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1.
J Rheumatol ; 2020 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-33259331

RESUMO

OBJECTIVE: Lupus nephritis (LN) may lead to end-stage kidney disease (ESKD) in 22% of patients over 15 years with the risk being particularly higher in diffuse proliferative forms. The rate of kidney function decline varies. However, a catastrophic course leading to ESKD within a few years from onset is uncommon. The aim of the present study was to assess the factors associated with rapid progression to ESKD in LN patients. METHODS: Patients from the Toronto Lupus Clinic with biopsy-proven LN at presentation and eGFR≥60ml/min/1.73m2 who developed ESKD within three years were retrieved. Pathology reports were reviewed with particular emphasis on distinct histopathologic features. Demographic, clinical, laboratory and therapeutic variables were also analyzed. RESULTS: Ten patients (1.8% of the total LN population) developed ESKD within three years of diagnosis. Their mean age was 34.2±7.3 years, mean time to ESKD 19.2±12.4 months, initial eGFR=90.2±24.9ml/min/1.73m2, proteinuria 2.7±1.04 grams/24h. The rate of kidney function decline was more than 43ml/min/1.73m2/year (median). One patient had LN class III, five had LN class IV, two membranous LN (class V) and another two had mixed IV/V. Moreover, two patients had extensive thrombotic microangiopathy, one collapsing glomerulonephritis and one concomitant anti-glomerular basement membrane (anti-GBM) nephropathy. Four patients showed no unusual kidney pathology; all of them had severe non-compliance (discontinued all medications to follow alternative treatment). CONCLUSION: Catastrophic progression to ESKD is uncommon in LN. The major associated factors are poor compliance and distinct histopathologic features such as thrombotic microangiopathy, collapsing glomerulopathy and concomitant anti-GBM nephropathy.

2.
Artigo em Inglês | MEDLINE | ID: mdl-33140092

RESUMO

OBJECTIVES: Most randomized controlled trials (RCTs) in SLE have failed to reach their respective end points, with the rates of response to placebo (plus standard-of-care treatment) being unexpectedly high. The aim of this systematic review was to quantify the response to placebo in non-renal, non-neuropsychiatric lupus. METHODS: The PubMed database was searched (from 2000 to December 2019) for phase II/III RCTs assessing the efficacy and safety of biologics in non-renal, non-neuropsychiatric SLE. Data on the efficacy and safety of the placebo-treated patients were collected in a pre-established data retrieval form. Descriptive statistics were used. RESULTS: A total of 24 RCTs (n = 11 128 in total) were included. Placebo-treated patients (n = 3899) were mostly females (93.5%), Caucasians (60.2%), of mean age 39.7 years, and having a mean disease duration of 7.4 years. Their mean initial SLEDAI 2000 was 10.4, whereas 60.5% had positive anti-dsDNA antibodies, 41.9% low C3 and 35.6% low C4 at randomization. Standard-of-care treatment included glucocorticosteroids in 85.9%, antimalarials in 72.8% and immunosuppressives in 48.5%. The response to placebo was 36.2% for the primary end point (as defined in each study), 39.8% for the SLE Responder Index-4 (SRI-4), 29.2% for SRI-5, 28.4% for SRI-6 and 30.9% for BILAG-based Combined Lupus Assessment response. Regarding safety, there were serious adverse events in 16.3% of patients, serious infections in 5.5% and malignancies in 0.3%, and death occurred in 0.56% of patients. CONCLUSION: More than one-third of the placebo-treated patients achieved their respective primary end points in RCTs with biologics in non-renal, non-neuropsychiatric SLE. The response rate was higher for certain end points, such as the SRI-4, while it decreased with more stringent end points.

3.
Eur J Rheumatol ; 7(Suppl 2): S110-S116, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32432527

RESUMO

Hydroxychloroquine (HCQ) has sparked much interest in the therapeutics of the Coronavirus Disease 2019 (COVID-19) pandemic. Its antiviral properties have been studied for years; regarding the Severe Acute Respiratory Syndrome-Corona Virus-2 (SARS-CoV-2), it has been shown that HCQ may act at multiple levels. These extend from the initial attachment of the virus to the respiratory epithelium to the inhibition of its replication by the alkalinisation of the phagolysosome's microenvironment and the post-translational modification of certain viral proteins. Preliminary clinical evidence from China and France showed significant virological and clinical benefit in HCQ-treated patients, while other studies, mostly including critically ill patients, did not show favorable results. In this review, we critically appraise the existing evidence on HCQ against SARS-CoV-2 with particular emphasis on its protective and therapeutic role. Safety concerns that are relevant to the short-term HCQ use are also discussed. In the context of the rapid evolution of the COVID-19 pandemic that strains the health care systems worldwide and considering limited population-wide testing rates in most of the vulnerable countries, early empiric short-term administration of HCQ in symptomatic individuals, may be a promising, safe and low-cost strategy.

