Síndrome de Guillain-Barré como forma de presentación de lupus eritematoso sistémico en paciente masculino / Guillain Barré Syndrome as a form of presentation of systemic lupus erythematosus in a male patient

El lupus eritematoso sistémico es una enfermedad reumática, autoinmune, inflamatoria, sistémica y crónica que se caracteriza por afectar principalmente pacientes femeninas en edad fértil. Su mecanismo patogénico se centra en la presencia de un proceso inflamatorio crónico que es el responsable de las manifestaciones clínicas y complicaciones de la enfermedad. Las manifestaciones clínicas se subdividen en articulares y extraarticulares. Entre estas últimas la afectación neurológica cobra especial atención tanto por su frecuencia de presentación elevada como por la gravedad de estas. El síndrome de Guillain-Barré es una enfermedad autoinmune, que puede aparecer como complicación del lupus eritematoso sistémico y se caracteriza por parálisis motora y otros síntomas y signos neurológicos. El objetivo del presente trabajo es reportar el caso de un paciente masculino, de 27 años de edad, que comienza con manifestaciones neuropáticas y se llega a diagnosticar un síndrome de Guillain-Barré que constituyó la forma de presentación del lupus eritematoso sistémico. Por ser una forma de presentación poco común, se considera importante su reporte para conocimiento de la comunidad médica(AU)

Systemic lupus erythematosus is a rheumatic, autoimmune, inflammatory, systemic and chronic disease that is characterized by mainly affecting female patients of childbearing age. Its etiopathogenic mechanism focuses on the presence of a chronic inflammatory process that is responsible for the clinical manifestations and complications of the disease. Clinical manifestations are subdivided into articular and extra-articular. Within the latter, neurological involvement receives special attention both due to its high frequency of presentation and its severity. Guillain-Barré syndrome is an autoimmune disease that can appear as a complication of lupus erythematosus and is characterized by motor paralysis and other neurological signs and symptoms. The objective of this paper is to report the case of a 27-year-old male patient who began with neuropathic manifestations and was diagnosed with Guillain-Barré syndrome, which was the presentation of systemic lupus erythematosus. Because it is an uncommon form of presentation, its report is considered important for the knowledge of the medical community(AU)

Amicrobial pustulosis of the folds: report of two cases / Pustulosis amicrobiana de los pliegues. Informe de dos casos

ABSTRACT Amicrobial Pustulosis of the Folds is a relapsing, chronic and rare neutrophilic dermatosis, characterized by papulopustular, eczematous and aseptic lesions on skin folds. This disorder usually occurs predominantly in females (30 years of age average) with a history of an autoimmune disorder, especially systemic lupus erythematosus. There is no standard therapy, but systemic corticosteroids, alone or in combination with other immunosuppressive drugs, are usually the first-line therapy. We report two females aged 37 and 20 years with the disease but without associated autoimmune diseases. They were successfully treated with non-steroidal treatments.

La pustulosis amicrobiana de los pliegues es una dermatosis neutrofílica crónica, recurrente y poco común. Se caracteriza por lesiones pápulo-pustulosas, eczematosas y asépticas de los pliegues cutáneos. Este cuadro se presenta predominantemente en mujeres de alrededor de 30 años con enfermedades autoinmunes, especialmente lupus eritematoso sistémico. No existe un tratamiento estándar pero los corticoides solos o con inmunosupresores se usan de primera línea. Informamos dos mujeres de 27 y 20 años sin patología autoinmune, con la enfermedad. Ellas fueron tratadas exitosamente sin usar esteroides.

Non-lupus full-house nephropathy: a case series / Nefropatia "full-house" não relacionada ao lúpus: uma série de casos

Abstract Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disease. However, some patients may exhibit a histological pattern of kidney injury, with characteristics indistinguishable from lupus nephritis, but without presenting any extrarenal symptoms or serologies suggestive of SLE. Such involvement has recently been called non-lupus full-house nephropathy. The objective is to report a series of clinical cases referred to the Laboratory of the Federal University of Maranhão that received the diagnosis of "full-house" nephropathy unrelated to lupus, upon immunofluorescence and to discuss its evolution and outcomes. Non-lupus full-house nephropathy represents a diagnostic and therapeutic challenge, because it is a new entity, which still needs further studies and may be the initial manifestation of SLE, isolated manifestation of SLE or a new pathology unrelated to SLE.

