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Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/diagnóstico , Ácido Úrico/sangre , Adulto , Factores de Edad , Anticuerpos Anticardiolipina/sangre , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/epidemiología , Dislipidemias/epidemiología , Femenino , Humanos , Hipertensión/epidemiología , Inmunoglobulina G/sangre , Inmunoglobulina M/sangre , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/epidemiología , Masculino , Persona de Mediana Edad , Factores SexualesRESUMEN
OBJECTIVE: To review all published cases of the rare association between thrombotic thrombocytopenic purpura (TTP) and Sjögren's syndrome (SS). The authors report an additional case of this unique association. METHODS: Systematic review of the literature and a case report. The database were articles published in PubMed/MEDLINE, Web of Science, LILACS, and SciELO, registered from 1966 to August 2020. The DESH terms were "Sjögren's syndrome" and "thrombotic thrombocytopenic purpura," without language limitation. RESULTS: Most patients were female (88%), and the age varied from 30 to 75 years old. Concerning the sequence of disease appearance, SS followed by TTP was seen in seven articles, TTP and SS in three, and simultaneous appearance of both diseases in three studies. Primary SS was observed in 16 patients, and secondary SS was detected in two cases: dermatomyositis and rheumatoid arthritis. Anemia was the most common TTP manifestation, followed by thrombocytopenia, fever, consciousness alteration, renal impairment, and schistocytes' appearance on a blood smear. Treatment involved plasmapheresis, plasma exchange, rituximab, glucocorticoid, and cyclophosphamide. A good outcome was noted in most studies; few patients died. CONCLUSIONS: TTP is a rare manifestation associated with SS. After the TTP diagnosis, plasmapheresis and/or plasma exchange should be immediately implemented.
RESUMEN
One of the most famous works by the Austrian symbolist painter Gustav Klimt and one of the most widely reproduced works of art worldwide, Adele Bloch-Bauer I which portrays the beautiful wife of Austrian magnate Ferdinand Bloch-Bauer. Adele was the only woman painted by Klimt on more than one occasion. Apart from the beauty and value of the painting, the daring sea of gold that surrounds Adele and the gentle intimacy with which her fragile figure is portrayed have shrouded the history of this painting in mystery. Beyond speculation as to a special bond between artist and model, observation of the painting with a keener, clinical gaze yields evidence of potential illness in the model: facial erythema which, if not produced artificially by makeup, could represent a malar rash; pallor or cyanosis of the hands; and her draped fingers, which seemingly attempt to hide a deformity. This paper seeks to provide a biographical review both of the painter, Gustav Klimt, and of the subject, Adele Bloch-Bauer; to analyse Klimt's two portrayals of her in a search for evidence of a potential intimate relationship between artist and muse and, finally, to compile clinical evidence of possible diagnoses for the Lady in Gold.
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Personajes , Lupus Eritematoso Sistémico/historia , Pinturas/historia , Fiebre Reumática/historia , Sífilis/historia , Austria , Diagnóstico Diferencial , Encefalitis/historia , Historia del Siglo XIX , Historia del Siglo XXRESUMEN
BACKGROUND: Few studies have addressed the ultrastructure of vascular permeability in urticaria. OBJECTIVES: To describe the types of endothelial cell organelles involved in vascular permeability in drug-induced acute urticaria (DIAU). METHODS: Seven patients with DIAU were enrolled in the study. Biopsies of urticarial lesions and apparently normal skin were performed. The 14 collected fragmentswere processed with immunogold electron microscopy using single stains for tryptase and factor XIIIa (FXIIIa) and double immunogold labeling for both tryptase and FXIIIa. RESULTS: Some sections demonstrated mast cells in the degranulation process, in both anaphylactic and piecemeal degranulation. After double immunogold staining, 10 nm (FXIIIa) and 15 nm (tryptase) gold particles wereboth present, covering the granules in the mast cells, indicating that both tryptase and FXIIIa were localized within the granules of these cells. Interestingly, we found strong evidence of the presence of caveolae and vesico-vacuolar organelles (VVOs) in the endothelial cells of the biopsies. In addition to these findings, we were able to demonstrate the presence of tryptase and FXIIIa in the endothelial celIs, in urticarial lesions and in apparently normal skin. CONCLUSIONS: VVOs are present in the endothelial cells of post-capillary venules in DIAU. This is the first report on the expression of FXIIIa and tryptase in the cytoplasm of endothelial cells in urticaria.
