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1.
MMWR Morb Mortal Wkly Rep ; 70(7): 236-239, 2021 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-33600382

RESUMO

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with manifestations that vary widely in severity. Although minority populations are at higher risk for SLE and have more severe outcomes (1), population-based estimates of mortality by race and ethnicity are often lacking, particularly for Asian and Hispanic/Latino persons. Among 812 patients in the California Lupus Surveillance Project (CLSP) during 2007-2009 (2,3), who were matched to the 2007-2017 National Death Index (NDI), 16.6% had died by 2017. This proportion included persons of White (14.4%), Black (25%), Asian (15.3%), and Hispanic/Latino (15.5%) race/ethnicity. Standardized mortality ratios (SMRs) of observed-to-expected deaths among persons with SLE within each racial/ethnic group were 2.3, 2.0, 3.8, and 3.9, respectively. These findings provide the first population-based estimates of mortality among Asian and Hispanic/Latino persons with SLE. Coordination of robust care models between primary care providers and rheumatologists could ensure that persons with SLE receive a timely diagnosis and appropriate treatments that might help address SLE-associated mortality.


Assuntos
Americanos Asiáticos/estatística & dados numéricos , Hispano-Americanos/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/mortalidade , Grupos Minoritários/estatística & dados numéricos , Adolescente , Adulto , Idoso , California/epidemiologia , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mortalidade/etnologia , Adulto Jovem
2.
Adv Rheumatol ; 60(1): 32, 2020 06 09.
Artigo em Inglês | MEDLINE | ID: mdl-32517786

RESUMO

Hydroxychloroquine and chloroquine, also known as antimalarial drugs, are widely used in the treatment of rheumatic diseases and have recently become the focus of attention because of the ongoing COVID-19 pandemic. Rheumatologists have been using antimalarials to manage patients with chronic immune-mediated inflammatory rheumatic diseases for decades. It is an appropriate time to review their immunomodulatory and anti-inflammatory mechanisms impact on disease activity and survival of systemic lupus erythematosus patient, including antiplatelet effect, metabolic and lipid benefits. We also discuss possible adverse effects, adding a practical and comprehensive approach to monitoring rheumatic patients during treatment with these drugs.


Assuntos
Antimaláricos/farmacologia , Artrite Reumatoide/tratamento farmacológico , Cloroquina/farmacologia , Hidroxicloroquina/farmacologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/imunologia , Antirreumáticos/uso terapêutico , Artrite Reumatoide/imunologia , Infecções por Coronavirus/tratamento farmacológico , Erupção por Droga/etiologia , Interações Medicamentosas , Feminino , Glucose/metabolismo , Cardiopatias/induzido quimicamente , Humanos , Lipídeos/sangue , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Lúpus Eritematoso Cutâneo/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pandemias , Agregação Plaquetária/efeitos dos fármacos , Pneumonia Viral/tratamento farmacológico , Gravidez , Insuficiência Renal/prevenção & controle , Doenças Retinianas/induzido quimicamente , Síndrome de Sjogren/tratamento farmacológico , Síndrome de Sjogren/imunologia
3.
Autoimmun Rev ; 19(6): 102531, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32234406

RESUMO

AIM: To describe changes in the 2001-2014 mortality of 6 autoimmune systemic diseases (AISDs), namely Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc), Idiopathic Inflammatory Myopathies (IIM), Sjögren's Syndrome (SS), Mixed Connective Tissue Disease (MCTD) and ANCA-associated vasculitis (AAV) at the country-, continent-, and world-levels. METHODS: Mortality data were retrieved from the World Health Organization (WHO) mortality database for each disease, based on ICD-10 codes. We computed age-standardized mortality rate (ASMR) as the estimated number of deaths per million inhabitants and its 95% confidence interval (95%CI). The association between gender, geographical areas and disease-specific mortality was analyzed using multivariate Poisson regression. The 2001-2014 temporal trends were analyzed using Jointpoint software. RESULTS: In 2014, the worldwide ASMR for SLE was 2.68 (95%CI: 2.62-2.75) deaths/millions inhabitants, 1.46 (1.42-1.51) for SSc, 0.47 (0.44-0.49) for IIM, 0.17 (0.15-0.18) for SS, 0.11 (0.10-0.13) for MCTD and 0.53 (0.50-0.56) for AAV, with ASMRs generally lower in Europe than in North America, Latin America and Asia. Between 2001 and 2014, the worldwide ASMR decreased significantly for SSc (-0.71%/year), IIM (-1.65%/year) and AAV (-1.01%/year; p < .001 for all) and increased for SS (+1.53%/year, p = .01). The worldwide ASMR of SLE decreased significantly between 2001 and 2003 (-6.37%, p < .05) before increasing slightly between 2004 and 2014 (+0.58%, p < .01). CONCLUSIONS: We observed a strong heterogeneity of standardized mortality rates across all countries analyzed for 6 autoimmune diseases. Those results further highlight the impact of world-wide inequities and major gaps in access to care and strategies for diagnosis and management of rare diseases, a crucial finding for world-wide physicians, patient associations and policy makers.