Resumo O lúpus eritematoso sistêmico (LES) é uma doença inflamatória crônica autoimune multissistêmica. Alguns pacientes, contudo, podem exibir um padrão histológico de lesão renal, com características indistinguíveis da nefrite lúpica, porém sem apresentar quaisquer sintomas extrarrenais ou sorologias sugestivas de LES. Tal acometimento tem sido recentemente denominado nefropatia "full-house" não relacionada ao lúpus. O objetivo é relatar uma série de casos clínicos encaminhados ao Laboratório da Universidade Federal do Maranhão que receberam o diagnóstico de nefropatia "full-house" não relacionada ao lúpus à imunofluorescência e discutir sua evolução e desfechos. A nefropatia "full-house" não relacionada ao lúpus representa um desafio diagnóstico e terapêutico por ser uma entidade nova, que ainda necessita de maiores estudos e pode ser a manifestação inicial do LES, manifestação isolada do LES ou uma patologia nova não relacionada ao LES.

Enterite mesentérica como complicação rara do lúpus eritematoso sistêmico / Mesenteric enteritis as a rare complication of systemic lupus erythematosus

A dor abdominal no paciente com lúpus eritematoso sistêmico tem amplo espectro clínico, variando desde condições inespecí- ficas, como diarreia e vômitos, até eventos de importante morbi- mortalidade, como o abdome agudo inflamatório e/ou perfura- tivo. A seguir, descreve-se um caso de paciente do sexo feminino, de 23 anos, internada por dor abdominal associada a vômitos e à diarreia crônica e progressiva. Foi diagnosticada com lúpus eritematoso sistêmico há 2 anos. Durante a internação, evoluiu com quadro de abdome agudo, e foi realizada tomografia compu- tadorizada de abdome, revelando importante edema de parede intestinal difuso. Isso, somado a alterações clínico-laboratoriais, permitiu o diagnóstico de enterite lúpica. Foi realizado tratamen- to conservador, com corticoterapia e terapia de suporte com correção de distúrbios eletrolíticos severos, sendo iniciado ciclo- fosfamida, com resolução dos sintomas gastrintestinais.

Abdominal pain in patients with systemic lupus erythematosus has a broad clinical spectrum, ranging from nonspecific symp- toms, such as diarrhea and vomiting, to events of significant morbidity and mortality, such as acute inflammatory and/or per- forating abdomen. This article describes a case of a 23-year-old female patient hospitalized for abdominal pain, associated with vomiting and progressive chronic diarrhea. She was diagnosed with systemic lupus erythematosus 2 years ago. During hospita- lization, the patient progressed with acute abdomen, and an ab- dominal computed tomography scan was performed, revealing major diffuse intestinal wall edema. This, added to clinical and laboratories alterations, allowed the diagnosis of lupus enteritis. A conservative treatment with corticotherapy and supportive therapy with correction of severe electrolyte disturbances were initiated, as well as the prescription of cyclophosphamide, with resolution of gastrointestinal symptoms.

Inflammation in Cardiovascular Disease: From Basic Concepts to Clinical Application

Abstract Although low-density lipoprotein cholesterol is central to the development and progression of atherosclerosis, the role of inflammation in the atherosclerotic process is becoming better understood and appreciated. Chronic inflammatory conditions such as rheumatoid arthritis, lupus, psoriasis, HIV infection, and inflammatory bowel disease have all been shown to be associated with an increased blood levels of inflammatory biomarkers and increased risk of cardiovascular events. Evidence from observational studies suggests that anti-inflammatory therapy decreases this risk in these conditions. Clinical trials of anti-inflammatory drugs in patients with coronary disease have yielded mixed results. Drugs that have failed in recent trials include the P38 MAP kinase inhibitor losmapimod, the phospholipase A2 inhibitors darapladib and varespladib, and methotrexate. Canakinumab, an interleukin-1β inhibitor, reduced cardiovascular events in patients with coronary disease in the Canakinumab Anti-inflammatory Thrombosis Outcome Study (CANTOS). Canakinumab increased the rate of fatal infections in CANTOS and is very expensive; it is thus unlikely to be widely used for risk reduction in cardiology. On the other hand, colchicine is a safe and inexpensive anti-inflammatory drug. In the Colchicine Cardiovascular Outcomes Trial (COLCOT), where patients within 30 days of a myocardial infarction were randomized to low-dose colchicine or placebo and followed for a median of almost 2 years, colchicine treatment was associated with a 23% reduction (p=0.02) in cardiovascular events. Newer studies with anti-inflammatory drugs have the potential to improve outcomes of patients with atherosclerosis, just as low-density lipoprotein cholesterol-lowering drugs have done over the past two decades.

