RESUMO
Epidemiological studies with systemic lupus erythematosus (SLE) patients have been reported worldwide but, until now, a large evaluation had not been performed in Brazil. Therefore, we determined the clinical and immunological features of 888 SLE patients followed at our service from 2008 to 2012. The mean age at SLE onset and the mean disease duration were 29.9 ± 9.5 years old and 14.5 ± 8.4 years, respectively. A predominance of female gender (91.9%) and Caucasian ethnicity (69.9%) were observed. Cumulative mucocutaneous manifestations (90.7%) were most commonly identified (malar rash (83.2%), photosensitivity (76.9%)) followed by articular (87.4%), hematological (44.0%) and renal (36.9%) involvements. Antinuclear antibody was detected in all patients, followed by anti-dsDNA (35.1%), anti-Sm (21.8%) and anti-ribosomal P protein antibodies (19.8%). Additional comparison of clinical and laboratory features between genders revealed that malar rash was observed more in female SLE patients (84.5% vs. 69.4%, p = 0.001). Male lupus patients presented a higher frequency of anti-dsDNA (45.8% vs. 34.2%, p = 0.047) and a trend of more nephritis (47.2% vs. 36.0%, p = 0.059). In conclusion, we identified a high prevalence of mucocutaneous manifestations in this Brazilian SLE cohort compared to other countries, mainly malar rash that was most commonly observed in female patients. Anti-dsDNA and other specific SLE autoantibodies were also identified in a higher frequency, predominantly in the male gender.
Assuntos
Anticorpos Antinucleares/imunologia , Lúpus Eritematoso Cutâneo/epidemiologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Nefrite Lúpica/epidemiologia , Adulto , Idade de Início , Brasil/epidemiologia , DNA/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Prevalência , Fatores Sexuais , Adulto JovemRESUMO
We compared outcomes of alveolar hemorrhage (AH) in juvenile (JSLE) and adult onset SLE (ASLE). From 263 JSLE and 1522 ASLE, the AH occurred in 13 (4.9%) and 15 (1.0%) patients, respectively (p < .001). Both groups had comparable disease duration (2.6 ± 3.0 vs. 5.6 ± 7.0 years, p = .151) and median SLEDAI scores [17.5 (2 to 32) vs. 17.5 (3 to 28), p = 1.000]. At AH onset, a higher frequency of JSLE were already on a high prednisone dose ( > 0.5 mg/kg/day) compared to ASLE (54% vs. 15%, p = .042). The mean drop of hemoglobin was significantly lower in JSLE (2.9 ± 0.9 vs. 5.5 ± 2.9 g/dL, p = .006). Although treatments with methylprednisolone, plasmapheresis, intravenous immunoglobulin and cyclophosphamide were similar in both groups (p > .050), regarding outcomes, there was a trend in high frequency of mechanical ventilation use (85% vs. 47%, p = .055) and also significant mortality (69% vs. 13%, p = .006) in JSLE compared to ASLE. The sepsis frequency was comparable in both groups (50% vs. 27%, p = .433). We have identified that AH in JSLE has a worse outcome most likely related to respiratory failure. The AH onset in JSLE already treated with high-dose steroids raises the concern of inadequate response to this treatment and reinforces the recommendation of early aggressive alternative therapies in this group of patients.
Assuntos
Hemorragia/etiologia , Pneumopatias/etiologia , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/mortalidade , Alvéolos Pulmonares , Adolescente , Adulto , Fatores Etários , Anti-Inflamatórios/uso terapêutico , Criança , Dispneia/etiologia , Feminino , Hemoglobinas/metabolismo , Hemoptise/etiologia , Hemorragia/sangue , Hemorragia/tratamento farmacológico , Humanos , Hipóxia/etiologia , Pneumopatias/sangue , Pneumopatias/tratamento farmacológico , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/complicações , Síndrome de Ativação Macrofágica/etiologia , Masculino , Metilprednisolona/uso terapêutico , Prednisona/uso terapêutico , Respiração Artificial , Estudos Retrospectivos , Sepse/etiologia , Estatísticas não Paramétricas , Adulto JovemRESUMO
Our objective was to compare the pattern of organ dysfunctions and outcomes of critically ill patients with systemic lupus erythematosus (SLE) with patients with other systemic rheumatic diseases (SRD). We studied 116 critically ill SRD patients, 59 SLE and 57 other-SRD patients. The SLE group was younger and included more women. Respiratory failure (61 percent) and shock (39 percent) were the most common causes of ICU admission for other-SRD and SLE groups, respectively. ICU length-of-stay was similar for the two groups. The 60-day survival adjusted for the groups’ baseline imbalances was not different (P = 0.792). Total SOFA scores were equal for the two groups at admission and during ICU stay, although respiratory function was worse in the other-SRD group at admission and renal and hematological functions were worse in the SLE group at admission. The incidence of severe respiratory dysfunction (respiratory SOFA >2) at admission was higher in the other-SRD group, whereas severe hematological dysfunction (hematological SOFA >2) during ICU stay was higher in the SLE group. SLE patients were younger and displayed a decreased incidence of respiratory failure compared to patients with other-SRDs. However, the incidences of renal and hematological failure and the presence of shock at admission were higher in the SLE group. The 60-day survival rates were similar.
