José Angelo de Souza Papi

Determinants of Risk for Venous and Arterial Thrombosis in Primary Antiphospholipid Syndrome and in Antiphospholipid Syndrome with Systemic Lupus Erythematosus

Objective. Antiphospholipid syndrome (APS) is characterized by thrombosis (venous and arterial) and pregnancy loss in conjunction with the lupus anticoagulant, IgG or IgM anticardiolipin, or IgG or IgM anti-ß2-glycoprotein I. In most series, only a minority of patients with antiphospholipid antibodies develop a clinical manifestation. Methods. A cross-sectional study of consecutive patients in the Hopkins Lupus Center was performed. Interviews were done and records were reviewed for the following variables: gender, ethnicity, hypertension, triglycerides, cholesterol, smoking, diabetes mellitus, homocysteine, cancer, hepatitis C, hormone replacement therapy/oral contraceptives, hereditary thrombophilia, anticardiolipin antibodies IgG, IgM and IgA, and lupus anticoagulant (LAC). Our aim was to identify risk factors associated with thrombosis and pregnancy loss in patients with antiphospholipid antibodies. Results. A total of 122 patients (84% female, 74% Caucasian) were studied. Patients were divided into 3 groups: primary APS, APS associated with systemic lupus erythematosus, and patients with systemic lupus erythematosus (SLE) with antiphospholipid antibodies but no thrombosis or pregnancy loss. Venous thrombosis was associated with high triglycerides (p = 0.001), hereditary thrombophilia (p = 0.02), anticardiolipin antibodies IgG > 40 (p = 0.04), and LAC (p = 0.012). Hypertriglyceridemia was associated with a 6.4-fold increase, hereditary thrombophilia with a 7.3-fold increase, and anticardiolipin IgG > 40 GPL with a 2.8-fold increase in the risk of venous thrombosis. Arterial thrombosis was associated with hypertension (p = 0.008) and elevated homocysteine (p = 0.044). Hypertension was associated with a 2.4-fold increase in the risk of arterial thrombosis. No correlations were found for pregnancy loss. Conclusion. The frequency of thrombosis and pregnancy loss is greater in APS associated with SLE than in primary APS. Risk factors differ for venous and arterial thrombosis in APS. Treatment of hypertension may be the most important intervention to reduce arterial thrombosis. Elevated triglycerides are a major associate of venous thrombosis, but the benefit of treatment is not known. Hereditary thrombophilia is an associate of venous but not arterial thrombosis, making it cost-effective to investigate only in venous thrombosis.

Cross‐cultural adaptation of the Brazilian–Portuguese version of the chronic urticaria quality‐of‐life questionnaire – CU‐Q2oL

To cite this article: Dias GAC, Pires GV, Valle SOR, França AT, Papi JA, Dortas SD Jr, Levy SAP, Baiardini I, Canonica GW. Cross‐cultural adaptation of the Brazilian‐Portuguese version of the chronic urticaria quality‐of‐life questionnaire ‐ CU‐Q2oL. Allergy 2011; 66: 1487–1493

The Prevalence of Low Bone Mineral Density in Brazilian Patients With Systemic Lupus Erythematosus and Its Relationship With the Disease Damage Index and Other Associated Factors

The aim of this study was to examine the prevalence of osteoporosis, osteopenia, and bone mineral density (BMD) less than the expected range based on age in patients with systemic lupus erythematosus (SLE) in a tropical region of Brazil and the relationship between reduced BMD and several associated factors, especially the SLE disease damage index (SDI). We scored 159 patients with creatinine clearance of 60 mL/min or more for SDI, which was modified by excluding the osteoporosis item. For postmenopausal women and men older than 50 yr, T-scores identified osteopenia (<-1.0 and >-2.5) and osteoporosis (≤-2.5). For all patients, a Z-score of -2.0 or less identified BMD less than the expected range for age. Other variables that influence BMD were studied. The prevalence of osteoporosis, osteopenia, and BMD less than the expected range for age was 28%, 54%, and 29.6%, respectively. The Z-scores were significantly lower in patients with a modified SDI ≥ 1 (mean ± standard deviation [SD]=-1.45 ± 1.18) compared with patients with a modified SDI=0 (mean ± SD=-0.94 ± 1.01; p=0.01). The lowest Z-score had a significant association with postmenopausal status (p=0.038) and significant correlations with the duration of glucocorticoid (GC) usage (p=0.033, r=-0.17), the cumulative amount of GC (p=0.000, r=-0.28), and parathyroid hormone levels (p=0.003, r=-0.24). A multiple linear regression revealed that the modified SDI (p=0.003) and the cumulative amount of GC (p=0.006) had significant independent associations with the lowest Z-score. In conclusion, a BMD less than the expected range for age occurs frequently in Brazilian patients with SLE independent of the renal failure. The patients with greater SDIs had lower Z-scores, which suggests a direct association between chronic inflammation from disease and a reduced BMD.