Cambio de diagnóstico y de categoría diagnóstica en pacientes con artritis idiopática juvenil en 7 años de seguimiento / Change of diagnosis and diagnostic category in patients with juvenile idiopathic arthritis within 7 years of follow-up

Resumen: Introducción: Al menos 50% de los pacientes pediátricos portadores de artritis idiopática juvenil (AIJ) continuará control en reumatología adulto. La clasificación de la Liga Internacional de Asociaciones de Reumatología (ILAR) vigente, actualmente en revisión, difiere de la clasificación de las artritis inflamatorias del adulto. Se ha reportado cambios de categoría en 10,8% de los pacientes durante el seguimiento. Objetivo: Analizar los pacientes con AIJ seguidos al menos 7 años para objetivar cambios de diagnós tico en la transición, e identificar factores de mal pronóstico funcional. Pacientes y Método: Estudio retrospectivo en base a registros clínicos. Se incluyó a la totalidad de los pacientes con AIJ controla dos en policlínico pediátrico del Hospital de Puerto Montt entre el año 2005 y 2017, que cumplieron siete o más años de seguimiento. Se realizó análisis descriptivo en base a variables clínicas: categoría diagnóstica, tiempo de evolución al diagnóstico, actividad clínica y serológica, y tiempo de evolución al inicio de la terapia farmacológica. Resultados: Se evaluaron 18 pacientes, 3 Oligo-articular (OA) persistente, 1 OA extendida, 4 Poli-articular (PA) factor reumatoide (FR) negativo, 4 PA FR positivo, 5 Sistémicas, 1 Psoriática, todos con seguimiento mayor a 7 años. Once de 18 niños fueron transfe ridos a adultos. Tres de 11 cambiaron de diagnóstico a Artritis Reumatoide (AR) más otra enferme dad autoinmune: Síndrome de Sjögren + Lupus eritematoso sistémico, Púrpura trombocitopénico inmune, Enfermedad autoinmune no clasificada y cinco de 11 niños de categoría ILAR: OA a Artritis reumatoide juvenil, OA extendida a PA FR negativo, 3 Sistémicas a PA FR negativo. Edad de inicio, formas poli-articulares, retrasos en diagnóstico y comienzo de terapia se asociaron a secuelas e infla mación persistente. Conclusiones: Ocho de once pacientes transferidos cambiaron denominación diagnóstica y/o presentaron otras enfermedades autoinmunes. Algunos factores de mal pronóstico deben mejorar.

Abstract: Introduction: At least 50% of pediatric patients with Juvenile Idiopathic Arthritis (JIA) will require continued fo llow-up in adult rheumatology. The present International League of Associations for Rheumatology (ILAR) classification, currently under revision, differs from its classification of inflammatory arthritis in adults. Category changes have been reported in 10.8% of patients during follow-up. Objective: To analyze JIA patients in follow-up for at least 7 years to detect diagnosis changes during transition to adult care, identifying factors of poor functional prognosis. Patients and Method: Retrospective study based on medical records of JIA patients seen at the pediatric polyclinic of the Puerto Montt Hospital between 2005 and 2017, who were monitored for at least 7 years. Descriptive analysis was performed according to clinical variables: diagnostic category, evolution before diagnosis, clinical and serological activity, and evolution before starting drug therapy. Results: We evaluated 18 pa tients, corresponding to 3 patients with persistent oligoarticular arthritis (OA), 1 with extended OA, 4 with polyarticular arthritis (PA) rheumatoid factor (RF) negative, 4 with PA RF positive, 5 with syste mic JIA, and 1 with psoriatic arthritis, all have had follow-up more than 7 years. 11 out of 18 patients transitioned to adult care. Three out of 11 patients changed diagnosis to Rheumatoid Arthritis (RA) plus another autoimmune disease such as Sjögren's Syndrome + Systemic Lupus Erythematosus, Immune thrombocytopenia, or unclassified autoimmune disease, and 5 out of 11 children changed ILAR category from OA to Juvenile Rheumatoid Arthritis, extended OA to PA RF negative, and 3 from Systemic arthritis to PA RF negative. Age of onset, polyarticular forms, delay in diagnosis, and the start of therapy were associated with sequelae and persistent inflammation. Conclusions: Eight of the eleven JIA patients who transitioned to adult care changed their diagnosis or presented other autoimmune diseases. Some factors of poor prognosis must improve.

Dry eye in systemic lupus erythematosus patients / Olho seco em pacientes com lúpus eritematoso sistêmico

Abstract Objective: To study the association of dry eye with lupus disease activity and cumulative damage. To verify if epidemiological, treatment and autoantibody profile of SLE (systemic Lupus erythematosus) patients influence the presence of dry eye. Methods: We studied 70 SLE patients for the presence of dry eye by Schirmer test, disease activity by SLEDAI (SLE-Disease activity index) and cumulative damage by SLICC/ACR DI (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index). Patients were also submitted to the OSDI (Ocular Surface Disease Index) questionnaire. Epidemiological and treatment data and autoantibody profile were extracted from the charts. Results: Dry eye by Schirmer test was present in 51.4% of the sample. No association of the presence of dry eye with SLEDAI and SLICC DI were found (p = ns). Subjective symptoms of dry eye measured by OSDI showed a modest correlation with SLEDAI (Spearman rho = 0.32). Treatment profile did not influence in the presence of dry eye that was more common in older patients (p < 0.0001). Anti dsDNA had a negative association with the presence of positive Schirmer test (p = 0.0008). Conclusions: Dry eye detected by Schirmer test in SLE patients has no association with disease activity nor cumulative damage. Anti dsDNA seems to have a protective effect in this context.