4.
J Rheumatol ; 47(9): 1366-1373, 2020 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-32238519

RESUMO

OBJECTIVE: Advanced chronic kidney disease (CKD) carries an increased risk for progression to endstage renal disease (ESRD). We aimed to determine the rate of progression and the factors that drive the decline of renal function in lupus nephritis (LN). METHODS: Patients with advanced LN-related CKD were identified from our longterm longitudinal cohort. Advanced CKD was defined as stage 3b [estimated glomerular filtration rate (eGFR) = 30-44 ml/min/1.73 m2] and stage 4 (eGFR = 15-29 ml/min/1.73 m2). All individuals were followed until progression to ESRD or the last visit and were divided into "progressors" and "non- progressors." Demographic, clinical, immunological, and therapeutic variables were compared at baseline. Multivariable Cox regression analysis (both time-dependent and independent) was performed to identify predictors for progression. RESULTS: One hundred eighteen patients (74 CKD 3b and 44 CKD 4) were included. Forty-five patients progressed (29 to ESRD and 16 from CKD 3b to CKD 4) after 6 years on average. No significant decline in the renal function was observed in 73 patients ("non-progressors") after 10 years on average. Active serology (high anti-dsDNA titers and low complements C3/C4) at the time of CKD diagnosis and any increase of the daily prednisone dose after baseline were strongly associated with progression. Treatment with renin angiotensin system (RAS) blockers was associated with less risk for progression. CONCLUSION: Dialysis is not inevitable in LN-related advanced CKD because 62% of our patients did not progress over 10 years of followup on average. Certain predictors were identified to affect progression to ESRD.

5.
Ann Rheum Dis ; 79(5): 612-617, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32156706

RESUMO

BACKGROUND: The 2017 American College of Cardiology/American Heart Association guidelines defined hypertension at ≥130/80 mm Hg. Studies on patients with connective tissue diseases were not considered. Our aim was to assess the impact of this definition on atherosclerotic vascular events (AVEs) in systemic lupus erythematosus. PATIENTS METHODS: Individuals from the Toronto Lupus Clinic with at least 2 years of follow-up and no prior AVE were divided in three groups according to their mean blood pressure (BP) over that period (≥140/90 mm Hg, 130-139/80-89 mm Hg and <130/80 mm Hg). They were followed until the first occurrence of an AVE (fatal or non-fatal coronary artery disease, cerebrovascular event and peripheral vascular disease) or last visit. Groups were compared as per the baseline atherosclerotic risk factors. A multivariable time-dependent analysis was performed to adjust for the presence of other risk factors. RESULTS: Of 1532 patients satisfying the inclusion criteria, 155 (10.1%) had a BP ≥140/90 mm Hg, 316 (20.6%) 130-139/80-89 mm Hg and 1061 (69.3%) were normotensives. After a mean follow-up of 10.8 years, 124 AVEs were documented. The incidence rates were 18.9, 11.5 and 4.5 per 1000 patient-years for the three groups, respectively (p=0.0007 between the 130-139/80-89 mm Hg group and the normotensives). A mean BP of 130-139/80-89 mm Hg over the first 2 years was independently associated with the occurrence of AVEs (HR 1.73, 95% CI 1.13 to 2.65, p=0.011). CONCLUSION: Patients with lupus with a sustained mean BP of 130-139/80-89 mm Hg over 2 years had a significantly higher incidence of AVEs compared with normotensive individuals. This BP level should be the target for antihypertensive therapy to minimise their cardiovascular risk.