Assuntos
Doenças Autoimunes/mortalidade , Causas de Morte , Internacionalidade , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/mortalidade , Humanos , Lúpus Eritematoso Sistêmico/mortalidade , Doença Mista do Tecido Conjuntivo/mortalidade , Miosite/mortalidade , Escleroderma Sistêmico/mortalidade , Síndrome de Sjogren/mortalidade
4.
Sci Rep ; 10(1): 4133, 2020 03 05.
Artigo em Inglês | MEDLINE | ID: mdl-32139803

RESUMO

Systemic lupus erythematosus (SLE) might increase deep neck infection (DNI) risk, but evidence supporting this hypothesis is limited. In this retrospective follow-up study, the SLE-DNI association was investigated using data from the Registry for Catastrophic Illness Patients, which is a subset of the Taiwan National Health Insurance Research Database. All patients newly diagnosed as having SLE in 1997-2011 were identified, and every SLE patient was individually matched to four patients without SLE according to sex, age, and socioeconomic status. The study outcome was DNI occurrence. DNI treatment modalities and prognoses in SLE and non-SLE patients, along with the association of steroid dose with DNI risk, were also studied. In total, 17,426 SLE and 69,704 non-SLE patients were enrolled. Cumulative DNI incidence was significantly higher in the SLE cohort than in the non-SLE cohort (p < 0.001). The Cox regression model demonstrated that SLE significantly increased DNI risk (hazard ratio: 4.70; 95% confidence interval: 3.50-6.32, p < 0.001). Moreover, in the sensitivity and subgroup analyses, the effect of SLE on DNI was stable. Relatively few SLE-DNI patients received surgical interventions (15.6% vs. 28.6%, p = 0.033). The between-group differences in tracheostomy use and hospitalisation duration were nonsignificant. In SLE patients, high steroid doses significantly increased DNI incidence (≥3 vs. <3 mg/day = 2.21% vs. 0.52%, p < 0.001). This is the first study demonstrating that SLE increases DNI risk by approximately five times and that high steroid dose increases DNI incidence in SLE patients.


Assuntos
Lúpus Eritematoso Sistêmico/epidemiologia , Adulto , Feminino , Humanos , Incidência , Estimativa de Kaplan-Meier , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Análise Multivariada , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Fatores Socioeconômicos , Adulto Jovem
5.
Lupus ; 29(2): 191-198, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31959041

RESUMO

OBJECTIVE: This study aimed to investigate how septicaemia, non-septicaemia infection and the disease itself are associated with disease activity and mortality in inpatients with systemic lupus erythematosus (SLE) in Taiwan. METHODS: We retrospectively reviewed 1115 patients and enrolled 427 with SLE admitted for lupus flare-ups and co-morbidities. Disease activity and infection type/site were recorded and categorized according to the causes of admission and mortality into three categories, of which two were specified as follows: (a) septicaemia admissions, non-septicaemia admissions; and (b) septicaemia mortality, non-septicaemia infection mortality and non-infection mortality. The relationships between lupus flare-ups and mortality in different groups were analysed using an unpaired t-test, Mann-Whitney U-test and logistic regression. RESULTS: Septicaemia was the major cause of mortality in SLE inpatients. There were 98 (22.95%) mortality patients among all 427 SLE patients. The septicaemia admissions had higher disease activity (SLE Disease Activity Index 2000 = 13.00 ± 7.98) than the non-septicaemia admissions (9.77 ± 5.72; p < 0.01). The mean current SLEDAI score of the septicaemia mortality group (14.91 ± 8.01) was higher than that of the non-septicaemia infection mortality group (10.05 ± 5.75; p = 0.02), in spite of the similar mean earlier SLEDAI score. The risk of mortality in the septicaemia mortality group due to previous septicaemia admissions was 13.2 times (odds ratio) higher than in the non-septicaemia infection mortality group and 15.6 times higher than in the non-infection mortality group. CONCLUSION: Septicaemia relates to increased lupus disease activity and is associated with a greater risk of mortality in the SLE patients than other causes of admission. Fewer previous septicaemia admissions decrease the risk of septicaemia mortality.