Resumo Objetivos: Estudar a associação do olho seco com a atividade do lúpus eritematoso sistêmico (LES) e seus danos cumulativos. Verificar se o perfil epidemiológico, de tratamento e de auto anticorpos de pacientes com LES influencia a presença de olho seco. Métodos: Foram estudados 70 pacientes com LES para a presença de olho seco pelo teste de Schirmer, atividade da doença por SLEDAI (SLE Disease Activity Index) e dano cumulativo por SLICC/ACR DI (Clínicas Colaborativas Internacionais de Lúpus Eritematoso Sistêmico/American College of Rheumatology Damage Index). Os pacientes também foram submetidos ao questionário OSDI (índice de doenças da superfície ocular). Os dados epidemiológicos e de tratamento e o perfil de auto anticorpos foram extraídos dos prontuários. Resultados: Olho seco pelo teste de Schirmer esteve presente em 51,4% da amostra. Nenhuma associação da presença de olho seco com SLEDAI e SLICC/ACR DI foi encontrada (p = ns). Os sintomas subjetivos do olho seco medidos por OSDI mostraram uma correlação modesta com SLEDAI (Rho de Spearman = 0,32) . O perfil do tratamento não influenciou na presença de olho seco que era mais comum em uns pacientes mais idosos (p < 0, 1). Anti dsDNA teve uma associação negativa com a presença de teste positivo de Schirmer (p = 0, 8). Conclusões: Olho seco detectado pelo teste de Schirmer em pacientes com LES não tem associação com atividade da doença nem dano cumulativo. Anti dsDNA parece ter um efeito protetor neste contexto.

Recaída de enfermedad entre pacientes hispanos lúpicos con enfermedad renal crónica en hemodiálisis. Presentación de casos / Relapse of disease among lupus Hispanic patients with chronic kidney disease treated with hemodialysis. Case Presentations

Introducción: Los pacientes con lupus eritematoso sistémico (LES) presentan una disminución de la actividad de la enfermedad cuando evoluciona a una enfermedad renal crónica (ERC). Se han publicado recaídas de LES en esta etapa en diferentes grupos étnicos, aunque no pacientes hispanos, tradicionalmente asociados a peor pronóstico. Objetivo: Describir los casos de pacientes con ERC en hemodiálisis con diagnóstico de recaída de la enfermedad. Presentación de casos: Se presentan 4 casos, 2 varones y 2 mujeres con una edad promedio de 28 años. Tuvieron un promedio de score de SLEDAI sin criterios renales de 20.25, quienes presentaron exacerbaciones de diferentes características. Todos recibieron tratamiento inmunosupresor y fallecieron 2 pacientes. Conclusiones: Nuestro sistema de salud debería establecer estrategia de seguimiento adecuado a estos pacientes con evaluaciones frecuentes de la actividad de su enfermedad de tal forma que permita hacer un diagnóstico y tratamiento temprano. Nuestro sistema de salud debe instaurar medidas de seguimiento de los pacientes con LES en hemodiálisis para el diagnóstico y tratamiento temprano de las recaídas por LES(AU)

Introduction: Patients with systemic lupus erythematosus (SLE) have a decrease in the activity of the disease when it evolves to chronic kidney disease (CKD). Relapses of SLE have been published in this stage in different ethnic groups, but not in Hispanic patients who are traditionally associated with a worse prognosis. Objective: To describe the cases of patients with CKD undergoing hemodialysis with a diagnosis of disease relapse. Case presentations: We present 4 cases (2 men and 2 women) with an average age of 28 years. They had an average SLEDAI score without renal criteria of 20.25, and presented exacerbations of different characteristics. All these patients received immunosuppressive treatment and 2 of them died. Conclusions: Our health system should establish adequate follow-up measures for patients with frequent evaluations of the activity and the disease so that it allows to make an early diagnosis and treatment. Our health system should implement new measures for the follow-up of patients with SLE receiving hemodialysis for the early diagnosis and treatment of relapses due to SLE(AU)

Síndrome de seudotumor cerebral en una mujer embarazada y con lupus eritematoso sistémico / Pseudotumor cerebri syndrome in a pregnant woman with systemic lupus erythematous

Resumen El lupus eritematoso sistémico es una enfermedad autoinmunitaria crónica que afecta múltiples sistemas orgánicos, incluido el sistema nervioso central. El seudotumor cerebral es un síndrome clínico que se caracteriza por aumento de la presión intracraneal en ausencia de lesiones que ocupen espacio u otra causa detectable, que afecta con frecuencia a mujeres jóvenes y obesas. Se presenta el caso de una mujer con diagnóstico de seudotumor cerebral y lupus eritematoso sistémico diagnosticado de novo durante el embarazo.