Assuntos
Doença da Artéria Coronariana/epidemiologia , Hipertensão/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Infarto do Miocárdio/epidemiologia , American Heart Association , Análise de Variância , Anti-Hipertensivos/uso terapêutico , Cardiologia/normas , Estudos de Coortes , Doença da Artéria Coronariana/diagnóstico , Feminino , Hospitais Universitários , Humanos , Hipertensão/classificação , Hipertensão/tratamento farmacológico , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Infarto do Miocárdio/diagnóstico , Ontário , Prevalência , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Sociedades Médicas , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/epidemiologia , Análise de Sobrevida , Estados Unidos
7.
Ann Rheum Dis ; 78(6): 802-806, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30992296

RESUMO

BACKGROUND: Survival in systemic lupus erythematosus (SLE) has improved substantially in the last 50 years. The aim of the present study was to assess the evolution of the all-cause, cause-specific and age-specific standardised mortality ratios (SMRs) of patients with lupus in Ontario, Canada. PATIENTS AND METHODS: Between 1971 and 2013, 1732 patients were followed in the Toronto Lupus Clinic. Causes of death were retrieved from death certificates, autopsy reports, hospital records or the records of the family physicians. They were categorised as atherosclerotic, infectious, malignancy, active lupus and others. For the calculation of the SMR (indirect standardisation method), data from the general population of Ontario, Canada were used (Statistics Canada). RESULTS: Two hundred and forty-nine patients (205 women) died (infections 24.5%, atherosclerosis 15.7%, active lupus 13.3%, malignancy 9.6%); mean age was 53.2±16.6 years and mean disease duration 15.2±11.7 years. The all-cause SMR was substantially decreased from the 1970s (13.5, 95% CI 8.6 to 18.5) to recent years (2.2, 95% CI 1.4 to 3.1). Similar trends were observed for atherosclerosis, infections and malignancies over time. The all-cause age-specific SMR was particularly high in younger (19-39 years old) patients (SMR=12.4, 95% CI 9.7 to 15.1) as compared with individuals older than 40 years (SMR=3.1, 95% CI 2.6 to 3.6). The cause-specific SMR was also higher in younger patients, particularly for infections and malignancies. CONCLUSIONS: The all-cause and cause-specific SMR significantly decreased over time, likely reflecting the advances in the management of SLE and certain comorbidities. The all-cause and cause-specific SMR was particularly high for younger patients (<40 years old).


Assuntos
Lúpus Eritematoso Sistêmico/mortalidade , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Aterosclerose/complicações , Aterosclerose/mortalidade , Causas de Morte , Atestado de Óbito , Feminino , Humanos , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/complicações , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Neoplasias/complicações , Neoplasias/mortalidade , Ontário/epidemiologia , Infecções Oportunistas/complicações , Infecções Oportunistas/mortalidade , Adulto Jovem
8.
J Rheumatol ; 46(4): 391-396, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30323009

RESUMO

OBJECTIVE: Antimalarials (AM) are recommended for all systemic lupus erythematosus (SLE) patients without specific contraindications. Their main adverse effect is retinal damage; however, heart disease has been described in isolated cases. The aim of our study is to describe 8 patients with AM-induced cardiomyopathy (AMIC) in a defined SLE cohort. METHODS: Patients attending the Toronto Lupus Clinic and diagnosed with definite (based on endomyocardial biopsy; EMB) and possible AMIC were included [based on cardiac magnetic resonance imaging (cMRI) and other investigations]. RESULTS: Eight female patients (median age 62.5 yrs, disease duration 35 yrs, AM use duration 22 yrs) were diagnosed with AMIC in the past 2 years. Diagnosis was based on EMB in 3 (extensive cardiomyocyte vacuolation, intracytoplasmic myelinoid, and curvilinear bodies). In 4 patients, cMRI was highly suggestive of AMIC (ventricular hypertrophy and/or atrial enlargement and late gadolinium enhancement in a nonvascular pattern). Another patient was diagnosed with complete atrioventricular block, left ventricular and septal hypertrophy, along with concomitant ocular toxicity. All patients had abnormal cardiac troponin I (cTnI) and brain natriuretic peptide (BNP), whereas 7/8 also had chronically elevated creatine phosphokinase. During followup, 1 patient died from refractory heart failure. In the remaining patients, hypertrophy regression and a steady decrease of heart biomarkers were observed after AM cessation. CONCLUSION: Once considered extremely rare, AMIC seems to be underrecognized, probably because of the false attribution of heart failure or hypertrophy to other causes. Certain biomarkers (cTnI, BNP) and imaging findings may lead to early diagnosis and enhance survival.