Assuntos
Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/mortalidade , Sepse/mortalidade , Índice de Gravidade de Doença , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taiwan/epidemiologia , Adulto Jovem
6.
Ann Rheum Dis ; 79(3): 356-362, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31915121

RESUMO

OBJECTIVES: Using a reversible multistate model, we prospectively examined neuropsychiatric (NP) events for attribution, outcome and association with health-related quality of life (HRQoL), in an international, inception cohort of systemic lupus erythematosus (SLE) patients. METHODS: Annual assessments for 19 NP events attributed to SLE and non-SLE causes, physician determination of outcome and patient HRQoL (short-form (SF)-36 scores) were measured. Time-to-event analysis and multistate modelling examined the onset, recurrence and transition between NP states. RESULTS: NP events occurred in 955/1827 (52.3%) patients and 592/1910 (31.0%) unique events were attributed to SLE. In the first 2 years of follow-up the relative risk (95% CI) for SLE NP events was 6.16 (4.96, 7.66) and non-SLE events was 4.66 (4.01, 5.43) compared with thereafter. Patients without SLE NP events at initial assessment had a 74% probability of being event free at 10 years. For non-SLE NP events the estimate was 48%. The majority of NP events resolved over 10 years but mortality was higher in patients with NP events attributed to SLE (16%) versus patients with no NPSLE events (6%) while the rate was comparable in patients with non-SLE NP events (7%) compared with patients with no non-SLE events (6%). Patients with NP events had lower SF-36 summary scores compared with those without NP events and resolved NP states (p<0.001). CONCLUSIONS: NP events occur most frequently around the diagnosis of SLE. Although the majority of events resolve they are associated with reduced HRQoL and excess mortality. Multistate modelling is well suited for the assessment of NP events in SLE.


Assuntos
Lúpus Eritematoso Sistêmico/psicologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/psicologia , Adulto , Feminino , Humanos , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/mortalidade , Vasculite Associada ao Lúpus do Sistema Nervoso Central/mortalidade , Masculino , Pessoa de Meia-Idade , Modelos Estatísticos , Análise Multinível , Estudos Prospectivos , Qualidade de Vida
7.
Rheumatology (Oxford) ; 59(3): 524-533, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31377781

RESUMO

OBJECTIVE: Damage in patients with systemic lupus erythematosus is irreversible change in organs due to disease activity, concomitant disease or medication side-effects. It is measured using the Systemic Lupus International Collaborative Clinics Damage Index (SDI) and is associated with increased mortality. Previous reports have suggested associations between damage accrual and various ethnic, disease and treatment factors, but there is a dearth of long-term follow-up data from large multi-ethnic cohorts. We describe a study of damage and mortality in 300 patients from London, UK followed for up to 40 years. METHODS: We carried out retrospective analysis of medical records and SDI scores of 300 patients followed for up to 40 years (median 13.3 years). Characteristics of the groups who did and did not develop damage and those who died or survived to the end of follow-up were compared using univariable and multivariable analysis. Kaplan-Meier analysis was used to analyse factors affecting mortality and accrual of damage. RESULTS: Damage developed in 231/300 (77%) of patients. There was a linear accrual of damage over 40 years follow-up. Factors associated with damage were African/Caribbean ethnicity, renal and cerebral involvement, early use of high-dose corticosteroids or immunosuppressants, anti-RNP and antiphospholipid antibodies. Damage was strongly associated with mortality. Of 87 patients who died, 93% had damage compared with 70% of survivors (P < 0.001). CONCLUSION: Development of damage is strongly associated with increased mortality. We identified groups at increased risk of developing damage, including those treated with high-dose steroids and immunosuppressants within the first two years.


Assuntos
Lúpus Eritematoso Sistêmico/patologia , Corticosteroides/uso terapêutico , Adulto , Progressão da Doença , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida , Reino Unido/epidemiologia
8.
Rheumatology (Oxford) ; 59(3): 495-504, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31321417

RESUMO

OBJECTIVE: SLE is associated with high risks of cardiovascular disease (CVD) and mortality, and has a wide spectrum of presentations. We investigated whether SLE severity at diagnosis was associated with CVD or mortality risk. METHODS: Within Medicaid (2000-10), we identified patients 18-65 years of age with incident SLE. Initial SLE severity was classified-mild, moderate, or severe-during the baseline year prior to the start of follow-up (incident index date) using a published algorithm based on SLE-related medications and diagnoses. Patients were followed from the index date to the first CVD event or death, disenrollment, loss to follow-up or end of follow-up period. Cox and Fine-Gray regression models, adjusted for demographics and comorbidities accounting for the competing risk of death (for CVD), estimated CVD and mortality risks by baseline SLE severity. RESULTS: Of 15 120 incident SLE patients, 48.7% had mild initial SLE severity, 33.9% moderate and 17.4% severe. Mean (s.d.) follow-up was 3.3 (2.4) years. After multivariable adjustment, CVD subdistribution hazard ratios (HRSD) were higher for initially severe [HRSD 1.64 (95% CI 1.32, 2.04)] and moderate [HRSD 1.19 (95% CI 1.00, 1.41)] SLE vs mild SLE. Mortality HRs were also higher for initially severe [HR 3.11 (95% CI 2.49, 3.89)] and moderate [HR 1.61 (95% CI 1.29, 2.01)] SLE vs mild SLE. CONCLUSION: SLE patients with high initial severity had elevated mortality and CVD events risks compared with those who presented with milder disease. This has implications for clinical care and risk stratification of newly diagnosed SLE patients.