Depressão, ansiedade e atividade de doença na artrite reumatóide

OBJECTIVE: to evaluate the prevalence of psychiatric disorders in female patients, diagnosed with Rheumatoid Arthritis and relate this disorder to disease activity. METHODS: sectional trial study with female patients, aged 18 years old or older, diagnosed with Rheumatoid Arthritis (following American College of Rheumatology - ACR criteria), more than six months of disease and incapacity levels II and III. Disease activity was evaluated with DAS 28 (Disease Activity Score-28) (remission up to 2.6/moderate activity 2.6 to 5.1/severe degree over 5.1). Psychiatric evaluation was conducted using SCID protocol; interviews were held to diagnose the presence of depression and anxiety disorders following the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV TR). Statistical analysis included the Kruskal-Wallis test and the Kendall test with a significance level of 95%. RESULTS: In the 107 patients analyzed, 36 subjects (33.7%) presented psychiatric disorders and 71(66.4%) no psychiatric disorders). Disease activity - DAS 28 score results; remission: 6 patients (5.6%); moderate activity: 59 patients (55.1%); severe disease: 42 patients (39.2%). The relationship between psychiatric disorders and disease activity (DAS 28) demonstrated: patients without psychiatric disorders - DAS 28 score: 4.56 and patients with psychiatric disorders, DAS 28 score: 5.43 (p=0.001). CONCLUSIONS: The prevalence of depression and anxiety disorders among 107 patients with Rheumatoid Arthritis was 33.7%. Disease activity, evaluated by the DAS 28 score, was higher in the group of patients with psychiatric disorders. No patients with psychiatric disorders were identified in the group in clinical remission.

Avaliação da função endotelial em pacientes com esclerose sistêmica limitada por meio do eco Doppler da artéria braquial

OBJECTIVES The aim of this study was to compare the brachial artery endothelium-dependent and endothelium-independent dilating responses in patients with limited systemic sclerosis (LSSc) with those of healthy subjects of the same gender, age and color. METHODS Twenty adult, non-obese, non-smoker, non-diabetic, non-dyslipidemic, and non-hypertensive women, who fulfilled the American College of Rheumatology criteria for the diagnosis of SSc, were submitted to right brachial artery Doppler ultrasound. The vasodilating responses were analyzed as follows: the endothelium-dependent dilating response, after a 5-minute ischemia in the right arm; and the endothelium-independent dilating response, after administering 300 mcg of nitroglycerin (NTG) sublingually. The results were compared with the response obtained in healthy subjects. RESULTS Brachial artery longitudinal diameter was significantly low at baseline 1: 3.57 ± 0.52 mm and 3.93 ± 0.39 mm for the LSSc group and the control group, respectively, P = 0.005. The vascular reactivity after the ischemia/reactive hyperemia and the NTG showed no significant difference between the groups (8.60 ± 5.45 mm vs. 9.26 ± 5.91 mm and 25.01 ± 12.55 mm vs. 19.59 ± 7.94 mm for the LSSc and control groups, respectively). Also, no statistically significant difference was found between red blood cell velocity (RBCV) after reactive hyperemia and NTG (110.2 ± 43.86 cm/s vs. 102.0 ± 25.89 cm/s and 63.80 ± 17.69 cm/s vs. 65.4 ± 12.90 cm/s in the LSSc and control groups, respectively). CONCLUSION Although the LSSc group showed lower brachial artery diameter, the endothelium-dependent and the endothelium-independent dilating responses were preserved in both groups.