Assuntos
Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Hematológicas/epidemiologia , Falência Renal Crônica/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Insuficiência de Múltiplos Órgãos/mortalidade , Transtornos Respiratórios/epidemiologia , Doenças Reumáticas/complicações , Estado Terminal , Métodos Epidemiológicos , Doenças Hematológicas/etiologia , Hospitalização/estatística & dados numéricos , Unidades de Terapia Intensiva , Falência Renal Crônica/etiologia , Tempo de Internação/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/mortalidade , Transtornos Respiratórios/etiologia , Doenças Reumáticas/classificação , Doenças Reumáticas/mortalidadeRESUMO
Our objective was to compare the pattern of organ dysfunctions and outcomes of critically ill patients with systemic lupus erythematosus (SLE) with patients with other systemic rheumatic diseases (SRD). We studied 116 critically ill SRD patients, 59 SLE and 57 other-SRD patients. The SLE group was younger and included more women. Respiratory failure (61%) and shock (39%) were the most common causes of ICU admission for other-SRD and SLE groups, respectively. ICU length-of-stay was similar for the two groups. The 60-day survival adjusted for the groups' baseline imbalances was not different (P = 0.792). Total SOFA scores were equal for the two groups at admission and during ICU stay, although respiratory function was worse in the other-SRD group at admission and renal and hematological functions were worse in the SLE group at admission. The incidence of severe respiratory dysfunction (respiratory SOFA >2) at admission was higher in the other-SRD group, whereas severe hematological dysfunction (hematological SOFA >2) during ICU stay was higher in the SLE group. SLE patients were younger and displayed a decreased incidence of respiratory failure compared to patients with other-SRDs. However, the incidences of renal and hematological failure and the presence of shock at admission were higher in the SLE group. The 60-day survival rates were similar.
Assuntos
Doenças Hematológicas/epidemiologia , Falência Renal Crônica/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Insuficiência de Múltiplos Órgãos/mortalidade , Transtornos Respiratórios/epidemiologia , Doenças Reumáticas/complicações , Adulto , Estado Terminal , Métodos Epidemiológicos , Feminino , Doenças Hematológicas/etiologia , Hospitalização/estatística & dados numéricos , Humanos , Unidades de Terapia Intensiva , Falência Renal Crônica/etiologia , Tempo de Internação/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Pessoa de Meia-Idade , Transtornos Respiratórios/etiologia , Doenças Reumáticas/classificação , Doenças Reumáticas/mortalidadeRESUMO
The objective of the study was to evaluate risk factors for pulmonary tuberculosis in systemic lupus erythematosus (SLE). Clinical/laboratorial features of 1283 SLE patients (ACR criteria) followed at the Lupus Clinic were obtained from the electronic register database from 2001 to 2009. Pulmonary tuberculosis was diagnosed in 20 patients (1.6%) (TB+ group). As control group (TB-), 40 patients without tuberculosis matched for age, gender, ethnicity, age at SLE diagnosis, and disease duration were arbitrarily selected. All 20 patients of the TB+ group presented confirmed pulmonary tuberculosis from 1 to 23 years after SLE diagnosis (7.6 ± 8.1 years). Frequencies of previous SLE involvements (cutaneous, articular, hematological, renal, pericarditis, pneumonitis, and central nervous system) were alike in TB+ and TB- groups (p > 0.05). In contrast, prior pleuritis was more frequent in the TB+ group (40% vs. 5%, p = 0.001). In fact, pulmonary tuberculosis was diagnosed in 8/10 patients with previous pleuritis. Immunosuppressive and corticosteroid therapies at the moment of tuberculosis diagnosis were also similar in both groups (p > 0.05). We have identified pleuritis as a relevant risk factor for pulmonary tuberculosis, suggesting that previous pleural injury is a critical part of the complex interplay between altered immune system, socio-economic conditions, and increased susceptibility to this mycobacterial infection.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Pleurisia/complicações , Tuberculose Pulmonar/etiologia , Adulto , Bases de Dados Factuais , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Pleurisia/etiologia , Fatores de Risco , Fatores Socioeconômicos , Tuberculose Pulmonar/epidemiologiaRESUMO