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Permeabilidad Capilar , Hipersensibilidad a las Drogas/inmunología , Urticaria/inducido químicamente , Enfermedad Aguda , Adulto , Niño , Citoplasma/metabolismo , Citoplasma/ultraestructura , Hipersensibilidad a las Drogas/etiología , Células Endoteliales/metabolismo , Células Endoteliales/ultraestructura , Factor XIIIa/metabolismo , Femenino , Humanos , Microscopía Electrónica , Persona de Mediana Edad , Orgánulos/metabolismo , Orgánulos/ultraestructura , Coloración y Etiquetado , Triptasas/metabolismo , Urticaria/inmunologíaRESUMEN
This study aims to perform global gonadal and sexual function assessments in systemic lupus erythematosus-related antiphospholipid syndrome (SLE-APS) patients. A cross-sectional study was conducted in ten SLE-APS male patients and 20 healthy controls. They were assessed by demographic data, clinical features, urological examination, sexual function, testicular ultrasound, seminal parameters, sperm antibodies, and hormone profile. The median of current age was similar in SLE-APS patients and controls with a higher frequency of erectile dysfunction in the former group (30 vs. 0 %, p = 0.029). The median penis circumference was significantly reduced in SLE-APS patients with erectile dysfunction compared to patients without this complication (8.17 vs. 9.14 cm, p = 0.0397). SLE-APS patients with previous arterial thrombosis had a significantly reduced median penis circumference compared to those without this complication (7.5 vs. 9.18 cm, p = 0.039). Comparing SLE-APS patients and controls, the former had a significant lower median of sperm concentration (41.1 vs. 120.06 × 10(6)/mL, p = 0.003), percentages of sperm motility (47.25 vs. 65.42 %, p = 0.047), normal sperm forms by WHO guidelines (11 vs. 23.95 %, p = 0.002), and Kruger criteria (2.65 vs. 7.65 %, p = 0.02). Regarding seminal analysis, the medians of sperm concentration and total sperm count were significantly lower in SLE-APS patients treated with intravenous cyclophosphamide vs. those untreated with this drug (p < 0.05). Therefore, we have observed a novel association of reduced penile size with erectile dysfunction and previous arterial thrombosis in SLE-APS patients. Penis assessment should be routinely done in SLE-APS patients with fertility problems. We also identified that intravenous cyclophosphamide underlies severe sperm alterations in these patients.
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Síndrome Antifosfolípido/patología , Impotencia Vasculogénica/patología , Lupus Eritematoso Sistémico/patología , Pene/patología , Espermatozoides/patología , Adolescente , Adulto , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/epidemiología , Brasil/epidemiología , Comorbilidad , Estudios Transversales , Hormonas Gonadales/sangre , Humanos , Impotencia Vasculogénica/sangre , Impotencia Vasculogénica/epidemiología , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/epidemiología , Masculino , Persona de Mediana Edad , Pene/irrigación sanguínea , Motilidad Espermática , Espermatozoides/fisiología , Testículo/diagnóstico por imagen , Testículo/patología , Ultrasonografía , Adulto JovenRESUMEN
The DRESS syndrome (drug reaction with eosinophilia and systemic symptoms), also known as DIHS (drug-induced hypersensitivity syndrome), presents clinically as an extensive mucocutaneous rash, accompanied by fever, lymphadenopathy, hepatitis, hematologic abnormalities with eosinophilia and atypical lymphocytes, and may involve other organs with eosinophilic infiltration, producing damage in several systems, especially kidney, heart, lungs, and pancreas. The pathogenesis is related to specific drugs (especially the aromatic anticonvulsants), altered immune response, sequential reactivation of herpes virus, and association with some HLA alleles. Glucocorticoids are the basis for the treatment of the syndrome, which may be given with intravenous immunoglobulin and, in selected cases, ganciclovir. This article reviews current concepts regarding the interaction of drugs, viruses and immune responses during this complex adverse-drug reaction.