Assuntos
Antimaláricos/efeitos adversos , Antimaláricos/uso terapêutico , Cardiomiopatias/induzido quimicamente , Cardiomiopatias/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Doenças Raras/induzido quimicamente , Doenças Raras/diagnóstico por imagem , Idoso , Biomarcadores/sangue , Biópsia , Canadá , Cardiomiopatias/patologia , Feminino , Seguimentos , Humanos , Imagem por Ressonância Magnética/métodos , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/sangue , Doenças Raras/patologia , Troponina I/sangue , Suspensão de Tratamento
9.
Clin Rheumatol ; 38(1): 269, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30430267

RESUMO

Prof. Ari Polachek on of the author of the published version of this article missed to add his second affiliation which is the Department of Rheumatology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. The new affiliation is now added and presented correctly in this article.

10.
Arthritis Care Res (Hoboken) ; 71(6): 822-828, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30055090

RESUMO

OBJECTIVE: A recent study conducted by our clinical group demonstrated that low disease activity (LDA), defined as a clinical Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score ≤2, maintained for 12 months, confers the same risk for damage accrual as complete remission after 2 years. The aim of the present study was to assess the validity of these findings in the long term (10 years). METHODS: Patients in the inception cohort of the Toronto Lupus Clinic, who had a minimum follow-up of 10 years and no interval >18 months between consecutive visits, were included in the analysis. Prolonged clinical remission was defined based on a SLEDAI-2K score of 0 (serology excluded), achieved within the first 5 years of enrollment and maintained for ≥10 years. Prolonged LDA was defined as SLEDAI-2K score ≤2 (serology excluded) with the same time frame restrictions. RESULTS: Of 267 patients, 27 (10.1%) achieved prolonged clinical remission and 48 (18%) attained prolonged LDA. There were no differences regarding demographic, clinical, and immunologic variables at any time. The mean prednisone dose at enrollment was higher in patients in whom remission was achieved, while patients with prolonged LDA were taking antimalarials more frequently, both at enrollment and after the 10-year time period. Cumulative damage and flare rate after 10 years, and mortality throughout follow-up were comparable. SLE in patients in the prolonged LDA group was in complete remission for 76% of the follow-up time. CONCLUSION: Prolonged clinical remission and LDA were demonstrated in 10.1% and 18% of our patients, respectively, and comparable outcomes were demonstrated in the long term, rendering sustained LDA an acceptable treat-to-target outcome in SLE.


Assuntos
Antimaláricos/uso terapêutico , Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adulto , Antimaláricos/efeitos adversos , Bases de Dados Factuais , Feminino , Glucocorticoides/efeitos adversos , Humanos , Imunossupressores/efeitos adversos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Ontário , Indução de Remissão , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
11.
J Rheumatol ; 46(1): 64-69, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30068764

RESUMO

OBJECTIVE: Cardiac involvement in systemic lupus erythematosus (SLE) is often undiagnosed in its early phases. Specific heart biomarkers may identify patients at risk. We sought to investigate the prevalence and associated factors for such biomarkers in SLE. METHODS: Brain natriuretic peptide (BNP) and cardiac troponin I (cTnI) were measured simultaneously in 151 consecutive patients with no history of heart disease or pulmonary arterial hypertension (PAH). None had electrocardiographic abnormalities suggestive of acute coronary syndrome. Cross-sectional comparisons and logistic regression analyses were performed. Patients with abnormal biomarkers were investigated to delineate the specific cause. RESULTS: Sixteen patients (16/151, 10.6%) had elevated BNP, and 9 of them also had abnormal cTnI. Compared to subjects with normal biomarkers, they were older, had longer disease and antimalarial (AM) use duration, and more frequently persistent creatine phosphokinase (CPK) elevation. Multivariable regression analysis showed prolonged AM treatment (> 5.6 yrs) and persistent CPK elevation to be important predictors for elevated cardiac biomarkers. Six patients were diagnosed with definite (based on endomyocardial biopsy, n = 2) or possible (based on cardiac magnetic resonance after exclusion of other causes) AM-induced cardiomyopathy (AMIC); all had both BNP and cTnI elevated. Alternative causes were identified in 5, while no definitive diagnosis could be made in the remaining patients. CONCLUSION: About 10% of patients with SLE had elevated myocardial biomarkers, in the absence of prior cardiac disease or PAH. One-third of them were diagnosed with AMIC. Prolonged AM therapy and persistent CPK elevation conferred an increased risk for abnormal BNP and cTnI, which might predict AMIC.


