RESUMEN
OBJECTIVE: To develop the second evidence-based Brazilian Society of Rheumatology consensus for diagnosis and treatment of lupus nephritis (LN). METHODS: Two methodologists and 20 rheumatologists from Lupus Comittee of Brazilian Society of Rheumatology participate in the development of this guideline. Fourteen PICO questions were defined and a systematic review was performed. Eligible randomized controlled trials were analyzed regarding complete renal remission, partial renal remission, serum creatinine, proteinuria, serum creatinine doubling, progression to end-stage renal disease, renal relapse, and severe adverse events (infections and mortality). The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to develop these recommendations. Recommendations required ≥82% of agreement among the voting members and were classified as strongly in favor, weakly in favor, conditional, weakly against or strongly against a particular intervention. Other aspects of LN management (diagnosis, general principles of treatment, treatment of comorbidities and refractory cases) were evaluated through literature review and expert opinion. RESULTS: All SLE patients should undergo creatinine and urinalysis tests to assess renal involvement. Kidney biopsy is considered the gold standard for diagnosing LN but, if it is not available or there is a contraindication to the procedure, therapeutic decisions should be based on clinical and laboratory parameters. Fourteen recommendations were developed. Target Renal response (TRR) was defined as improvement or maintenance of renal function (±10% at baseline of treatment) combined with a decrease in 24-h proteinuria or 24-h UPCR of 25% at 3 months, a decrease of 50% at 6 months, and proteinuria < 0.8 g/24 h at 12 months. Hydroxychloroquine should be prescribed to all SLE patients, except in cases of contraindication. Glucocorticoids should be used at the lowest dose and for the minimal necessary period. In class III or IV (±V), mycophenolate (MMF), cyclophosphamide, MMF plus tacrolimus (TAC), MMF plus belimumab or TAC can be used as induction therapy. For maintenance therapy, MMF or azathioprine (AZA) are the first choice and TAC or cyclosporin or leflunomide can be used in patients who cannot use MMF or AZA. Rituximab can be prescribed in cases of refractory disease. In cases of failure in achieving TRR, it is important to assess adherence, immunosuppressant dosage, adjuvant therapy, comorbidities, and consider biopsy/rebiopsy. CONCLUSION: This consensus provides evidence-based data to guide LN diagnosis and treatment, supporting the development of public and supplementary health policies in Brazil.
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Inmunosupresores , Nefritis Lúpica , Sociedades Médicas , Nefritis Lúpica/diagnóstico , Nefritis Lúpica/tratamiento farmacológico , Humanos , Inmunosupresores/uso terapéutico , Brasil , Creatinina/sangre , Proteinuria/diagnóstico , Proteinuria/etiología , Ácido Micofenólico/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Reumatología/normas , Rituximab/uso terapéutico , Biopsia , Ciclofosfamida/uso terapéutico , Leflunamida/uso terapéutico , Glucocorticoides/uso terapéutico , Hidroxicloroquina/uso terapéutico , Azatioprina/uso terapéutico , Inducción de Remisión , Ciclosporina/uso terapéutico , Medicina Basada en la Evidencia , Consenso , Progresión de la Enfermedad , Fallo Renal Crónico , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
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.