Assuntos
Doenças Cardiovasculares/epidemiologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Adolescente , Adulto , Idoso , Feminino , Humanos , Incidência , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Adulto Jovem
9.
Rheumatology (Oxford) ; 59(1): 146-152, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31257438

RESUMO

OBJECTIVES: aPL are present in between 20 and 30% of patients with SLE. They can cause vascular events (VE) or pregnancy morbidity. aCL and anti-beta-2-glycoprotein I (anti-ß2GPI) are measured in clinical practice. Domain I (DI) of ß2GPI is the main site for aPL binding. We investigated the prevalence of IgG anti-DI, aCL and anti-ß2GPI antibodies in early SLE and their association with mortality and development of VE. METHODS: Samples from 501 patients with SLE that had been obtained and stored early during their disease were tested for IgG anti-DI, aCL and anti-ß2GPI antibodies by ELISA. LA status and history of VE were obtained by reviewing medical records. Kaplan-Meier analysis was used to investigate mortality and occurrence of VE, comparing groups with and without aPL in early disease. RESULTS: Of 501 patients, 190 (38%) had at least one of these aPL, of whom 112 had anti-DI alone. Of 276 patients with complete vascular history, 83 had experienced VE. The 39 patients who were double or triple-ELISA-positive for any combination of the three aPL were more likely to have or develop lupus anticoagulant (P<0.0001) than those who were single-ELISA-positive or negative. In Kaplan-Meier analysis, they showed a trend towards developing more VE (P = 0.06). CONCLUSION: IgG anti-DI antibodies were present in early serum samples from 29% of patients and were more common than IgG aCL or anti-ß2GPI. There was some evidence suggesting that double or triple-ELISA-positivity for these antibodies identified a group with worse outcomes.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Doenças Cardiovasculares/imunologia , Doenças Cardiovasculares/mortalidade , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/mortalidade , Adolescente , Adulto , Idoso , Anticorpos Anticardiolipina/sangue , Criança , Pré-Escolar , Feminino , Humanos , Imunoglobulina G/imunologia , Lactente , Lúpus Eritematoso Sistêmico/sangue , Masculino , Pessoa de Meia-Idade , Gravidez , Adulto Jovem , beta 2-Glicoproteína I/imunologia
10.
Ann Rheum Dis ; 79(3): 363-369, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31826855

RESUMO

OBJECTIVES: To investigate associations between a high genetic disease risk and disease severity in patients with systemic lupus erythematosus (SLE). METHODS: Patients with SLE (n=1001, discovery cohort and n=5524, replication cohort) and healthy controls (n=2802 and n=9859) were genotyped using a 200K Immunochip single nucleotide polymorphism array. A genetic risk score (GRS) was assigned to each individual based on 57 SLE risk loci. RESULTS: SLE was more prevalent in the high, compared with the low, GRS-quartile (OR 12.32 (9.53 to 15.71), p=7.9×10-86 and OR 7.48 (6.73 to 8.32), p=2.2×10-304 for the discovery and the replication cohorts, respectively). In the discovery cohort, patients in the high GRS-quartile had a 6-year earlier mean disease onset (HR 1.47 (1.22 to 1.75), p=4.3×10-5), displayed higher prevalence of damage accrual (OR 1.47 (1.06 to 2.04), p=2.0×10-2), renal disorder (OR 2.22 (1.50 to 3.27), p=5.9×10-5), anti-dsDNA (OR 1.83 (1.19 to 2.81), p=6.1×10-3), end-stage renal disease (ESRD) (OR 5.58 (1.50 to 20.79), p=1.0×10-2), proliferative nephritis (OR 2.42 (1.30 to 4.49), p=5.1×10-3), anti-cardiolipin-IgG (OR 1.89 (1.13 to 3.18), p=1.6×10-2), anti-ß2-glycoprotein-I-IgG (OR 2.29 (1.29 to 4.06), p=4.8×10-3) and positive lupus anticoagulant test (OR 2.12 (1.16 to 3.89), p=1.5×10-2) compared with patients in the low GRS-quartile. Survival analysis showed earlier onset of the first organ damage (HR 1.51 (1.04 to 2.25), p=3.7×10-2), first cardiovascular event (HR 1.65 (1.03 to 2.64), p=2.6×10-2), nephritis (HR 2.53 (1.72 to 3.71), p=9.6×10-7), ESRD (HR 6.78 (1.78 to 26.86), p=6.5×10-3) and decreased overall survival (HR 1.83 (1.02 to 3.30), p=4.3×10-2) in high to low quartile comparison. CONCLUSIONS: A high GRS is associated with increased risk of organ damage, renal dysfunction and all-cause mortality. Our results indicate that genetic profiling may be useful for predicting outcomes in patients with SLE.