Assuntos
Antimaláricos/efeitos adversos , Cardiomiopatias/diagnóstico por imagem , Lúpus Eritematoso Sistêmico/sangue , Peptídeo Natriurético Encefálico/sangue , Troponina I/sangue , Adulto , Idoso , Antimaláricos/uso terapêutico , Biomarcadores/sangue , Cardiomiopatias/induzido quimicamente , Creatina Quinase/sangue , Estudos Transversais , Feminino , Humanos , Malária/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Fatores de Risco
12.
Semin Arthritis Rheum ; 48(6): 1035-1040, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30415943

RESUMO

BACKGROUND: In recent years, low disease activity emerged as a state that is associated with improved long-term outcomes in systemic lupus erythematosus (SLE). Our aim was to review the current concepts for low disease activity in SLE in order to serve as the basis of a future consensus for standardization. METHODS: The PubMed database was searched for relevant articles from inception up to July 2018. Medical Subject Headings (MeSH terms) included "lupus" AND "low disease activity" OR "minimal disease activity". RESULTS: Three different definitions of low disease activity in lupus have been proposed. Minimal disease activity (MDA) is defined as a clinical SLE Disease Activity Index 2000 (SLEDAI-2K)≤1 on antimalarials, immunosuppressives in standard doses and prednisone ≤5 mg/day. Low disease activity (LDA) allows for a clinical SLEDAI-2K≤2 maintained on antimalarials only. Lupus Low Disease Activity State (LLDAS) accepts a SLEDAI-2K≤4 with no activity from major organ systems, a Physician's Global Assessment of ≤1 with no new activity, prednisone dose ≤7.5 mg/day and standard doses of antimalarials, immunosuppressives and biologics. Active serology (anti-dsDNA and complement C3/C4) is not included in the MDA and LDA but counts towards disease activity in the LLDAS definition. All definitions were associated with less damage-accrual and mortality in the long-term that were comparable to those of clinical remission. CONCLUSIONS: There is solid evidence that low disease activity is associated with improved outcomes in SLE and could serve as a therapeutic target in daily practice and clinical trials. Future research should focus on advancing a consensus for the best possible definition.


Assuntos
Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/diagnóstico , Prednisona/uso terapêutico , Progressão da Doença , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Indução de Remissão , Índice de Gravidade de Doença
13.
J Rheumatol ; 45(10): 1426-1439, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30173152

RESUMO

OBJECTIVE: To develop recommendations for the assessment of people with systemic lupus erythematosus (SLE) in Canada. METHODS: Recommendations were developed using the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) approach. The Canadian SLE Working Group (panel of Canadian rheumatologists and a patient representative from Canadian Arthritis Patient Alliance) was created. Questions for recommendation development were identified based on the results of a previous survey of SLE practice patterns of members of the Canadian Rheumatology Association. Systematic literature reviews of randomized trials and observational studies were conducted. Evidence to Decision tables were prepared and presented to the panel at 2 face-to-face meetings and online. RESULTS: There are 15 recommendations for assessing and monitoring SLE, with varying applicability to adult and pediatric patients. Three recommendations focus on diagnosis, disease activity, and damage assessment, suggesting the use of a validated disease activity score per visit and annual damage score. Strong recommendations were made for cardiovascular risk assessment and measuring anti-Ro and anti-La antibodies in the peripartum period and conditional recommendations for osteoporosis and osteonecrosis. Two conditional recommendations were made for peripartum assessments, 1 for cervical cancer screening and 2 for hepatitis B and C screening. A strong recommendation was made for annual influenza vaccination. CONCLUSION: These are considered the first guidelines using the GRADE method for the monitoring of SLE. Existing evidence is largely of low to moderate quality, resulting in more conditional than strong recommendations. Additional rigorous studies and special attention to pediatric SLE populations and patient preferences are needed.