Prevalência de hepatite C em pacientes com lúpus eritematoso sistêmico do HUCCF-UFRJ

Os pacientes com lupus eritematoso sistemico (LES) apresentam fatores de risco para a aquisicao de hepatite C, como hospitalizacoes e hemotransfusoes, e compartilham com os pacientes infectados pelo virus da hepatite C (HCV) varias manifestacoes clinico-laboratoriais relacionadas a auto-imunidade. OBJETIVOS: O objetivo do presente estudo e determinar a prevalencia de hepatite C em uma populacao com LES acompanhada no ambulatorio de programas especiais - Colagenoses - do Hospital Universitario Clementino Fraga Filho (HUCFF-UFRJ). METODOS: Foi realizado um estudo de corte-transversal, incluindo os primeiros 91 pacientes com o diagnostico de LES provavel ou definitivo procedentes do referido ambulatorio, no periodo de abril a setembro de 1997. Todos os doentes foram avaliados, sob o ponto de vista clinico e laboratorial, para determinacao de atividade de doenca lupica. Foram realizados o teste de triagem UBI HCV EIA 4.0 e o teste suplementar INNO-LIA HCV AbIII nos 91 pacientes selecionados e , em 32 pacientes escolhidos ao acaso e nos pacientes reativos aos testes imunossorologicos foi realizada a pesquisa do RNA-HCV pelo metodo da reacao de polimerizacao em cadeia (PCR). RESULTADOS: Observou-se um aumento significativo da prevalencia de anti-HCV na amostra, quando comparada a populacao de doadores de sangue do HUCFF (6,6% versus 1,39% com o teste EIA, valor p=0,02 e intervalo de confianca 95%=5,5 a 13,8), contudo , considerando-se resultados obtidos com os testes confirmatorios imunoblot e PCR aplicados em serie ( prevalencia de HCV de 2,2%, com intervalo de confianca 95%=0,2 a 7,7) , a prevalencia de HCV em pacientes com LES nao parece mais alta que em doadores de sangue. CONCLUSOES: A prevalencia de hepatite C na amostra estudada utilizando-se um teste de triagem (EIA) foi significativamente maior do que a de uma populacao de doadores de sangue (6,6% versus 1,39%). Porem, aplicando-se em serie os testes confirmatorios INNO-LIAâ e PCR ( prevalencia de hepatite C de 2,2%) parece nao haver evidencias de que pacientes com LES tenham maior risco de adquirir o HCV do que a populacao em geral, embora a prevalencia tende a ser maior que a populacao de doadores de sangue. Em pacientes com diagnostico provavel de LES deve-se avaliar o custo/beneficio de se investigar HCV e crioglobulinemia mista essencial (CME).BACKGROUND Patients with systemic lupus erythematosus (SLE) are at risk for the acquisition of hepatitis C, owing to frequent hospitalization and hemotransfusion. Many clinical and laboratory manifestations related to auto-immunity are shared between Lupus and HCV infected patients. OBJECTIVE To determine the prevalence of hepatitis C in the population with SLE monitored in the out-patient service of the special programs in--Collagenoses--of the University Hospital Clementino Fraga Filho (HUCFF-UFRJ). METHODS A Cross sectional study was performed, including 91 patients with the diagnosis of probable or definitive SLE (with 3 or more of the 11 diagnostic criteria for SLE used by the American College of Rheumatology-ACR) proceeding from the mentioned center between April/97 to September/97. Patients were evaluated on clinical and laboratory basis to determine activity of lupus disease (SLEDAI). All of patients were tested with UBI HCVEIA 4.0 assay and INNO-LIAHCV AbIII confirmatory assay. PCR was performed on all EIA positive INNO-LIAHCV AbIII assay positive patients besides 32 patients negatives in both serological assays. RESULTS A significative increase in HCV prevalence among SLE patients was observed when compared with a control group of blood donors at HUCFF-UFRJ (6.6 and 1.39%, respectively; p = 0.02 and confidence interval of 95% = 5.5-13.8). However, if confirmative assays (INNO-LIA HCV ABIII and PCR) are used, this difference can not be observed any more HCV prevalence of 2.2% with confidence interval of 95% = 0.2-7.7). CONCLUSIONS HCV prevalence using ELISA assay for screening showed a significant difference between SLE patients and a blood donors control group (6.6 and 1.39% respectively). However, if confirmatory assays are subsequently applied, no difference in HCV prevalence among both groups can be observed. Patients with a possible diagnosis of SLE should have a cost/benefit analysis of testing for HCV.

