ABSTRACT
Abstract The pathogenesis of systemic lupus erythematosus (SLE) is complex. Few studies in Brazilian population have addressed cell phenotypes associated with immunological responses and their associations with SLE activity. The aim of this study is to investigate cell phenotypes associated to SLE diagnosis, treatment and activity. Twenty-eight SLE female patients (17 inactive, 11 active) and 10 healthy women were included in this study. Markers of natural killer (Nk), T and B cells in peripheral blood were evaluated by flow cytometry. Nkt cells were decreased only in SLE active patients. Activated CD4+, regulatory T FoxP3+ and B cells were decreased in both active and inactive SLE patients, compared to control group. The data corroborate the disruption of immune regulatory response in SLE patients and suggest phenotipic changes as possible biomarkers of SLE activity.
Subject(s)
Humans , Female , Flow Cytometry/methods , Lupus Erythematosus, Systemic/pathology , Patients/classification , Biomarkers/analysis , Natural Killer T-CellsABSTRACT
OBJECTIVE: This study has investigated whether high levels of Reticulocytes-C4d (R-C4d) and Platelets-C4d (P-C4d) reflecting recent activity in SLE patients are correlated with changes in natural anticoagulation components, coagulation activation and endothelial injury markers. METHODS: This study included three groups: 1) healthy women (control, nâ¯=â¯30); 2) women with low activity of the disease (SLEDAI 2â¯Kâ¯≤â¯4, nâ¯=â¯30); 3) women with active disease (moderate or high activity) (SLEDAI 2â¯Kâ¯>â¯4, nâ¯=â¯30). Median fluorescence intensity (MFI) of R-C4d and P-C4d were determined by flow cytometry using double labeling with specific monoclonal antibodies. Endothelial injury and hypercoagulability were evaluated by measuring Thrombomodulin and D-dimer levels. RESULTS: Higher MFI index of R-C4d were related to the recent activity of SLE, and higher expression of P-C4d indicated an elevated risk of thrombotic complications. Increased levels of soluble thrombomodulin and D-dimer were observed in patients with active SLE. CONCLUSION: R-C4d is helpful to monitor early disease activity and PC4-d may be an important tool to detect a prothrombotic phenotype in SLE. Elevated levels of D-dimer and thrombomodulin add value to P-C4d data and corroborate a hypercoagulable profile in women with SLE, contributing to an increased prothrombotic risk associated with inflammation.
Subject(s)
Blood Coagulation , Blood Platelets/metabolism , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/physiopathology , Peptide Fragments/blood , Reticulocytes/metabolism , Adult , Aged , Case-Control Studies , Complement C4b , Female , Humans , Middle Aged , Young AdultABSTRACT
Systemic lupus erythematosus (SLE) is an autoimmune disease of the connective tissue with a large spectrum of clinical manifestations. Immune deregulation leads to autoantibody and immune complexes overproduction, complement activation, and persistent tissue inflammation. Considering that the current diagnosis depends on the interpretation of the complex criteria established by the American College of Rheumatology and that the disease course is characterized by unpredictable activations and remissions, each patient develops different manifestations, and therefore, the discovery of specific biomarkers is urgently required. Therefore, this study aimed to identify putative biomarkers for active and inactive SLE potentially capable in distinguishing laboratorial SLE from other autoimmune diseases. The 2D-DIGE proteomics technique was used to evaluate the differential abundance of proteins between patients with active SLE, inactive SLE, patients with other autoimmune disease, and healthy individuals. Six proteins showed increased abundance in active SLE (A) and inactive SLE (I) compared to the C and O groups, but not between groups A and I. There were two transthyretin (TTR) fragments or proteins with a structure similar to TTR (accession numbers: PDB: 1GKO_A and 2PAB_A), retinol-binding protein 4 (RBP4) isoform X1 (no information in databases such as UNIPROT), and antibody fragments. Two proteins, APO-AIV and SP-40,40, were upregulated in group A than in O and C and in group I versus C, but not in group I versus O. Therefore, we suggest these proteins to be considered as candidates for the diagnosis of SLE.