Assuntos
Diretrizes para o Planejamento em Saúde , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Programas de Rastreamento , Adulto , Canadá , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/etiologia , Criança , Feminino , Pessoal de Saúde , Hepatite C/diagnóstico , Hepatite C/etiologia , Humanos , Infecções/diagnóstico , Infecções/etiologia , Lúpus Eritematoso Sistêmico/complicações , Masculino , Osteonecrose/diagnóstico , Osteonecrose/etiologia , Osteoporose/diagnóstico , Osteoporose/etiologia , Período Periparto/sangue , Gravidez , Reumatologistas , Medição de Risco , Índice de Gravidade de Doença , Revisões Sistemáticas como Assunto , Neoplasias do Colo do Útero/diagnóstico , Neoplasias do Colo do Útero/etiologia , Vacinação
14.
J Rheumatol ; 45(8): 1131-1135, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29858240

RESUMO

OBJECTIVE: Disease course in systemic lupus erythematosus (SLE) is primarily relapsing-remitting. Long quiescent and chronically active patterns are less frequent. We recently described an atypical "monophasic" course in a small number of patients. The aim of the present study was to assess the prevalence and characteristics of such patients in a defined SLE cohort. METHODS: The inception patients of the University of Toronto Lupus Clinic (enrolled within 18 mos of diagnosis) were investigated. No time interval > 18 months was allowed between consecutive visits. A monophasic course was defined as Systemic Lupus Erythematosus Disease Activity Index 2000 = 0 (serology excluded), achieved within 5 years since enrollment and maintained for ≥ 10 years. Descriptive statistics were used. RESULTS: Of 267 inception patients, 27 (10.1%) achieved prolonged clinical remission (≥ 10 yrs) and 20 (7.5%) sustained remission for the entire followup (18 yrs on average). Twelve patients were receiving no maintenance treatment 10 years after achieving remission. Clinical manifestations at diagnosis (apart from skin and musculoskeletal involvement) included 25% in each of central nervous system involvement and lupus nephritis (LN). Half the patients were serologically active. Ten years after achieving remission, two-thirds of the patients had discontinued glucocorticosteroids; the remaining were treated with 5 mg/day on average. Seven patients relapsed after 10 years, 4 with arthritis, 2 LN, and 1 catastrophic antiphospholipid syndrome. CONCLUSION: A monophasic disease course was observed in 7.5% in this inception cohort. Patients sustained remission for 18 years on average, eventually without medications. Further study of such patients may provide unique pathophysiologic insights for SLE.


Assuntos
Progressão da Doença , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Indução de Remissão , Adulto , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Recidiva , Índice de Gravidade de Doença , Adulto Jovem
16.
J Rheumatol ; 44(12): 1841-1849, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29093154

RESUMO

OBJECTIVE: We previously reported the effect of certain factors on cardiovascular disease (CVD) in 250 women with systemic lupus erythematosus (SLE) followed for 8 years. The aim of this study was to delineate their evolution after 15 years of followup. METHODS: There were 210 women with SLE and 138 age-matched healthy women available for analysis after 15 years. Cardiovascular events (CVE) included angina pectoris, myocardial infarction (fatal and nonfatal), transient ischemic attack, and stroke (fatal and nonfatal). Analysis was performed with SAS 9.3 software; p < 0.05 was considered significant. RESULTS: CVE occurred in 41/210 patients (19.5%) and 9/138 controls (6.5%), most of them in the second part (2008-2015) of the study (24/210, 11.4% vs 17/241, 7.1% in SLE group). Coronary artery disease was more common in patients (32/210, 15.2% vs 5/138, 3.6%, p = 0.0041). There was no significant difference for cerebrovascular disease (10/210, 4.8% vs 3/138, 2.2%, p = 0.213). SLE was the most prominent CVE predictor in the first 8 years (HR 2.8, 95% CI 1.3-6.3). Hypertension and diabetes were more frequent in patients who developed CVE during the second half of the study. Thirty-one deaths occurred in patients with SLE (10 because of CVD) and 6 in controls (none because of CVD). CONCLUSION: The relative importance of atherosclerotic risk factors is significantly differentiated over time in SLE. Disease-related factors seem to dominate CV risk during the early stages while traditional factors, partially related to corticosteroid treatment, play a significant role later in the disease course.