Assuntos
Predisposição Genética para Doença/epidemiologia , Lúpus Eritematoso Sistêmico/genética , Nefrite Lúpica/genética , Medição de Risco/estatística & dados numéricos , Índice de Gravidade de Doença , Adulto , Anticorpos Anticardiolipina/sangue , Estudos de Casos e Controles , Feminino , Genótipo , Humanos , Falência Renal Crônica/genética , Falência Renal Crônica/mortalidade , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/mortalidade , Nefrite Lúpica/mortalidade , Masculino , Pessoa de Meia-Idade , Prevalência , Risco , Fatores de Risco , Taxa de Sobrevida , beta 2-Glicoproteína I/imunologia
11.
Arthritis Care Res (Hoboken) ; 72(3): 447-451, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-30821926

RESUMO

OBJECTIVE: To assess the impact of achieving Lupus Low Disease Activity State ≥50% of the time (LLDAS-50) on damage accrual and mortality in an inception cohort of patients with systemic lupus erythematosus (SLE). METHODS: We used data from the Tromsø Lupus Cohort, a longitudinal population-based study of all patients with SLE in the 2 northernmost counties in Norway. LLDAS was defined as 1) a Systemic Lupus Erythematosus Disease Activity Index 2000 score of ≤4, with no activity in major organ systems, 2) no new features of lupus disease activity, 3) current therapy with prednisolone (or equivalent) dosage of ≤7.5 mg daily, and 4) well-tolerated standard maintenance dosages of immunosuppressive drugs. RESULTS: A total of 69 patients (33.5%) spent at least half of their follow-up time in LLDAS (thus, achieving LLDAS-50) and had significantly better survival and lower risk of developing severe damage over time, according to the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. After correcting for age and sex, LLDAS-50 was associated with a significant reduction in risk of having severe damage (hazard ratio [HR] 0.37 [95% confidence interval (95% CI) 0.19-0.73], P < 0.01), and also a reduction in mortality (HR 0.31 [95% CI 0.16-0.62], P < 0.01). CONCLUSION: Our study validates the findings of the inception cohort by demonstrating that achievement of LLDAS-50 is associated with a significant reduction in severe damage and, for the first time, demonstrates a reduction in mortality.


Assuntos
Lúpus Eritematoso Sistêmico/mortalidade , Índice de Gravidade de Doença , Adulto , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Noruega/epidemiologia
12.
PLoS One ; 14(11): e0225516, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31747435

RESUMO

OBJECTIVES: To investigate the prevalence, clinical characteristics, and prognosis of thrombocytopenia (TP) in Chinese patients with systemic lupus erythematosus (SLE). METHODS: The study was conducted based on the Chinese SLE Treatment and Research group (CSTAR) registry. Thrombocytopenia was defined as the platelet count<100,000/mm3 at enrollment. Severe thrombocytopenia was defined as the platelet count<50,000/mm3. The prevalence of SLE-related TP, the associations of thrombocytopenia with demographic data, organ involvements, laboratory findings, disease activity, damage, and mortality were investigated. RESULTS: Of 2104 patients with SLE, 342 patients (16.3%) were diagnosed with thrombocytopenia. The prevalence of neuropsychiatric SLE, vasculitis, myositis, nephritis, mucocutaneous lesions, pleuritis, fever, leukocytopenia and hypocomplementemia were significantly higher in patients with thrombocytopenia (p<0.05). SLE disease activity index (SLEDAI) was significantly higher in patients with thrombocytopenia (p<0.05). Multivariate analysis showed that leukocytopenia (OR = 2.644), lupus nephritis (OR = 1.539), hypocomplementemia (OR = 1.497) and elevated SLEDAI (OR = 1.318) were independently associated with thrombocytopenia (p<0.05). Long disease duration (OR = 1.006) was an independent risk factor of severe thrombocytopenia, while anti-rRNP (OR = 0.208) was an independent protective factor of severe thrombocytopenia (p<0.05). Long disease duration was an independent risk factor of mortality in patients with thrombocytopenia (RR = 1.006). The 6-year survival of patients with thrombocytopenia was significantly lower than patients without thrombocytopenia (88.2% vs. 95.5%). CONCLUSIONS: Thrombocytopenia was a common manifestation of SLE and was associated with leukocytopenia, nephritis and severe disease activity. Severe thrombocytopenia tended to occur in long-term and relatively inactive SLE. Patients with SLE-related thrombocytopenia has a decreased long-term survival rate. Long disease duration was an independent risk factor of mortality in patients with thrombocytopenia.