Subject(s)
Biomarkers , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/diagnosis , Mass Spectrometry , Two-Dimensional Difference Gel Electrophoresis , Adult , Autoimmune Diseases/blood , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Case-Control Studies , Female , Humans , Lupus Erythematosus, Systemic/immunology , Male , Middle Aged , Proteomics/methods , Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization , Young AdultABSTRACT
Os autores relatam o caso de uma paciente com artrite reumatoide que evoluiu com grave neutropenia e esplenomegalia, sendo firmado o diagnóstico de Síndrome de Felty, que posteriormente desenvolveu Calazar. Ambas têm apresentação clínica e laboratorial semelhantes, tornando o diagnóstico diferencial difícil. O relato deste caso objetiva chamar a atenção para o reconhecimento da infecção por leishmaniose visceral em pacientes portadores de doenças reumáticas, assim como a possibilidade de um paciente com Calazar mimetizar um quadro de doença reumática sistêmica.
Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.
Subject(s)
Female , Humans , Middle Aged , Felty Syndrome/diagnosis , Leishmaniasis, Visceral/diagnosis , Diagnosis, Differential , RheumatologyABSTRACT
Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.
Subject(s)
Felty Syndrome/diagnosis , Leishmaniasis, Visceral/diagnosis , Diagnosis, Differential , Female , Humans , Middle Aged , RheumatologyABSTRACT
A granulomatose de Wegener é uma vasculite sistêmica que acomete vasos de pequeno e médio calibres. As manifestações clássicas ocorrem no trato respiratório superior, inferior e rins, mas outros órgãos podem ser envolvidos. O sistema auditivo pode ser freqüentemente acometido nas suas várias porções (orelha externa, média e/ou interna) com manifestações diversas. A perda auditiva neurossensorial pode ser conseqüência de alterações na orelha interna e deve ser precocemente reconhecida. O tratamento rápido e efetivo pode evitar danos irreversíveis. Descrevemos três casos de granulomatose de Wegener e hipoacusia sensorial, com respostas diferentes ao tratamento.
Wegener's granulomatosis is a primary systemic vasculitis that affects small and medium-sized vessels. The classical manifestations occur in the upper and lower respiratory tract and in the kidneys, but other organs may be involved. The auditory system can be frequently affect in its various portions (outer, middle and/or inner ear), showing different manifestations. Neurosensory hearing loss might be a consequence of changes in the inner ear and should be recognized early. Rapid and effective treatment may prevent irreversible damage. We report herein three cases of Wegener's granulomatosis and sensory hypoacusis, which responded differently to treatment.
ABSTRACT
A granulomatose de Wegener é uma vasculite sistêmica dos vasos de médio e pequeno calibre. Classicamente, há formação de granulomas com necrose no trato respiratório e glomerulonefrite necrosante. Embora seu acometimento mais comum envolva o trato respiratório superior, pulmões e rins, uma vasta gama de manifestações em vários órgãos e tecidos é descrita. Relatamos o caso de um paciente que, paralelamente às manifestações típicas da doença, desenvolveu algumas alterações raras como a parotidite e a paquimeningite hipertrófica. Diante de quadros graves, atípicos e/ou refratários ao tratamento convencional, torna-se necessário o aprofundamento no estudo e uso de novas armas terapêuticas.
Subject(s)
Humans , Male , Adult , Glomerulonephritis , Granuloma , Granulomatosis with Polyangiitis , Meningitis , Parotitis , VasculitisABSTRACT
Relatamos o caso de uma criança de oito meses de idade com infecção das vias aéreas superiores, seguida de irritabilidade e pelo aparecimento de lesões purpúricas na face, extremidades e pavilhões auriculares e edema de dorso dos pés e das mãos. Inicialmente recebeu dexametasona, ampicilina e cloranfenicol para tratamento de suposta meningococcemia. Entretanto, as lesões características e a boa evolução clínica do quadro levaram-nos ao diagnóstico de uma forma rara de vasculite cutânea: edema agudo hemorrágico da infância.