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Hipersensibilidad a las Drogas/etiología , Hipersensibilidad a las Drogas/inmunología , Hipersensibilidad a las Drogas/virología , Eosinofilia/inducido químicamente , Eosinofilia/inmunología , Eosinofilia/virología , Herpesviridae/inmunología , Hipersensibilidad a las Drogas/terapia , Eosinofilia/terapia , Humanos , Factores de Riesgo , SíndromeRESUMEN
OBJECTIVES: This study was undertaken to evaluate a possible association of adipocytokines with metabolic syndrome (MetS), inflammation and other cardiovascular risk factors in primary antiphospholipid syndrome (PAPS). METHODS: Fifty-six PAPS patients and 72 controls were included. Adiponectin, leptin, visfatin, resistin, plasminogen activator inhibitor-1 (PAI-1), lipoprotein (a), glucose, ESR, CRP, uric acid and lipid profiles were measured. The presence of MetS was determined as defined by the International Diabetes Federation (IDF), and insulin resistance was rated using the homeostasis model assessment (HOMA) index. RESULTS: Concentrations of leptin were higher [21.5 (12.9-45.7) ng/mL] in PAPS patients than in the controls [12.1 (6.9-26.8) ng/mL), p=0.001]. In PAPS patients, leptin and PAI-1 levels were positively correlated with BMI (r=0.61 and 0.29), HOMA-IR (r=0.71 and 0.28) and CRP (r=0.32 and 0.36). Adiponectin levels were negatively correlated with BMI (r=-0.28), triglycerides (r=-0.43) and HOMA-IR (r=-0.36) and positively correlated with HDL-c (r=0.37) and anti-ß2GPI IgG (r=0.31). The presence of MetS in PAPS patients was associated with higher levels of leptin (p=0.002) and PAI-1 (p=0.03) levels and lower levels of adiponectin (p=0.042). Variables that independently influenced the adiponectin concentration were the triglyceride levels (p<0.001), VLDL-c (P=0.002) and anti-ß2GPI IgG (p=0.042); the leptin levels were BMI (p<0.001), glucose (p=0.046), HOMA-IR (p<0.001) and ESR (p=0.006); and the PAI-1 levels were CRP (p=0.013) and MetS (p=0.048). CONCLUSIONS: This study provides evidence that adipocytokines may be involved in low-grade inflammation, insulin resistance and MetS in PAPS patients.
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Adipoquinas/sangre , Síndrome Antifosfolípido/sangre , Mediadores de Inflamación/sangre , Inflamación/sangre , Resistencia a la Insulina , Síndrome Metabólico/sangre , Adiponectina/sangre , Adulto , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/inmunología , Biomarcadores/sangre , Estudios de Casos y Controles , Distribución de Chi-Cuadrado , Estudios Transversales , Citocinas/sangre , Femenino , Humanos , Inflamación/diagnóstico , Inflamación/inmunología , Leptina/sangre , Modelos Lineales , Lípidos/sangre , Masculino , Síndrome Metabólico/diagnóstico , Síndrome Metabólico/inmunología , Persona de Mediana Edad , Análisis Multivariante , Nicotinamida Fosforribosiltransferasa/sangre , Inhibidor 1 de Activador Plasminogénico/sangre , Resistina/sangreRESUMEN
BACKGROUND: Antibodies directed against endothelial cell surface antigens have been described in many disorders and have been associated with disease activity. Since the most prominent histopathologic feature in mixed connective tissue disease (MCTD) is the widespread and unique proliferative vascular lesion, our aim was to evaluate the frequency of anti-endothelial cell antibodies (AECA) in this condition. OBJECTIVES: To evaluate the frequency of AECA in this disease and assess its clinical and laboratory associations. METHODS: Seventy-three sera from 35 patients with MCTD (Kasukawa's criteria), collected during a 7 year period, were tested for immunoglobulins G and M (IgG and IgM) AECA by cellular ELISA, using HUVEC (human umbilical vein endothelial cells). Sera from 37 patients with systemic lupus erythematosus (SLE), 22 with systemic sclerosis (SSc) and 36 sera from normal healthy individuals were used as controls. A cellular ELISA using HeLa cells was also performed as a laboratory control method. RESULTS: IgG-AECA was detected in 77% of MCTD patients, 54% of SLE patients, 36% of SSc patients and 6% of normal controls. In MCTD, IgG-AECA was associated with vasculitic manifestations, disease activity and lymphopenia, and was also a predictor of constant disease activity. Immunosuppressive drugs were shown to reduce IgG-AECA titers. Since antibodies directed to HeLa cell surface were negative, AECA was apparently unrelated to common epitopes present on epithelial cell lines. CONCLUSIONS: AECA are present in a large proportion of patients with MCTD and these antibodies decrease after immunosuppressive treatment.