We have previously developed and validated a self-administered questionnaire, modelled after the Systemic Lupus International Collaborating Clinics Damage Index (SDI), the Lupus Damage Index Questionnaire (LDIQ), which may allow the ascertainment of this construct in systemic lupus erythematosus (SLE) patients followed in the community and thus expand observations made about damage. We have now translated, back-translated and adapted the LDIQ to Spanish, Portuguese and French and applied it to patients followed at academic and non-academic centres in North and South America, Portugal and Spain while their physicians scored the SDI. A total of 887 patients (659 Spanish-speaking, 140 Portuguese-speaking and 80 French-speaking patients) and 40 physicians participated. Overall, patients scored all LDIQ versions higher than their physicians (total score and all domains). Infrequent manifestations had less optimal clinimetric properties but overall agreement was more than 95% for the majority of items. Higher correlations were observed among the Spanish-speaking patients than the Portuguese-speaking and French-speaking patients; further adjustments may be needed before the Portuguese and French versions of the LDIQ are applied in community-based studies. The relationship between the LDIQ and other outcome parameters is currently being investigated in a different patient sample.
Assuntos
Idioma , Lúpus Eritematoso Sistêmico , Inquéritos e Questionários , Adulto , Feminino , Inquéritos Epidemiológicos , Humanos , Lúpus Eritematoso Sistêmico/patologia , Lúpus Eritematoso Sistêmico/fisiopatologia , América do Norte , Portugal , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , América do Sul , Espanha , Inquéritos e Questionários/normasRESUMO
Seropositivity of anti-Helicobacter pylori antibody (HP + ) was examined among Japanese Brazilians. The study was announced through 18 Japanese community culture associations in São Paulo, Curitiba, Mogi das Cruzes, and Mirandopolis in 2001. Among 969 participants, 963 individuals aged 33 - 69 years were analyzed. The overall HP + % was 48.1% (95% confidence interval, 44.9 - 51.3%). There was no difference in HP + % between 399 males and 564 females (49.6% and 47.0%, respectively). The HP + % increased with age; 35.3% for those aged 33 - 39 years, 46.2% for those aged 40 - 49 years, 46.5% for those aged 50 - 59 years, and 56.9% for those aged 60 - 69 years, but no differences were observed among the generations (Issei, Nisei, and Sansei) for each 10-year age group. Mogi das Cruzes, a rural area, showed a higher HP + %. Length of education was inversely associated with the positivity; the odds ratio (OR) relative to those with eight years or less of schooling was 0.61 (0.42 - 0.89) for those with 12 years or more. The associations with smoking and alcohol drinking were not significant. Fruit intake was associated with the HP + %; the OR relative to everyday intake was 1.38 (1.05 - 1.83) for less frequent intake, while intake frequencies of green tea, miso soup, and pickled vegetables (tsukemono) were not. Multivariate analysis including sex, 10-year age group, residence, education, and fruit intake showed that all factors except sex were significant. This is the largest study of HP infection among Japanese Brazilians, and the results indicated a similar pattern of age-specific infection rate to that for Japanese in Japan.
Assuntos
Envelhecimento/fisiologia , Povo Asiático , Infecções por Helicobacter/epidemiologia , Helicobacter pylori/isolamento & purificação , Estilo de Vida/etnologia , Caracteres Sexuais , Adulto , Idoso , Consumo de Bebidas Alcoólicas , Brasil/epidemiologia , Efeito de Coortes , Comportamento Alimentar , Feminino , Infecções por Helicobacter/etiologia , Infecções por Helicobacter/microbiologia , Helicobacter pylori/imunologia , Humanos , Japão/etnologia , Masculino , Pessoa de Meia-Idade , Razão de Chances , FumarRESUMO
The anticlotting and antithrombotic activities of heparin, heparan sulfate, low molecular weight heparins, heparin and heparin-like compounds from various sources used in clinical practice or under development are briefly reviewed. Heparin isolated from shrimp mimics the pharmacological activities of low molecular weight heparins. A heparan sulfate from Artemia franciscana and a dermatan sulfate from tuna fish show a potent heparin cofactor II activity. A heparan sulfate derived from bovine pancreas has a potent antithrombotic activity in an arterial and venous thrombosis model with a negligible activity upon the serine proteases of the coagulation cascade. It is suggested that the antithrombotic activity of heparin and other antithrombotic agents is due at least in part to their action on endothelial cells stimulating the synthesis of an antithrombotic heparan sulfate.