Subject(s)
Humans , Male , Infant , IgA Vasculitis , VasculitisABSTRACT
Hipertensão pulmonar grave é uma doença debilitante, com expectativa de vida reduzida, que acomete adultos jovens. Complicações pleuropulmonares no lúpus eritematoso sistêmico ocorrem em 50 por cento a 70 por cento dos pacientes. A hipertensão pulmonar grave no lúpus eritematoso sistêmico é rara e tem prognóstico reservado. Descreve-se, pela primeira vez, um paciente com hipertensão pulmonar grave associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio secundário que apresentou boa resposta ao uso do sildenafil oral, após falha do tratamento convencional com corticosteróides, ciclofosfamida, warfarin e diltiazem.
Subject(s)
Humans , Female , Adult , Hypertension/etiology , Hypertension/drug therapy , Lupus Erythematosus, Systemic/complications , Antiphospholipid Syndrome/complications , Vasodilator Agents/therapeutic use , Severity of Illness Index , Treatment OutcomeABSTRACT
Os autores descrevem um caso de lúpus eritematoso sistêmico (LES) em corticoterapia a longo prazo, que evoluiu com lesões cutâneas em membro superior esquerdo, sendo diagnosticada cromomicose causada por Fonsecaea pedrosoi. A prevalência da cromomicose é maior em pacientes imunocompetentes e sua associação com doenças imunossupressoras, incluindo as colagenoses, é rara.
Subject(s)
Humans , Female , Middle Aged , Chromoblastomycosis , Lupus Erythematosus, SystemicABSTRACT
Descreve-se a ocorrência de bloqueio cardíaco congênito em dois irmäos, em cuja mäe a avaliaçäo clínico-laboratorial mostrou-se compatível com o diagnóstico de lúpus eritematoso sistêmico. Os autores fazem uma revisäo da literatura, com ênfase nos aspectos clínicos e etiopatogêncios da associaçäo lúpus materno/bloqueo cardíaco congênito
Subject(s)
Infant, Newborn , Humans , Male , Female , Heart Block/congenital , Lupus Erythematosus, Systemic/congenital , Heart Block/complications , Brazil , Lupus Erythematosus, Systemic/complicationsABSTRACT
The authors present the case of a patient with juvenile anklosisng spondylitis who developed mesangial IgA nephropathym, which was diagnosed nine years after the beginning of the spondyloarthropathy. They also review the literature about the association fo these two diseases emphasizing the etiopathogenical mechanisms involved
Subject(s)
Adult , Humans , Male , Glomerulonephritis, IGA , Spondylitis, Ankylosing , Fluorescent Antibody Technique , Glomerulonephritis, IGA/diagnosis , Spondylitis, AnkylosingABSTRACT
Relata-se caso de paciente portadora de hepatite crônica persistente, que após cinco meses do quadro da hepatite desenvolveu crioglobulinemia mista policlonal, na forma de síndrome de Meltzer. Concluiu-se que muitos casos de "crioglobulinemia mista essencial", na verdade, säo secundárias a doenças hepáticas preexistentes
Subject(s)
Adult , Humans , Female , Arthritis/complications , Biopsy , Cryoglobulinemia/classification , Cryoglobulinemia/complications , Liver/pathology , Hepatitis, Chronic/complications , Purpura/complicationsABSTRACT
Seguiram-se 12 pacientes portadores de espondilite anquilosante juvenil. Todos do sexo masculino, nove brancos e três pretos, com idade ao inicio da moléstia variando entre 8-15 anos. Em oito pacientes a manifestaçäo inicial foi de artrite periférica, em dois essa artrite associou-se a lombalgia, em um lombalgia isolada e em outro uveíte antierior aguda e näo granulomatosa. Com a evoluçäo, oito pacientes apresentaram quadros de entesites. Dentre as manifestaçöes extra-articulares, observou-se: bloqueio atrioventricular em um, sobrecarga ventricular esquerda em um, sobrecarga ventricular direita e em um, nefropatia por depósitos de IgA em um e uveíte anterior näo granulomatosa em quatro pacientes. Concluiu-se que crianças do sexo masculino entre os 8-16 anos, com artrite de membros inferiores, queixas de entesites, presença de uveíte anterior aguda e näo granulomatosa, negatividade para o fator reumatóide e anticorpos antinucleares e presença de HLA-B27, devem ser investigadas à procura de espondilite anquilosante juvenil