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Autoanticuerpos/sangre , Enfermedad Mixta del Tejido Conjuntivo/inmunología , Adulto , Biomarcadores/sangre , Estudios Transversales , Endotelio Vascular/inmunología , Ensayo de Inmunoadsorción Enzimática/métodos , Femenino , Células Endoteliales de la Vena Umbilical Humana , Humanos , Inmunoglobulina G/sangre , Inmunoglobulina M/sangre , Estudios Longitudinales , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/inmunología , Masculino , Enfermedad Mixta del Tejido Conjuntivo/sangre , Esclerodermia Sistémica/sangre , Esclerodermia Sistémica/inmunologíaRESUMEN
The association of RASopathies [Noonan syndrome (NS) and Noonan-related syndromes] and autoimmune disorders has been reported sporadically. However, a concomitant evaluation of autoimmune diseases and an assessment of multiple autoantibodies in a large population of patients with molecularly confirmed RASopathy have not been performed. The clinical and laboratory features were analyzed in 42 RASopathy patients, the majority of whom had NS and five individuals had Noonan-related disorders. The following autoantibodies were measured: Anti-nuclear antibodies, anti-double stranded DNA, anti-SS-A/Ro, anti-SS-B/La, anti-Sm, anti-RNP, anti-Scl-70, anti-Jo-1, anti-ribosomal P, IgG and IgM anticardiolipin (aCL), thyroid, anti-smooth muscle, anti-endomysial (AE), anti-liver cytosolic protein type 1 (LC1), anti-parietal cell (APC), anti-mitochondrial (AM) antibodies, anti-liver-kidney microsome type 1 antibodies (LKM-1), and lupus anticoagulant. Six patients (14%) fulfilled the clinical criteria for autoimmune diseases [systemic lupus erythematous, polyendocrinopathy (autoimmune thyroiditis and celiac disease), primary antiphospholipid syndrome (PAPS), autoimmune hepatitis, vitiligo, and autoimmune thyroiditis]. Autoimmune antibodies were observed in 52% of the patients. Remarkably, three (7%) of the patients had specific gastrointestinal and liver autoantibodies without clinical findings. Autoimmune diseases and autoantibodies were frequently present in patients with RASopathies. Until a final conclusion of the real incidence of autoimmunity in Rasopathy is drawn, the physicians should be alerted to the possibility of this association and the need for a fast diagnosis, proper referral to a specialist and ultimately, adequate treatment.
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Autoanticuerpos/sangre , Enfermedades Autoinmunes/inmunología , Síndrome de Noonan/inmunología , Autoanticuerpos/clasificación , Enfermedades Autoinmunes/epidemiología , Granuloma de Células Gigantes , Humanos , Síndrome de Noonan/epidemiologíaRESUMEN
To report a case of triple association of juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis and urticarial vasculitis as well as a review of the relevant literature. A 12-year-old male patient diagnosed with overlap syndrome between SLE and juvenile dermatomyositis since 2004 evolved with erythematous plaques, which were compatible with an urticarial rash. Clinical, laboratory and histopathological findings indicated a diagnosis of urticarial vasculitis. The patient previously had a C1q deficiency. Using the established treatment with methylprednisolone (1 g/day for 3 days), increasing doses of deflazacort and introduction of a dapsone, as well as mycophenolate mofetil regimen, with the suspension of azathioprine resulted in complete resolution of skin lesions. Urticarial vasculitis can present in various diseases. In SLE, presentation of urticarial vasculitis in children is rarely found. The triple association of juvenile-onset SLE, juvenile dermatomyositis and urticarial vasculitis is unusual, and this is the first case described in literature.