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Humanos , Femenino , Citometría de Flujo/métodos , Lupus Eritematoso Sistémico/patología , Pacientes/clasificación , Biomarcadores/análisis , Células T Asesinas NaturalesRESUMEN
A mucosectomia é utilizada durante a confecção de reservatórios ileais nas proctocolectomias por doença inflamatória intestinal. Entretanto, tem-se relatado aumento da incidência de obstruções intestinais em pacientes submetidos ao procedimento, sendo que o contato de uma grande área de submucosa poderia ser responsável pela ocorrência de estenoses, além de poder relacionar-se com aumento da pressão de ruptura desses segmentos. Com o objetivo de avaliar as alterações macroscópicas após a mucosectomia e a pressão de ruptura desses segmentos, foram estudados 16 ratos Holtzman distribuídos em dois grupos: A (n=8), anastomose colo-cólica em plano único extramucoso invertente com pontos separados de fio absorvível e B (n=8), mucosectomia e anastomose colocólica em plano único evertente com pontos separados de fio absorvível. No terceiro dia pós-operatório, foram estudadas as alterações macroscópicas e a pressão de ruptura dos segmentos colônicos contendo as anastomoses. Os resultados obtidos mostraram aumento na incidência e intensidade das aderências peritoneais nos animais do grupo B (p=0,01), incidência de 100% de estenoses das anastomoses, sem que ocorresse qualquer estenose no grupo A (p=0,01) e aumento não significativo na pressão de ruptura das anastomoses no grupo B (4,3 ± 2,4 mmHg vs. 10,5 ± 8,2 mmHg). Concluindo, a mucosectomia aumentou a reação inflamatória tecidual, sendo causa não só de aumento na pressão de ruptura como na incidência de estenoses de anastomoses colônicas de ratos.
The mucosectomy is used during the making of the ileal pouch in proctocolectomy due to inflammatory bowel disease. However, there are reports of increased incidence of intestinal obstructions in patients undergoing the procedure, when the contact of a large submucosal area could be responsible for the stenosis, and can relate to the increase of burst pressure of these segments. To evaluate the macroscopic alterations after the mucosectomy and the burst pressure of these segments, 16 Holtzman rats were studied divided into two groups: A (n = 8), colo-colic anastomosis in extra mucosal single invertent plan with separate absorbable sutures and B (n = 8), mucosectomy and colo-colic anastomosis in a single evertent plane with separate absorbable sutures. On the third post-operatory day the macroscopic alterations and burst pressure of the colony segments containing the anastomosis were studied. The found results showed increase in the incidence and intensity of peritoneal adherences in the animals of group B (p=0,01), incidence of 100% of the anastomosis steanosis, with no occurrence of stenosis in the group A (p=0,01), and no significant increase in the burst pressure in the group B (4,3 ± 2,4 mmHg vs. 10,5 ±8,2 mmHg). Conclusion : the mucosectomy increased the tissue inflammatory reaction, giving cause to the increase of the burst pressure as well as the incidence of colonic anastomosis steanaosis in rats.
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Animales , Ratas , Cicatrización de Heridas , Colon/cirugía , Anastomosis Quirúrgica , Ratas Sprague-DawleyRESUMEN
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.
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Femenino , Humanos , Persona de Mediana Edad , Síndrome de Felty/diagnóstico , Leishmaniasis Visceral/diagnóstico , Diagnóstico Diferencial , ReumatologíaRESUMEN
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.
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Humanos , Femenino , Persona de Mediana Edad , Cromoblastomicosis , Lupus Eritematoso SistémicoRESUMEN
Síndromes paraneoplásicas definem um grupo de sinais, sintomas e situações clínicas de disfunções metabólicas e envolvimento funcional de órgãos, resultado da ação do tumor contra o hospedeiro, mas sem relação direta com o tumor ou suas metástases. Podem indicar o primeiro sinal de malignidade ou indicar recidiva após o tratamento. A patogenia é complexa, envolvendo a produção tumoral de substâncias resultantes de alterações genéticas e imunológicas. Este artigo descreve as principais síndromes endócrinas, hematológicas, dermatologicas e neuromusculares, suas manifestações e sua patogenia.
Paraneoplastic syndromes define a group of signs, symptoms and clinical situations of metabolic dysfunction or organ-funtional involvement which are the result of the effects that the tumor exerts on the host. Their occurrence may be the first sign of malignancy or be a sign of disease relapse. The pathogenesis is complex and involves tumoral production of substances that result of genetic and imunological mechanisms. This article discribes the most important endocrinologic, hematologic, dermatologic and neuromuscular syndromes, including their pathogenesis and manifestations.