Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Ácido Úrico/sangue , Adulto , Fatores Etários , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/epidemiologia , Dislipidemias/epidemiologia , Feminino , Humanos , Hipertensão/epidemiologia , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Fatores SexuaisRESUMO
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.
RESUMO
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.
Assuntos
Pessoas Famosas , Lúpus Eritematoso Sistêmico/história , Pinturas/história , Febre Reumática/história , Sífilis/história , Áustria , Diagnóstico Diferencial , Encefalite/história , História do Século XIX , História do Século XXRESUMO
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.
Assuntos
Permeabilidade Capilar , Hipersensibilidade a Drogas/imunologia , Urticária/induzido quimicamente , Doença Aguda , Adulto , Criança , Citoplasma/metabolismo , Citoplasma/ultraestrutura , Hipersensibilidade a Drogas/etiologia , Células Endoteliais/metabolismo , Células Endoteliais/ultraestrutura , Fator XIIIa/metabolismo , Feminino , Humanos , Microscopia Eletrônica , Pessoa de Meia-Idade , Organelas/metabolismo , Organelas/ultraestrutura , Coloração e Rotulagem , Triptases/metabolismo , Urticária/imunologiaRESUMO
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.
Assuntos
Síndrome Antifosfolipídica/patologia , Impotência Vasculogênica/patologia , Lúpus Eritematoso Sistêmico/patologia , Pênis/patologia , Espermatozoides/patologia , Adolescente , Adulto , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/epidemiologia , Brasil/epidemiologia , Comorbidade , Estudos Transversais , Hormônios Gonadais/sangue , Humanos , Impotência Vasculogênica/sangue , Impotência Vasculogênica/epidemiologia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Pênis/irrigação sanguínea , Motilidade dos Espermatozoides , Espermatozoides/fisiologia , Testículo/diagnóstico por imagem , Testículo/patologia , Ultrassonografia , Adulto JovemRESUMO
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.
Assuntos
Hipersensibilidade a Drogas/etiologia , Hipersensibilidade a Drogas/imunologia , Hipersensibilidade a Drogas/virologia , Eosinofilia/induzido quimicamente , Eosinofilia/imunologia , Eosinofilia/virologia , Herpesviridae/imunologia , Hipersensibilidade a Drogas/terapia , Eosinofilia/terapia , Humanos , Fatores de Risco , SíndromeRESUMO
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.
Assuntos
Adipocinas/sangue , Síndrome Antifosfolipídica/sangue , Mediadores da Inflamação/sangue , Inflamação/sangue , Resistência à Insulina , Síndrome Metabólica/sangue , Adiponectina/sangue , Adulto , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/imunologia , Biomarcadores/sangue , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Estudos Transversais , Citocinas/sangue , Feminino , Humanos , Inflamação/diagnóstico , Inflamação/imunologia , Leptina/sangue , Modelos Lineares , Lipídeos/sangue , Masculino , Síndrome Metabólica/diagnóstico , Síndrome Metabólica/imunologia , Pessoa de Meia-Idade , Análise Multivariada , Nicotinamida Fosforribosiltransferase/sangue , Inibidor 1 de Ativador de Plasminogênio/sangue , Resistina/sangueRESUMO
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.
Assuntos
Autoanticorpos/sangue , Doença Mista do Tecido Conjuntivo/imunologia , Adulto , Biomarcadores/sangue , Estudos Transversais , Endotélio Vascular/imunologia , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Células Endoteliais da Veia Umbilical Humana , Humanos , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Doença Mista do Tecido Conjuntivo/sangue , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/imunologiaRESUMO
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.