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Dermatomiositis/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Urticaria/complicaciones , Vasculitis/complicaciones , Niño , Dermatomiositis/patología , Humanos , Lupus Eritematoso Sistémico/patología , Masculino , Síndrome , Urticaria/patología , Vasculitis/patologíaRESUMEN
BACKGROUND: Reduced response to pandemic (2009) H1N1 (pH1N1) vaccine in patients with rheumatoid arthritis (RA) was recently reported. OBJECTIVES: To evaluate the contribution of age, disease activity, medication and previous antibody levels to this reduced response. METHODS: 340 adult RA patients and 234 healthy controls were assessed before and 21 days after adjuvant-free influenza A/California/7/2009 (pH1N1) vaccine. Disease activity (DAS28), current treatment and pH1N1 antibody titres were collected. Seroprotection, seroconversion and factor increase in geometric mean titre (GMT) were calculated and adverse events registered. RESULTS: RA and controls showed similar (p>0.05) prevaccination GMT (8.0 vs 9.3) and seroprotection (10.8% vs 11.5%). After vaccination a significant reduction (p<0.001) was observed in all endpoints: GMT and factor increase in GMT, seroprotection and seroconversion rates. Disease activity did not preclude seroconversion or seroprotection and remained unchanged in 97.4% of patients. Methotrexate was the only disease-modifying antirheumatic drug associated with reduced responses (p=0.001). Vaccination was well tolerated. CONCLUSIONS: The data confirmed both short-term anti-pH1N1 vaccine safety and, different from most studies with seasonal influenza, reduced seroprotection in RA patients, unrelated to disease activity and to most medications (except methotrexate). Extrapolation of immune responses from one vaccine to another may therefore not be possible and specific immunisation strategies (possibly booster) may be needed. Clinicaltrials.gov no NCT01151644.
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Artritis Reumatoide/inmunología , Subtipo H1N1 del Virus de la Influenza A/inmunología , Vacunas contra la Influenza/inmunología , Gripe Humana/prevención & control , Adyuvantes Inmunológicos , Adulto , Anciano , Anticuerpos Antivirales/sangre , Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Estudios de Casos y Controles , Femenino , Humanos , Inmunosupresores/uso terapéutico , Vacunas contra la Influenza/efectos adversos , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVES: To evaluate lipid profile changes after anti-TNF therapy in patients with psoriatic arthritis (PsA). METHODS: Fifteen PsA patients (eight polyarticular, four oligoarticular, two axial, and one mutilating) under infliximab were included. None had dyslipoproteinemia or previous statin use. Total cholesterol (TC) and its fractions, inflammatory markers, and prednisone use were evaluated. RESULTS: The comparisons of lipid levels between baseline and after three months (3M) of anti-TNF therapy showed that there was a significant increase in mean triglycerides (117.8 ± 49.7 versus 140.1 ± 64.1 mg/dL, P = 0.028) and VLDL-c (23.6 ± 10.5 versus 28.4 ± 13.7 mg/dL, P = 0.019) levels. In contrast, there were no differences in the mean TC (P = 0.28), LDL-c (P = 0.42), and HDL-c (P = 0.26) levels. Analysis of the frequencies of each lipid alteration at baseline and at 3M were alike (P > 0.05). Positive correlations were found between VLDL-c and CRP (r = 0.647, P = 0.009) and between triglycerides and CRP (r = 0.604, P = 0.017) levels at 3M. ESR reduction was observed after 3M (P = 0.04). Mean prednisone dose remained stable at beginning and at 3M (P = 0.37). CONCLUSION: This study demonstrated that anti-TNF may increase TG and VLDL-c levels in PsA patients after three months.