Abstract Systemic lupus erythematous is a chronic multi-systemic autoimmune disease that affects multiple organ systems, including the central nervous system. Pseudotumor cerebri is a disorder associated with increased intracranial pressure in the absence of a space-occupying lesion or other identifiable cause that affects young and obese women. We present the case of a pregnant woman with both pseudotumor cerebri and a new diagnosis of active systemic lupus erythematous.

Diagnóstico simultâneo de lúpus eritematoso sistêmico e chikungunya / Simultaneous diagnosis of systemic erythematosus lupus and chikungunya

Chikungunya é uma arbovirose caracterizada por febre alta, poliartralgia periférica, exantema, mialgia axial e intensa fadiga. Até 65% dos pacientes apresentam recuperação total dos sintomas em 4 semanas, contudo as dores articulares podem persistir por um período de 12 meses após a infecção em 4,1% dos pacientes e por até dois anos em 1,6% dos indivíduos acometidos. Um ano após a infecção 0,3% dos pacientes podem apresentar artrite inflamatória crônica. Os pacientes que permanecem com dores musculoesqueléticas e artrite pós-infecciosa são frequentemente tratados com drogas imunossupressoras. Por poderem apresentar quadro clínico semelhante e pelo fato de infecção ser comum nos pacientes portadores de doenças reumatológicas, o lúpus eritematoso sistêmico (LES) é uma afecção que deve ser abordada como diagnóstico diferencial em regiões endêmicas para essa arbovirose. Sabendo-se do crescente número de casos de chikungunya no Brasil e da relação fortemente estabelecida entre aumento de morbimortalidade em lúpicos nos quadros infecciosos, faz- se necessária uma ampla discussão dos casos emergentes. (AU)

Chikungunya is an arbovirose characterized by high fever, severe peripheral polyarthralgia, exanthema, axial myalgia and intense fatigue. Up to 65% of patients present complete recovery of symptoms at 4 weeks, however, joint pain may persist for a period of 12 months after infection in 4.1% of patients and for up to 2 years in 1.6% of affected individuals. One year after infection, 0.3% of patients may present with chronic inflammatory arthritis. Patients who remain with musculoskeletal pain and post-infectious arthritis are often treated with Immunosuppressive drugs. Due to the presence of similar clinics and the fact that double infection is common in patients with rheumatologic diseases in some populations, systemic lupus erythematosus (SLE) is a condition that must be addressed during the stipulation of differential diagnosis in regions endemic to arboviruses. Given the increasing number of cases of chikungunya in developing countries and the strongly established relationship between increased morbidity and mortality in lupus in infectious diseases, a broad discussion of emerging cases is required. (AU)

Chronic low back pain in patients with systemic lupus erythematosus: prevalence and predictors of back muscle strength and its correlation with disability / Lombalgia crônica em pacientes com lúpus eritematoso sistêmico: prevalência e preditores da força muscular de extensão de tronco e sua correlação com a incapacidade

Abstract Objective: To determine the prevalence of Chronic Low Back Pain and predictors of Back Muscle Strength in patients with Systemic Lupus Erythematosus. Methods: Cross-sectional study. Ninety-six ambulatory patients with lupus were selected by non-probability sampling and interviewed and tested during medical consultation. The outcomes measurements were: Point prevalence of chronic low back pain, Oswestry Disability Index, Tampa Scale of Kinesiophobia, Fatigue Severity Scale and maximal voluntary isometric contractions of handgrip and of the back muscles. Correlation coefficient and multiple linear regression were used in statistical analysis. Results: Of the 96 individuals interviewed, 25 had chronic low back pain, indicating a point prevalence of 26% (92% women). The correlation between the Oswestry Index and maximal voluntary isometric contraction of the back muscles was r = −0.4, 95% CI [−0.68; −0.01] and between the maximal voluntary isometric contraction of handgrip and of the back muscles was r = 0.72, 95% CI [0.51; 0.88]. The regression model presented the highest value of R 2 being observed when maximal voluntary isometric contraction of the back muscles was tested with five independent variables (63%). In this model handgrip strength was the only predictive variable (β = 0.61, p = 0.001). Conclusions: The prevalence of chronic low back pain in individuals with systemic lupus erythematosus was 26%. The maximal voluntary isometric contraction of the back muscles was 63% predicted by five variables of interest, however, only the handgrip strength was a statistically significant predictive variable. The maximal voluntary isometric contraction of the back muscles presented a linear relation directly proportional to handgrip and inversely proportional to Oswestry Index i.e. stronger back muscles are associated with lower disability scores.