Assuntos
Lúpus Eritematoso Sistêmico/patologia , Trombocitopenia/diagnóstico , Adulto , Grupo com Ancestrais do Continente Asiático , China/epidemiologia , Feminino , Humanos , Modelos Logísticos , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prevalência , Prognóstico , Modelos de Riscos Proporcionais , Sistema de Registros , Fatores de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Trombocitopenia/complicações , Trombocitopenia/epidemiologia , Adulto Jovem
13.
Lupus ; 28(14): 1699-1704, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31640531

RESUMO

Patients with systemic lupus erythematosus (SLE) are at high risk of tuberculosis (TB) because of their immunocompromised status and the use of immunosuppressive drugs. In endemic regions, TB complicates the diagnosis and treatment of SLE, but the risk factors of mortality in these patients have not been investigated. In this study, we reviewed medical records during 2006-2016. Patients who fulfilled the 1997 American College of Rheumatology SLE criteria and presented with definite TB were enrolled. The primary outcome was mortality during TB treatment. There were 5388 SLE patients screened, and 30 patients were enrolled. Seven patients died during follow-up. Compared with the survival group, patients in the mortality group had significantly more central nervous system involvement of TB, higher Systemic Lupus Erythematosus Disease Activity Index-2000 scores and more cyclophosphamide use before TB, and higher prednisolone dose before and during TB treatment. Cox regression showed that prednisolone dose during TB treatment was an independent risk factor for mortality (per 10 mg/day increase, hazard ratio (HR) 1.61, p = .019). For SLE patients, prednisolone dose during TB treatment is an independent risk factor for mortality. Keeping prednisolone dose at less than 25 mg per day during TB treatment might be a reasonable strategy in these patients.


Assuntos
Glucocorticoides/efeitos adversos , Hospedeiro Imunocomprometido , Lúpus Eritematoso Sistêmico/mortalidade , Prednisolona/efeitos adversos , Tuberculose/mortalidade , Adulto , Idoso , Ciclofosfamida/uso terapêutico , Feminino , Glucocorticoides/administração & dosagem , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Taiwan/epidemiologia , Tuberculose/complicações , Tuberculose/tratamento farmacológico
14.
Nutr Metab Cardiovasc Dis ; 29(12): 1400-1407, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31648884

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) is associated with a higher risk of cardiovascular disease. However, it is not clear whether or not SLE is associated with poor outcomes after acute myocardial infarction (AMI). METHODS AND RESULTS: Using the Taiwan National Health Insurance Database, we identified the SLE group as patients with AMI who have a concurrent discharge diagnosis of SLE. We also selected an age-, sex-, hospital level-, and admission calendar year-matched non-SLE group at a ratio of 1:3 from the total non-SLE group. One hundred fifty-one patients with SLE, 113,791 patients without SLE, and 453 matched patients without SLE were admitted with a diagnosis of AMI. Patients with SLE were significantly younger, predominantly female, and more likely to have chronic kidney disease than those without SLE. The in-hospital mortality rates were 12.6%, 9.0%, and 4.2% in the SLE, total non-SLE, and matched non-SLE groups, respectively. The in-hospital mortality was significantly higher in the SLE group than in the total non-SLE group (OR = 1.98; 95% CI = 1.2-3.26) and the matched non-SLE group (mortality OR = 2.20; 95% CI = 1.06-4.58). In addition, the SLE group was associated with a borderline significant risk of prolonged hospitalization when compared with the non-SLE group. CONCLUSION: SLE is associated with a higher risk of in-hospital mortality and a borderline significantly higher risk of prolonged hospitalization after AMI.


Assuntos
Mortalidade Hospitalar , Lúpus Eritematoso Sistêmico/mortalidade , Infarto do Miocárdio/mortalidade , Infarto do Miocárdio/terapia , Adulto , Fatores Etários , Idoso , Bases de Dados Factuais , Feminino , Humanos , Tempo de Internação , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/diagnóstico , Prognóstico , Insuficiência Renal Crônica/mortalidade , Medição de Risco , Fatores de Risco , Fatores Sexuais , Taiwan/epidemiologia , Fatores de Tempo , Adulto Jovem
15.
Rheumatol Int ; 39(12): 2069-2075, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31570977

RESUMO

The aim of this study was to determine the causes of mortality in patients with systemic lupus erythematosus (SLE) at the University Hospital Coventry and Warwickshire (UHCW) NHS Trust over a 10 year period. This was a retrospective study of patients who had died in UHCW NHS Trust between 2007 and 2016, where SLE or lupus was mentioned on the death certificate. Ethics approval was obtained from the Research and Development. We identified 22 patients out of 1979 admissions with SLE who had died during the period between 2007 and 2016, 7 of these patients were under 50 years of age. The leading cause of death was infection with pneumococcus being associated with two deaths. Active disease was associated with younger age at death. Median age at death was 58.5 years, with median duration of disease of 14.5 years. Constitutional and mucocutaneous features were the most common items scoring on disease activity, seen in 68.2% and 45.45%, respectively. We identified three patients with biopsy proven lupus nephritis and one patient with CNS lupus. Surprisingly, none of the patients died because of vascular problems. The study suggests a changing trend in SLE mortality with none of the deaths in this cohort being due to cardiovascular or cerebrovascular disease. Infection continues to be the biggest reason for mortality in this cohort and greater emphasis is needed on vaccination for preventable infections like pneumococcus.