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Anticuerpos Monoclonales/administración & dosificación , Antirreumáticos/administración & dosificación , Artritis Psoriásica/tratamiento farmacológico , Metabolismo de los Lípidos/efectos de los fármacos , Adulto , Anticuerpos Monoclonales/uso terapéutico , Antirreumáticos/uso terapéutico , Artritis Psoriásica/sangre , Artritis Psoriásica/patología , Proteína C-Reactiva/análisis , HDL-Colesterol/sangre , LDL-Colesterol/sangre , VLDL-Colesterol/análisis , VLDL-Colesterol/sangre , Femenino , Humanos , Infliximab , Masculino , Persona de Mediana Edad , Prednisona/administración & dosificación , Estudios Prospectivos , Triglicéridos/sangreRESUMEN
BACKGROUND: Despite the WHO recommendation that the 2010-2011 trivalent seasonal flu vaccine must contain A/California/7/2009/H1N1-like virus there is no consistent data regarding its immunogenicity and safety in a large autoimmune rheumatic disease (ARD) population. METHODS: 1668 ARD patients (systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), ankylosing spondylitis (AS), systemic sclerosis, psoriatic arthritis (PsA), Behçet's disease (BD), mixed connective tissue disease, primary antiphospholipid syndrome (PAPS), dermatomyositis (DM), primary Sjögren's syndrome, Takayasu's arteritis, polymyositis and Granulomatosis with polyangiitis (Wegener's) (GPA)) and 234 healthy controls were vaccinated with a non-adjuvanted influenza A/California/7/2009(H1N1) virus-like strain flu. Subjects were evaluated before vaccination and 21 days post-vaccination. The percentage of seroprotection, seroconversion and the factor increase in geometric mean titre (GMT) were calculated. RESULTS: /st> After immunisation, seroprotection rates (68.5% vs 82.9% p<0.0001), seroconversion rates (63.4% vs 76.9%, p<0.001) and the factor increase in GMT (8.9 vs 13.2 p<0.0001) were significantly lower in ARD than controls. Analysis of specific diseases revealed that seroprotection significantly reduced in SLE (p<0.0001), RA (p<0.0001), PsA (p=0.0006), AS (p=0.04), BD (p=0.04) and DM (p=0.04) patients than controls. The seroconversion rates in SLE (p<0.0001), RA (p<0.0001) and PsA (p=0.0006) patients and the increase in GMTs in SLE (p<0.0001), RA (p<0.0001) and PsA (p<0.0001) patients were also reduced compared with controls. Moderate and severe side effects were not reported. CONCLUSIONS: The novel recognition of a diverse vaccine immunogenicity profile in distinct ARDs supports the notion that a booster dose may be recommended for diseases with suboptimal immune responses. This large study also settles the issue of vaccine safety. (ClinicalTrials.gov #NCT01151644).
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Enfermedades Autoinmunes/inmunología , Subtipo H1N1 del Virus de la Influenza A/inmunología , Vacunas contra la Influenza/inmunología , Gripe Humana/prevención & control , Enfermedades Reumáticas/inmunología , Adyuvantes Inmunológicos , Adulto , Anticuerpos Antivirales/biosíntesis , Métodos Epidemiológicos , Femenino , Humanos , Tolerancia Inmunológica , Huésped Inmunocomprometido , Vacunas contra la Influenza/efectos adversos , Masculino , Persona de Mediana Edad , Vacunación/efectos adversos , Vacunación/métodos , Adulto JovenRESUMEN
INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL AND METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100% of the professionals at the first visit and by 36% annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44%, 64%, and 48%, respectively. The professionals were divided into two groups: Group A (≤ 15 years of practice, n = 12) and B (≥ 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.