Resumo Objetivo: Determinar a prevalência de lombalgia crônica (LBC) e os preditores de força muscular nas costas (FMC) em pacientes com lúpus eritematoso sistêmico (LES). Métodos: Estudo transversal. Selecionaram-se 96 pacientes ambulatoriais com LES por amostragem não probabilística, entrevistados e testados durante consultas médicas. As medidas de desfecho foram: prevalência ocasional de LBC, Índice de Incapacidade de Oswestry, Escala Tampa para Cinesiofobia, Escala de Gravidade da Fadiga e contrações isométricas voluntárias máximas (CIVM) de preensão manual e dos músculos das costas. Usaram-se o coeficiente de correlação e a regressão linear múltipla na análise estatística. Resultados: Dos 96 indivíduos entrevistados, 25 apresentavam LBC, o que indicou uma prevalência circunstancial de 26% (92% mulheres). A correlação entre o Índice de Incapacidade de Oswestry e a contração isométrica voluntária máxima dos músculos das costas foi de r = -0,4, IC 95% [-0,68; -0,01] e entre a CIVM de preensão manual e dos músculos das costas foi de r = 0,72, IC 95% [0,51; 0,88]. O modelo de regressão apresentou o maior valor de R2 observado quando a CIVM dos músculos das costas foi testada com cinco variáveis independentes (63%). Nesse modelo, a força de preensão manual foi a única variável preditiva (ß = 0,61, p = 0,001). Conclusões: A prevalência de LBC em indivíduos com LES foi de 26%. A CIVM dos músculos das costas foi 63% prevista por cinco variáveis de interesse. No entanto, apenas a força de preensão manual foi uma variável preditiva estatisticamente significativa. A CIVM dos músculos das costas apresentou uma relação linear diretamente proporcional à força de preensão manual e inversamente proporcional ao Índice de Incapacidade de Oswestry (ou seja, músculos das costas mais fortes estão associados a menores pontuações de incapacidade).

Manifestaciones orales de lupus eritematoso sistémico: a propósito de un caso / Oral clinical features of systemic lupus erythematosus a clinical case

El Lupus Eritematoso Sistémico (LES) es una enfermedad crónica autoinmune con múltiples manifestaciones clínicas, que pueden ir desde el decaimiento general con presencia de lesiones cutáneas, hasta un compromiso sistémico severo afectando diversos órganos. Como en otras enfermedades de este tipo, puede verse comprometida la mucosa oral presentando zonas eritematosas, erosiones y úlceras, como también lesiones blancas discoideas, manifestaciones que se observan más intensas cuanto más descompensado se halla el cuadro general. Se presenta el caso de una paciente joven con diagnóstico de LES desde hace tres años, portadora de lesiones faciales, labiales y gingivitis asociada a Biofilm bacteriano y otros factores locales contribuyentes. Es importante que los cirujano-dentistas estén atentos a las manifestaciones que el LES puede presentar en la boca, como una de sus primeras evidencias clínicas, y así contribuir a su diagnóstico oportuno, derivando al enfermo al médico especialista. De igual forma, es importante que el médico considere el tratamiento de las lesiones orales, que suelen afectar de forma importante la calidad de vida de estos enfermos.

Systemic lupus erythematosus is a chronic auto-immune disease with multiple clinical expressions varying from feeling generally run down with cutaneous lesions to severe systemic compromise affecting several organs. As with similar diseases there may be involvement of the oral mucosa, with areas of erythema, erosions and ulcers as well as white discoid lesions, all more pronounced with increasing disease severity. We present the case of a young girl diagnosed with systemic lupus erythematosus three years before who presented facial, lip and gingival lesions associated with bacterial biofilm and other contributing local factors. It is important that dental surgeons be aware of the possibility of finding systemic lupus erythematosus lesions in a patient´s mouth, it being one of the first signs of the disease, thereby helping to reach a timely diagnosis by referring the patient to a specialist. Similarly, the physician should consider treating oral lesions as these have an important effect on the patient´s quality of life.

Lupus cystitis presenting with hidronephrosis and gastrointestinal involvement / Cistite lúpica apresentando hidronefrose e envolvimento gastrointestinal

Abstract Introduction: Lupus cystitis is a rare manifestation of systemic lupus erythematosus, characterized by thickening of the bladder wall, associated with ureterohydronephrosis. In several cases gastrointestinal symptoms are the main manifestation. The optimal immunosuppressive regimen is still unknown. Methods: 16-year-old girl with systemic lupus erythematosus was admitted with dysuria, renal impairment, diarrhea, abdominal pain, nausea and vomiting. An abdominal CT scan demonstrated moderate bilateral dilation from the pelvis to the ureterovesical junction, and the bladder exhibited reduced capacity and diffusely thickened walls. A diagnosis of chronic interstitial cystitis was performed and treatment with high dose methylprednisolone resulted in a significant relief of gastrointestinal and urinary symptoms and a reduction of the bladder thickness with improvement of their capacity. Conclusion: Lupus cystitis is a rare and underdiagnosed urinary manifestation of LES and the presence of mild urinary symptoms and abdominal pain may alert about this possibility.