Assuntos
Hospitais Universitários , Lúpus Eritematoso Sistêmico/mortalidade , Adulto , Feminino , Mortalidade Hospitalar , Humanos , Pacientes Internados , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Medicina Estatal , Reino Unido/epidemiologia
16.
Exp Clin Transplant ; 17(6): 720-726, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31580235

RESUMO

OBJECTIVES: Systemic lupus erythematosus and granulomatosis with polyangiitis are systemic inflammatory conditions associated with renalfailure that can recur after renal transplant. Patients with these conditions are treated with chronic immunosuppression, potentially increasing risk of secondary malignancies. Here, we investigated long-term outcomes in renal transplant recipients with these conditions. MATERIALS AND METHODS: Transplant recipients with end-stage kidney disease due to systemic lupus erythematosus and granulomatosis with polyangiitis seen between 1982 and 2016 at a national kidney transplant center were included. Primary outcome variables were long-term allograft survival and incidence of secondary malignancy. Secondary outcome measures were incidence of delayed graft function, primary disease recurrence, and serum creatinine at follow-up. RESULTS: Ninety-eight transplant procedures (90 from deceased donors) in 92 consecutive patients (mean age 42.3 ± 14.4 y) were included: 55 with systemic lupus erythematosus and 37 with granulomatosis with polyangiitis. Follow-up duration was 110.53 ± 81.95 months (range, 1-393 mo). Overall renal allograft survival was 94.7% at 1 year, 85.4% at 5 years, and 75.4% at 10 years posttransplant. Patientswith systemic lupus erythematosus showed overall allograft survival of 91.6% at 1 year, 84.3% at 5 years, and 74.4% at 10 years. There was 1 allograft failure due to recurrence of primary disease in this group. Patients with granulomatosis with polyangiitis showed overall allograft survival of 100% at 1 year, 92.4% at 5 years, and 92.4% at 10 years. There were 21 mortalities, with 5 (23.8%) due to secondary malignancy. In total, 46 malignancies were diagnosed in 31 patients. CONCLUSIONS: We found excellent long-term renal allograft survival rates in patients with systemic lupus erythematosus and granulomatosis with polyangiitis, with secondary malignancy rates similar to those shown in recipients without autoimmune diseases. These findings provide clinicians with long-term data on transplant recipients with end-stage renal failure due to systemic inflammatory conditions.


Assuntos
Sobrevivência de Enxerto , Granulomatose com Poliangiite/epidemiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Lúpus Eritematoso Sistêmico/epidemiologia , Nefrite Lúpica/epidemiologia , Neoplasias/epidemiologia , Adulto , Biomarcadores/sangue , Creatinina/sangue , Bases de Dados Factuais , Função Retardada do Enxerto/epidemiologia , Feminino , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/mortalidade , Humanos , Imunossupressores/efeitos adversos , Incidência , Irlanda/epidemiologia , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/mortalidade , Transplante de Rim/efeitos adversos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/mortalidade , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/tratamento farmacológico , Nefrite Lúpica/mortalidade , Masculino , Pessoa de Meia-Idade , Neoplasias/diagnóstico , Neoplasias/mortalidade , Recidiva , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
17.
Lupus ; 28(12): 1480-1487, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31558101

RESUMO

INTRODUCTION: Thrombocytopaenia and autoimmune haemolytic anaemia (AIHA) have considerable impact on prognosis in systemic lupus erythematosus (SLE). We investigated the frequencies of these haemocytopaenias, along with their associations and effect on outcome in a single-centre cohort of patients with SLE. METHODS: Demographic characteristics, clinical features, autoantibody profiles, damage and mortality data were compared between patients with and without each haematological abnormality. Variables displaying significant differences between the groups were entered into logistic regression. RESULTS: Ninety-three patients had AIHA and 215 had thrombocytopaenia. Both were associated with neuropsychiatric (NP) involvement, with each other, leucopaenia, antiphospholipid syndrome (APS) and antiphospholipid antibodies. More patients in both groups had organ damage, and their damage scores were higher. Association to NP damage was discernible. In addition, cardiovascular and renal damage and diabetes were more pronounced in patients with thrombocytopaenia. At logistic regression analysis, younger age, anticardiolipin antibody IgM positivity, leucopaenia and thrombocytopaenia were associated with AIHA whilst lupus anticoagulant activity, AIHA, leucopaenia, APS and NP involvement were associated with thrombocytopaenia. Among damage items, peripheral vascular damage, diabetes, NP damage, renal and ocular damage displayed significant associations with thrombocytopaenia, whereas none of the items did with AIHA. Patients with AIHA had significantly reduced survival rates at 10 and 20 years. CONCLUSIONS: We observed that AIHA and thrombocytopaenia were associated with severe lupus, affecting major organs and causing end organ damage. Thus, they may be considered as prognostic markers. Furthermore, AIHA and especially thrombocytopaenia may also be a marker for a subgroup of lupus patients who have or may develop APS.