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Pediatría , Pautas de la Práctica en Medicina , Enfermedades Reumáticas , Reumatología , Vacunación/estadística & datos numéricos , Niño , HumanosRESUMEN
INTRODUÇÃO/OBJETIVOS: Avaliar a prática clínica com relação à verificação do cartão vacinal e à indicação de vacinas específicas em pacientes com doenças reumáticas pediátricas em uso de diferentes drogas, e evidenciar a possível associação entre frequência de vacinação e tempo de prática clínica dos reumatologistas pediátricos do estado de São Paulo. MATERIAL E MÉTODOS: Um questionário foi enviado para os reumatologistas pediátricos do Departamento de Reumatologia da Sociedade de Pediatra de São Paulo. Esse instrumento incluiu questões sobre tempo de prática em Reumatologia Pediátrica, vacinação de pacientes com Lúpus Eritematoso Sistêmico Juvenil (LESJ), artrite idiopática juvenil (AIJ), dermatomiosite juvenil (DMJ) e imunização de acordo com os tratamentos utilizados. RESULTADOS: Cartão de vacinação foi visto por 100 por cento dos profissionais na primeira consulta e por 36 por cento anualmente. Vacinas de agentes vivos não foram recomendadas para pacientes com LESJ, AIJ e DMJ em 44 por cento, 64 por cento e 48 por cento, respectivamente. Os profissionais foram divididos em dois grupos: A (< 15 anos de prática, n = 12) e B (> 16 anos, n = 13). Nenhuma diferença estatística foi observada no uso de vacinas de agentes vivos e vacinas de agentes inativos ou componentes proteicos em relação ao tratamento nos dois grupos (P > 0,05). Além disso, os grupos foram similares em relação à opinião sobre a gravidade de imunossupressão em pacientes com LESJ, AIJ e DMJ com ou sem atividade e a terapêutica utilizada (P > 0,05). CONCLUSÕES: A frequência de vacinação por reumatologistas pediátricos de São Paulo é baixa, especialmente após a primeira consulta, e não é influenciada pelo tempo de prática profissional.
INTRODUCTION/OBJECTIVES: Evaluate clinical practice through assessment of vaccination card and recommendation of specific vaccines in pediatric patients with rheumatic diseases in use of different drugs and reveal the possible association between vaccination frequency and time of the clinical practice of pediatric rheumatologists in the state of São Paulo. MATERIAL AND METHODS: A questionnaire was sent to pediatric rheumatologists of the Departamento de Reumatologia da Sociedade de Pediatria de São Paulo. This instrument included questions about practice time on Pediatric Rheumatology, vaccination of patients with juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), and immunization according to the treatments used. RESULTS: Vaccination card was seen by 100 percent of the professionals at the first visit and by 36 percent annually. Vaccines of live agents were not recommended for patients with JSLE, JIA, and JDM in 44 percent, 64 percent, and 48 percent, respectively. The professionals were divided into two groups: Group A (< 15 years of practice, n = 12) and B (> 16 years, n = 13). No statistical difference was observed in the use of live agent vaccine and vaccines with inactivated agents or protein components in the two treatment groups (P > 0.05). Moreover, the groups had similar opinion regarding severity of immunosuppression in patients with JSLE, JIA, and JDM (with or without activity) and treatment used (P > 0.05). CONCLUSIONS: The frequency of immunization by pediatric rheumatologists in São Paulo is low, especially after the first visit, and not influenced by time of professional practice.
Asunto(s)
Niño , Humanos , Pediatría , Pautas de la Práctica en Medicina , Enfermedades Reumáticas , Reumatología , Vacunación/estadística & datos numéricosRESUMEN
BACKGROUND: Neurologic disorders are among the most common and important clinical manifestations associated with the antiphospholipid syndrome (APS). It is characterized by diverse neurological manifestations. These include stroke, transient ischaemic attack, Sneddon's syndrome, convulsions/epilepsy, dementia, cognitive deficits, headaches/migraine, chorea, multiple sclerosis-like, transverse myelitis, ocular symptoms and Guillain-Barré syndrome. MATERIAL AND METHODS: We review the latest data about neurologic disorders and APS. RESULTS: In patients under 45 years of age, 20% of strokes are potentially associated with APS. Our study group recently reported a correlation between primary APS and peripheral neuropathy. Only one study investigated the occurrence of peripheral neuropathy in patients diagnosed with PAPS through electrophysiological study and showed alterations in 35% of patients. The mechanism of nervous system involvement in APS is considered to be primarily thrombotic. However, other mechanisms have been described, such as antiphospholipid antibodies that bind to the neural tissue, deregulating their functions and having an immediate pathogenic effect. CONCLUSIONS: This review summarizes the latest data regarding the clinical aspects, radiological and therapeutic of major neurologic manifestations associated with antiphospholipid antibodies.