Resumo Introdução: A Cistite Lúpica é uma manifestação rara do Lúpus Eritematoso Sistémico, caracterizada pelo espessamento da parede da bexiga, podendo estar associada com ureterohidronefrose. Em vários casos, os sintomas gastrointestinais são a principal manifestação e ainda não se sabe qual o regime imunossupressor mais adequado nesta situação. Método: Uma jovem de 16 anos de idade com lúpus eritematoso sistémico foi admitida com disúria, insuficiência renal, diarreia, dor abdominal, náuseas e vómitos. A tomografia computadorizada de abdome demonstrou dilatação moderada de ureter e pelve renal bilateralmente e bexiga exibindo capacidade reduzida e paredes difusamente espessadas. Foi feito o diagnóstico de cistite intersticial crónica e o tratamento com dose elevada de metilprednisolona resultou em um alívio significativo dos sintomas urinários e gastrointestinais e uma redução da espessura da bexiga, com melhora da sua capacidade. Conclusão: A Cistite Lúpica é uma manifestação urinária rara e subdiagnosticada do LES e a presença de sintomas urinários leves e dor abdominal devem alertar para essa possibilidade diganóstica.

Síndrome de superposición: lupus eritematoso sistémico y hepatitis autoinmune en una adolescente / An Overlap Syndrome involving systemic lupus erythematosus and autoimmune hepatitis in an adolescent girl

La superposición del lupus eritematoso sistémico y la hepatitis autoinmune se describe ocasionalmente. Aunque ambas enfermedades pueden compartir ciertos hallazgos, como poliartralgias, hipergammaglobulinemia y anticuerpo antinúcleo positivo, son consideradas dos diferentes. Se presenta a una paciente de 14 años con lupus eritematoso sistémico, que, luego de dos años, consultó por ictericia. Sin antecedentes de ingesta de drogas, alcohol o exposición a virus hepatotropos. Tenia un aumento de las enzimas hepáticas con anticuerpos antinúcleo, anti-ADN de doble cadena y LKM 1 positivos. La biopsia hepática mostró una hepatitis de interfase con infiltrado linfoplasmocitario. De esta manera, cumplia con los criterios diagnósticos tanto para lupus eritematoso sistémico como para hepatitis autoinmune. Tratada con corticoides y micofenolato mofetil, mejoró su clinica y laboratorio. Conclusión. La hepatitis autoinmune puede ocurrir en el curso del lupus eritematoso sistémico. Un diagnóstico temprano es importante para prevenir el avance de la enfermedad; es obligatoria la realización de la biopsia hepática.

Overlap of systemic lupus erythematosus and autoimmune hepatitis is a rare condition, occasionally described. Both autoimmune diseases can share common autoimmune features such as polyartralgia, hypergammaglobulinemia and positive antinuclear antibody, but they have been considered as two different entities. We report a 14 year old female patient with systemic lupus erythematosus who developed autoimmune hepatitis two years later. She had jaundice with no history of viral infections, drug intake, alcohol abuses or exposition to blood products. She was positive for antinuclear antibody, double stranded DNA antibody and LKM 1 antibody. Histological examination of the liver showed interface hepatitis with lymphoplasmacytic cell infiltration. The patient satisfied the international criteria for both systemic lupus erythematosus and Type 2 autoimmune hepatitis. Clinical symptoms and laboratory findings improved under treatment with steroids and mycophenolate mofetil. Conclusion. Autoimmune hepatitis may occur during the course of systemic lupus erythematosus and an early diagnosis is important for preventing advanced liver disease. Definitive diagnosis of autoimmune hepatitis requires confirmation by means of a liver biopsy.

Avaliação clínica e microbiológica relacionada à doença periodontal em pacientes com lúpus eritematoso sistêmico / Clinical and microbiological analysis of parameters related to periodontal disease in systemic lupus erythematous patients