Assuntos
Anemia Hemolítica Autoimune/complicações , Síndrome Antifosfolipídica/sangue , Lúpus Eritematoso Sistêmico/complicações , Insuficiência de Múltiplos Órgãos/etiologia , Trombocitopenia/complicações , Adolescente , Adulto , Anticorpos Anticardiolipina/metabolismo , Anticorpos Antifosfolipídeos/metabolismo , Síndrome Antifosfolipídica/diagnóstico , Autoanticorpos/sangue , Feminino , Humanos , Leucopenia/diagnóstico , Leucopenia/etiologia , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/mortalidade , Vasculite Associada ao Lúpus do Sistema Nervoso Central/diagnóstico , Masculino , Insuficiência de Múltiplos Órgãos/diagnóstico , Insuficiência de Múltiplos Órgãos/mortalidade , Prognóstico , Índice de Gravidade de Doença , Taxa de Sobrevida , Turquia/epidemiologia , Adulto Jovem
19.
Lupus ; 28(12): 1488-1494, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31551031

RESUMO

OBJECTIVE: To ascertain the mortality rate and causes of death in patients with systemic lupus erythematosus (SLE) within a defined region in southern Sweden during the time period 1981-2014 and determine whether these have changed over time. METHODS: In 1981, a prospective observation study of patients with SLE was initiated in southern Sweden. All incident SLE patients within a defined geographic area were identified using previously validated methods including diagnosis and immunology registers. Patients with a confirmed SLE diagnosis were then followed prospectively at the Department of Rheumatology in Lund. Clinical data was collected at regular visits. Patients were recruited from 1981 to 2006 and followed until 2014. The patient cohort was split into two groups based on the year of diagnosis to determine secular trends. Causes of death were retrieved from medical records and from the cause of death registry at The National Board of Health and Welfare in Sweden. RESULTS: In all, 175 patients were diagnosed with SLE during the study period. A total of 60 deaths occurred during a total of 3053 years of follow-up. In the first half of the study inclusion period 46 patients died, compared with 14 in the latter. The majority of patients (51.7%) died of cardiovascular disease. Infections caused 15% of the deaths and malignancy was the cause of death in 13.3% of patients. SLE was the main cause of death for 6.7% of the patients and a contributing factor for half of the patients. Standardized mortality ratio was increased in patients by a factor of 2.5 compared with the general population. Deaths occurred at an even rate throughout the whole observation period. No significant difference in standardized mortality ratio was observed between genders but was increased in older female patients. Furthermore, secular mortality trends were not identified. CONCLUSIONS: In this long-term epidemiologic follow-up study of incident SLE, we report a substantially raised mortality rate amongst SLE patients compared with the general population. The mortality rates have not changed significantly during the observation period that spanned three decades. The main cause of death was cardiovascular disease and this finding was consistent over time.


Assuntos
Doenças Cardiovasculares/mortalidade , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/mortalidade , Neoplasias/mortalidade , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Cardiovasculares/epidemiologia , Causas de Morte/tendências , Feminino , Seguimentos , Humanos , Incidência , Infecções/epidemiologia , Infecções/mortalidade , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Mortalidade , Neoplasias/epidemiologia , Estudos Prospectivos , Sistema de Registros , Taxa de Sobrevida , Suécia/epidemiologia
20.
Lupus ; 28(11): 1360-1367, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31382851

RESUMO

OBJECTIVE: The objective of this article is to investigate the distinct features, morbidity and mortality of systemic lupus erythematosus (SLE) patients registered in rheumatology tertiary centers of Shiraz University of Medical Sciences from 2008 to 2018. METHOD: A cross-sectional study was conducted on registered patients by reviewing their medical records. The clinical presentations were classified based on each organ's distinct involvement. RESULT: Data of 1322 patients with a mean age of 40 at the time of the study were reviewed. The most common primary manifestation of the disease was arthralgia (37.5%), and the most common clinical presentation in course of the disease was skin involvement (61.8%). The most common cause of hospital admission was disease flare (27.8% (108/388)), and mortality was mainly due to pulmonary complications (27.5% (8/29)). Patients who had hypothyroidism had statistically significantly lower rates of constitutional symptoms (p = 0.006), gastrointestinal involvement (p = 0.024), hypertension (p = 0.002), headache (p = 0.019), depression (p = 0.049) and anemia (p = 0.014). Patients who presented with malar rash and photosensitivity in their course of disease were detected to have statistically significantly lower ages of disease onset (p = 0.013 and 0.003, respectively). CONCLUSION: This study is an update to an epidemiologic study conducted in 2008 in the south of Iran. By comparing results between the two studies, we have tried to investigate the change in morbidity and mortality of SLE during these years.


Assuntos
Artralgia/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/fisiopatologia , Adulto , Idade de Início , Artralgia/etiologia , Estudos Transversais , Feminino , Humanos , Irã (Geográfico) , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
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