Asunto(s)
Síndrome Antifosfolípido/complicaciones , Enfermedades del Sistema Nervioso/etiología , Anticuerpos Antifosfolípidos , Humanos , Enfermedades del Sistema Nervioso/tratamiento farmacológicoRESUMEN
Crianças e adolescentes com doenças reumatológicas apresentam maior prevalência de doenças infecciosas quando comparados com a população em geral, em decorrência de atividade da doença, possível deficiência imunológica secundária à própria doença, ou uso de terapia imunossupressora. A vacinação é uma medida eficaz para a redução da morbidade e mortalidade nesses pacientes. O objetivo deste artigo foi realizar um consenso de eficácia e segurança das vacinas em crianças e adolescentes com doenças reumatológicas infantis baseadas em níveis de evidência científica. Imunização passiva para os pacientes e orientações para as pessoas que convivem com doentes imunodeprimidos também foram incluídas. Os 32 pediatras reumatologistas membros do Departamento de Reumatologia da Sociedade de Pediatria de São Paulo (SPSP) e/ou da Comissão de Reumatologia Pediátrica da Sociedade Brasileira de Reumatologia elaboraram o consenso, sendo que alguns desses profissionais estão envolvidos em pesquisas e publicações científicas nesta área. A pesquisa dos termos eficácia e/ou segurança das diferentes vacinas em crianças e adolescentes com doenças reumatológicas foi realizada nas bases de Medline e Scielo, de 1966 até março de 2009, incluindo revisões, estudos controlados e relatos de casos. O grau de recomendação e o nível científico de evidências dos estudos foram classificados em quatro níveis para cada vacina. De um modo geral, as vacinas inativadas e de componentes são seguras nos pacientes com doenças reumatológicas, mesmo em uso de terapias imunossupressoras. Entretanto, vacinas com agentes vivos atenuados são, em geral, contraindicadas para os pacientes imunossuprimidos.
Incidence of infectious diseases is higher in children and adolescents with rheumatic diseases than in the general population due to disease activity, possible immune deficiency secondary to the disease itself, or the use of immunosuppressive drugs. Vaccination is effective in reducing morbidity and mortality in those patients. The objective of this study was to establish an evidence-based consensus on the efficacy and safety of vaccination in children and adolescents with rheumatic diseases. Passive immunization of patients and guidelines for people who live with immunosuppressed patients were also included. The 32 pediatric rheumatologists of the Rheumatology Department of the Pediatrics Society of São Paulo, (SPSP, from the Portuguese), São Paulo, SP, Brazil, and/or the Commission on Pediatrics Rheumatology of the Brazilian Society of Rheumatology are responsible for this consensus; some of those professionals are involved on research and scientific publications in this field. The words efficacy and/or safety of different vaccines in children and adolescents with rheumatologic diseases were searched in Medline and Scielo data bases from 1966 to March 2009, including reviews, controlled studies, and case reports. The degree of recommendation and the scientific evidence of the studies were classified in four levels for each vaccine. As a rule, inactive and protein components vaccines are safe for patients with rheumatologic diseases, even in the presence of immunosuppressive therapy. However, live attenuated vaccines are, in general, contraindicated for immunosuppressed patients.
Asunto(s)
Humanos , Niño , Adolescente , Artritis Juvenil , Consenso , Inmunización Pasiva , Lupus Eritematoso Sistémico , Enfermedades Reumáticas , Vacunación , VacunasRESUMEN
Catastrophic antiphospholipid (Asherson's) syndrome (CAPS) is known to be a severe variant (1%) of antiphospholipid syndrome, with a high rate of mortality (50%). The distinguishing feature of CAPS is microvascular thromboses in the presence of antiphospholipid antibodies. Molecular mimicry between beta2-glycoprotein I and infectious agents, endothelial cell activation and reduced fibrinolysis are the most frequently described pathophysiological mechanisms. Genetic risk factors have also been implicated in the onset of CAPS, although these have not yet been identified. There have been no randomized, controlled trials evaluating the efficacy of any medication on CAPS; however, when CAPS is suspected, aggressive multimodal treatment is required. Patients who receive a combination of anticoagulation therapy, glucocorticosteroids and plasma exchange with or without intravenous immunoglobulin show the best survival rates. Herein, we review the clinical and laboratory findings, diagnostic criteria, pathophysiology, treatment and prognosis of CAPS.