Assuntos
Angina Pectoris/etiologia , Aterosclerose/etiologia , Ataque Isquêmico Transitório/etiologia , Lúpus Eritematoso Sistêmico/complicações , Infarto do Miocárdio/etiologia , Acidente Vascular Cerebral/etiologia , Adulto , Angina Pectoris/mortalidade , Aterosclerose/mortalidade , Feminino , Humanos , Ataque Isquêmico Transitório/mortalidade , Lúpus Eritematoso Sistêmico/mortalidade , Pessoa de Meia-Idade , Infarto do Miocárdio/mortalidade , Prognóstico , Estudos Prospectivos , Fatores de Risco , Acidente Vascular Cerebral/mortalidade , Taxa de Sobrevida
18.
Curr Rheumatol Rev ; 13(3): 206-218, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28675998

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) is characterized by various clinical manifestations and immunologic abnormalities. Cardiovascular and respiratory system involvement are increasingly recognized as critical for patients' prognosis. In this review, current knowledge concerning diagnosis, pathogenesis and treatment of the cardiac and pulmonary lupus manifestations are discussed. METHOD: Review of the literature. RESULTS: Although pericarditis is the most frequent heart manifestation in the context of lupus, valvular disease and less often myocarditis may be detected. In the latter, treatment should be prompt and aggressive to prevent chronic sequelae like congestive heart failure. Later on disease course, accelerated atherosclerosis is considered as one of the most important co-morbidities of SLE with cardiovascular events being one of the leading causes of death at relatively young ages. Stratification of the patients at risk and stringent management of the traditional risk factors are warranted. Respiratory system involvement affects all anatomic structures of the lungs, pleura and pulmonary vasculature while its severity ranges from asymptomatic pleural disease to acute respiratory failure. The most common features include pleuritis, interstitial lung disease and pulmonary embolism on the background of antiphospholipid syndrome. Less usual complications include lupus pneumonitis, diffuse alveolar hemorrhage, shrinking lung syndrome and pulmonary arterial hypertension. CONCLUSION: There are no specific guidelines for the management of these manifestations and therapeutic approach remains empiric.


Assuntos
Doenças Cardiovasculares/etiologia , Pneumopatias/etiologia , Lúpus Eritematoso Sistêmico/complicações , Doenças Cardiovasculares/patologia , Feminino , Humanos , Pneumopatias/patologia , Masculino
19.
Clin Rheumatol ; 36(4): 879-884, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28168661

RESUMO

The coexistence of psoriasis with systemic lupus erythematosus (SLE) has been reported in limited case series, raising hypotheses about shared pathogenetic mechanisms. Nevertheless, important differences regarding treatment do exist. The aim of the present study was to determine the prevalence and characteristics of psoriasis in a defined cohort of lupus patients. Patients with psoriasis were retrieved from the University of Toronto Lupus Clinic from its inception in 1970 up to 2015. Charts were hand-searched to collect information concerning demographic, clinical, and therapeutic variables. Patients were matched with non-psoriasis lupus patients to identify the impact of supervening psoriasis on lupus activity, damage accrual, and venous thromboembolic (VTEs) and cardiovascular events (CVEs). Psoriasis was diagnosed in 63 patients (49 females, 14 males) for a prevalence of 3.46% (63/1823). The male-to-female ratio was significantly higher in non-psoriasis patients (0.286 vs. 0.138, p = 0.017). Plaque psoriasis was the most prominent type (55/63, 87.3%) whereas three patients had pustular disease; one had psoriatic arthritis. Nine patients (14.3%) were administered systemic treatment with methotrexate (n = 5), azathioprine (n = 1), ustekinumab (n = 3), and etanercept (n = 1). Psoriasis was definitely deteriorated by hydroxychloroquine in one patient. There was no significant impact of psoriasis on disease activity, damage accrual, VTEs, and CVEs. The prevalence of psoriasis was twice as high as that of the general Canadian population in this lupus cohort. Plaque psoriasis was the most prominent subtype, and topical treatment was adequate in the majority of patients. Supervening psoriasis had no significant impact on lupus activity and damage accrual.


Assuntos
Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Psoríase/tratamento farmacológico , Psoríase/epidemiologia , Adolescente , Adulto , Antirreumáticos/uso terapêutico , Azatioprina/uso terapêutico , Estudos de Coortes , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Masculino , Metotrexato/uso terapêutico , Ontário , Adulto Jovem
20.
Open Access Rheumatol ; 9: 1-9, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28053559

RESUMO

Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud's phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

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