Staphylococcal superantigen-specific IgE antibodies: degree of sensitization and association with severity of asthma

ABSTRACT Objective: To determine the presence of staphylococcal superantigen-specific IgE antibodies and degree of IgE-mediated sensitization, as well as whether or not those are associated with the severity of asthma in adult patients. Methods: This was a cross-sectional study involving outpatients with asthma under treatment at a tertiary care university hospital in the city of Rio de Janeiro, Brazil. Consecutive patients were divided into two groups according to the severity of asthma based on the Global Initiative for Asthma criteria: mild asthma (MA), comprising patients with mild intermittent or persistent asthma; and moderate or severe asthma (MSA). We determined the serum levels of staphylococcal toxin-specific IgE antibodies, comparing the results and performing a statistical analysis. Results: The study included 142 patients: 72 in the MA group (median age = 46 years; 59 females) and 70 in the MSA group (median age = 56 years; 60 females). In the sample as a whole, 62 patients (43.7%) presented positive results for staphylococcal toxin-specific IgE antibodies: staphylococcal enterotoxin A (SEA), in 29 (20.4%); SEB, in 35 (24.6%); SEC, in 33 (23.2%); and toxic shock syndrome toxin (TSST), in 45 (31.7%). The mean serum levels of IgE antibodies to SEA, SEB, SEC, and TSST were 0.96 U/L, 1.09 U/L, 1.21 U/L, and 1.18 U/L, respectively. There were no statistically significant differences between the two groups in terms of the qualitative or quantitative results. Conclusions: Serum IgE antibodies to SEA, SEB, SEC, and TSST were detected in 43.7% of the patients in our sample. However, neither the qualitative nor quantitative results showed a statistically significant association with the clinical severity of asthma.

Drugs or disease: evaluating salivary function in RA patients

Oral complications of RA may include temporomandibular joint disorders, mucosa alterations and symptoms of dry mouth. The aim of this study was to evaluate the salivary gland function of subjects with rheumatoid arthritis (RA) comparing it to healthy controls. Subjects with other systemic conditions known to affect salivary functions were excluded. A questionnaire was applied for the evaluation of xerostomia. Resting and chewing-stimulated salivary flow rates (SFR) were obtained under standard conditions. There were 145 subjects included of the study (104 RA and 38 controls). About 66.7% of the RA subjects and 2.4% in control group presented xerostomia. The median resting SFR were 0.24 ml/min for RA subjects and 0.40 mL/min for controls (p = 0.04). The median stimulated SFR were 1.31 mL/min for RA subjects and 1.52 ml/min for controls (p = 0.33). No significant differences were found between resting and stimulated SFR of RA subjects not using xerogenic medications and controls. There was significantly higher number of subjects presenting hyposalivation in the RA group than among controls, even when subjects using xerogenic medications were eliminated from the analysis. In conclusion, hyposalivation and xerostomia were more frequent among RA subjects not using xerogenic medication than among controls, although there were no significant differences in the median SFR between groups.

Staphylococcal superantigen-specific IgE antibodies and its correlation with asthma severity

Background Elevated IgE antibodies to Staphylococcal toxins (superantigens) are related to asthma severity in a few studies. We have investigated this hypothesis in an adult asthmatic population from the Clinical Immunology outpatient service of an University Hospital. Our objectives were: to detect the presence and the degree of IgEmediated sensitization to staphylococcal toxins in vitro in asthma patients; to correlate the presence and concentration of specific IgE against staphylococcal toxins with asthma severity; and to assess whether elevated levels of serum IgE specific for staphylococcal toxins may have predictive value for asthma severity.

Prevalence of autoantibodies in a group of hereditary angioedema patients

Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB) is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patients with Hereditary Angioedema attended at Clementino Fraga Filho University Hospital accepted to participate in this study. Prevalence of AAB was 40%. Our data indicate high prevalence of AAB in patients with Hereditary Angioedema. Large-scale studies should be considered to determine the significance of these AAB in the follow-up care of patients with Hereditary Angioedema.