Assuntos
Humanos , Animais , Bovinos , Anticoagulantes/farmacologia , Endotélio Vascular/citologia , Fibrinolíticos/farmacologia , Heparina/farmacologia , Heparitina Sulfato/farmacologia , Anticoagulantes/química , Anticoagulantes/metabolismo , Crustáceos , Fibrinolíticos/química , Fibrinolíticos/metabolismo , Glicosaminoglicanos/metabolismo , Glicosaminoglicanos/farmacologia , Heparina de Baixo Peso Molecular/química , Heparina de Baixo Peso Molecular/metabolismo , Heparina de Baixo Peso Molecular/farmacologia , Heparina/metabolismo , Heparitina Sulfato/biossíntese , AtumRESUMO
The anticlotting and antithrombotic activities of heparin, heparan sulfate, low molecular weight heparins, heparin and heparin-like compounds from various sources used in clinical practice or under development are briefly reviewed. Heparin isolated from shrimp mimics the pharmacological activities of low molecular weight heparins. A heparan sulfate from Artemia franciscana and a dermatan sulfate from tuna fish show a potent heparin cofactor II activity. A heparan sulfate derived from bovine pancreas has a potent antithrombotic activity in an arterial and venous thrombosis model with a negligible activity upon the serine proteases of the coagulation cascade. It is suggested that the antithrombotic activity of heparin and other antithrombotic agents is due at least in part to their action on endothelial cells stimulating the synthesis of an antithrombotic heparan sulfate.
Assuntos
Anticoagulantes/farmacologia , Endotélio Vascular/efeitos dos fármacos , Fibrinolíticos/farmacologia , Heparina/farmacologia , Heparitina Sulfato/farmacologia , Animais , Anticoagulantes/química , Anticoagulantes/metabolismo , Bovinos , Crustáceos , Endotélio Vascular/citologia , Fibrinolíticos/química , Fibrinolíticos/metabolismo , Glicosaminoglicanos/metabolismo , Glicosaminoglicanos/farmacologia , Heparina/química , Heparina/metabolismo , Heparina de Baixo Peso Molecular/química , Heparina de Baixo Peso Molecular/metabolismo , Heparina de Baixo Peso Molecular/farmacologia , Heparitina Sulfato/biossíntese , Humanos , Relação Estrutura-Atividade , AtumRESUMO
The capsular polysaccharide from E. Coli, strain K5 composed of ...-->4)beta-D-GlcA(1-->4)alpha-D-GlcNAc(1-->4)beta-D-GlcA (1-->..., chemically modified K5 polysaccharides, bearing sulfates at C-2 and C-6 of the hexosamine moiety and at the C-2 of the glucuronic acid residues as well as 2-O desulfated heparin were used as substrates to study the specificity of heparitinases I and II and heparinase from Flavobacterium heparinum. The natural K5 polysaccharide was susceptible only to heparitinase I forming deltaU-GlcNAc. N-deacetylated, N-sulfated K5 became susceptible to both heparitinases I and II producing deltaU-GlcNS. The K5 polysaccharides containing sulfate at the C-2 and C-6 positions of the hexosamine moiety and C-2 position of the glucuronic acid residues were susceptible only to heparitinase II producing deltaU-GlcNS,6S and deltaU,2S-GlcNS,6S respectively. These combined results led to the conclusion that the sulfate at C-6 position of the glucosamine is impeditive for the action of heparitinase I and that heparitinase II requires at least a C-2 or a C-6 sulfate in the glucosamine residues of the substrate for its activity. Iduronic acid-2-O-desulfated heparin was susceptible only to heparitinase II producing deltaU-GlcNS,6S. All the modified K5 polysaccharides as well as the desulfated heparin were not substrates for heparinase. This led to the conclusion that heparitinase II acts upon linkages containing non-sulfated iduronic acid residues and that heparinase requires C-2 sulfated iduronic acid residues for its activity.