DNA antibody levels, improvements in HRQOL according to the LupusQol domains, and less severity of fatigue according to the FACIT-Fatigue scale.Conclusion. Fatigue was detected in almost half (45–53%) of SLE patients. It is associated with a higher disease activity by SLEDAI-2K and with a high anti-DNA antibody level. The patients with fatigue are observed to have an obvious worsening of HRQOL according to all LupusQol domains.

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Systemic lupus erythematosus children in Egypt: Homeland spectrum amid the global situation

Lupus ◽

10.1177/09612033211043010 ◽

2021 ◽

pp. 096120332110430

Author(s):

Nahla N Eesa ◽

Hend Abdel Nabi ◽

Rasha El Owaidy ◽

Iman Khalifa ◽

Ahmed R Radwan ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Disease Onset ◽

Damage Index ◽

Clinical Manifestations ◽

Skin Lesions ◽

Systemic Lupus ◽

Disease Characteristics ◽

Egyptian Children

Objectives This study aims to present the manifestations of juvenile systemic lupus erythematosus (JSLE) across Egypt, to focus on age at onset and gender-driven influence on disease characteristics, and to compare findings to other countries. Methods The study included 404 Egyptian children with systemic lupus erythematosus (SLE) presenting to one of the specialized rheumatology centers corresponding to 13 major governorates. Juvenile cases age was ≤ 16°years at the time of recruitment. The SLE Disease Activity Index (SLEDAI) and damage index (DI) were assessed. Results The mean age was 13.2 ± 2.4°years; 355 females and 49 males (7.2:1), and the disease duration was 2.3 ± 1.6 years, while age at disease onset was 11.1 ± 2.5°years. Their SLEDAI was 13.5 ± 12.3, and DI, 0.36 ± 0.78. The overall estimated prevalence of childhood-SLE patients in the recruited cohort in Egypt was 1/100,000 population (0.24/100000 males and 1.8/100000 females). 7.4% developed pre-pubertal SLE (≤ 7 years); 73.3%, peri-pubertal; and 19.3% during early adolescence. The differences according to age group were equal for gender and clinical manifestations except skin lesions present in 59.3% of pre-pubertal onset, 74.6% of peri-pubertal, and 84.2% of adolescents ( p = 0.029), and renal involvement in 73.8% of peripubertal, 62.1% of pre-pubertal and 58.9% of adolescents ( p = 0.03). Laboratory investigations, SLEDAI, and DI were similar among age categories. Lupus nephritis was more common in Egypt compared to JSLE from other countries. Conclusion Our large multicenter study identified that female gender influenced disease characteristics with more frequent skin involvement. Skin lesions were significantly higher in adolescents, while renal involvement in peri-pubertal children.

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Clinical and immunological pattern and outcome of Egyptian systemic lupus erythematosus patients: a single center experience

Lupus ◽

10.1177/0961203318776085 ◽

2018 ◽

Vol 27 (9) ◽

pp. 1562-1569 ◽

Cited By ~ 9

Author(s):

G A Mahmoud ◽

A A Shahin ◽

H S Zayed ◽

A Moghazy ◽

B M Eissa

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Survival Rates ◽

Disease Onset ◽

Elevated Serum ◽

Damage Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Oral Ulcers ◽

Increased Risk

Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002–2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90.8% with oral ulcers affecting 52.5%), arthritis (80.3%), nephritis (67.8%), hematologic involvement (64.9%), serositis (55.2%) and neuropsychiatric manifestations (44.3%). The frequencies of antinuclear antibodies were 94.3%, anti-dsDNA 74.8%, anti-Smith 11%, anticardiolipin antibodies 29.5% and lupus anticoagulant 19.8%. Infections, predominantly bacterial, affected 337 (43.8%) patients. Thirty-three (4.3%) patients died. The main causes of death were sepsis and disease activity. The five- and 10-year survival rates for the total cohort were 97.4% and 96.3%, respectively, and were 96% and 92%, respectively for those with nephritis ( p = 0.008). Autoimmune hemolytic anemia, thrombocytopenia, elevated serum creatinine, a higher damage index, infections, a higher glucocorticoid dose and cyclophosphamide use ≥ six months were associated with an increased risk of mortality with odds ratios of 3.69, p < 0.01; 4.12, p < 0.001; 1.54, p < 0.001; 1.43, p < 0.001; 5.08, p < 0.001; 5.04, p < 0.001 and 2.25, p = 0.03, respectively. Conclusion Compared to other cohorts, a relatively lower mean age at systemic lupus erythematosus onset and higher frequencies of oral ulcers, serositis and nephritis were found.

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

Lupus ◽

10.1177/0961203320935184 ◽

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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