O lúpus eritematoso sistêmico (LES) é uma doença autoimune caracterizada por hiperatividade imunológica crônica pela ação de autoanticorpos que afetam diversos órgãos. Embora o uso crônico de imunossupressores predisponha o paciente a infecções, poucas pesquisas avaliaram uma possível associação entre doença periodontal e LES. Os objetivos deste trabalho foram investigar, por meio de estudo caso-controle, a prevalência e a gravidade da doença periodontal em pacientes com LES, e identificar e quantificar as principais bactérias periodontais presentes no biofilme subgengival. Foram incluídas 60 mulheres em atendimento no Setor de Reumatologia do Hospital Universitário de Brasília, de 20 a 65 anos de idade, sendo subdivididas em LES-A (ativo; n= 31) e LES-I (inativo; n=29). O grupo controle foi composto por 31 mulheres com os mesmos critérios de inclusão, porém sem doenças sistêmicas. As pacientes foram avaliadas quanto às medidas de profundidade de sondagem (P.S), perda de inserção clínica (PIC), índice de sangramento do sulco (ISS) e índice de placa (IPl) no exame inicial. Foram coletados biofilmes subgengivais dos quatro sítios mais profundos para identificação e quantificação de periodontopatógenos por meio de hibridação DNA-DNA checkerboard. Não houve diferenças estatisticamente entre os grupos relativamente aos parâmetros clínicos periodontais, exceto para o ISS, que foi menor no LESA (11,19% ± 14,62%) comparativamente ao grupo controle (17,30% ± 14,88%), porém sem diferenças quando comparado com o grupo LES-I (11,34% ± 11,59%). Houve baixa prevalência de bolsas periodontais, de PIC ≥ 4 mm e de espécies de Actinomyces em todos os grupos. Verificou-se aumento na contagem de bactérias do complexo vermelho no grupo LES-I (4,07 x 105; 95% CI: 0,16-0,79) em relação ao grupo controle (2,50 x 105; 95% CI: 1,23-3,77), com diferenças estatisticamente significante apenas referente ao grupo LES-A (p< 0,05; Kruskal Wallis pós-teste Dunn; 0,45 x 105; 95% CI: 0,16-0,79). Os resultados desse estudo demonstraram que os parâmetros periodontais são semelhantes entre pacientes com LES e grupo controle. O grupo de LES-I apresentou maior tempo dessa doença; aumento da contagem de microorganismos (especialmente dos complexos vermelho e verde em amostras de biofilme subgengival) e pior condição periodontal.(AU)

Systemic lupus erythematous (SLE) is an autoimmune disorder characterized by chronic immunological hyperactivity resultant from the action of autoantibodies, affecting many organs. Although the use of immune suppressors may predispose infections, few studies have investigated the prevalence and severity of periodontal disease in SLE patients. The aim of this study is to investigate the prevalence and severity of periodontal disease in SLE patients and the sub gingival levels of different pathogens. A total of 60 women attending of Brasília University Hospital, aged 18-65 years, were invited to participate in the study. SLE patients were allocated in two subgroups according with disease activity: SLE-A (active disease; n= 31) and SLE-I (inactive disease; n= 29). A number of 31 systemically healthy women at the same age range composed control group. Patients were clinically examined according to probing depth (PD), clinical attachment level (CAL), sulcular bleeding index (SBI) and plaque index (PLI) at baseline examination. Sub gingival biofilm samples were collected from the deepest four sites before periodontal treatment in order to identify and quantify the level of periodontopathogens by checkerboard DNA-DNA hybridization. No significant differences were found between groups in PD, CAL and PLI. Significant differences were observed in GBI between SLE-A (11,19% ± 14,62%) compared to controls (17,30% ± 14.88%), although with no differences when compared to SLE-I (11,34% ± 11,59%). There was a low prevalence of PD and attachment loss ≥ 4 mm at all groups. A low prevalence of Actinomyces was observed at all groups, with an increase in red complex species at LES-I (4,07 x 105; 95% CI: 0,16-0,79) compared to control (2,50 x 105; 95% CI: 1,23-3,77), although with significant differences (p< 0,05; Kruskal Wallis post hoc Dunn) only when compared to SLE-A (0,45 x 105; 95% CI: 0,16-0,79). These findings show no differences in the periodontal conditions of SLE compared to systemically healthy patients, except for a decrease in gingival bleeding index, especially at SLE-A. Reductions in microorganisms' count were observed at SLE-A, while an increase in bacterial count, especially at red and green complex, were observed at subgingival biofilm of SLE-I patients.(AU)

Recomendaciones sobre el manejo del compromiso renal del lupus eritematoso sistémico: Documento de Consenso de las Sociedades Chilenas de Nefrología y Reumatología / A consensus of the Chilean Nephrology and Rheumatology Societies on renal involvement in systemic lupus erythematosus

Renal involvement affects over one half of patients with Systemic Lupus Erythematosus increasing their mortality and morbidity, including chronic renal disease and the need of renal replacement therapies. Aiming to achieve a consensus in the most relevant topics on diagnosis, therapy and follow-up of patients with lupus renal disease, the Chilean Societies of Nephrology and Rheumatology constituted a workgroup that, based on a critical review of the available literature and their experience, raised and answered by consensus a set of relevant questions. This document includes aspects related to the clinical diagnosis, the importance of a suitable histological classification, therapeutic alternatives to induce and maintain disease remission, strategies for follow-up, additional therapies and ginecological-obstetric issues.

Complicaciones en pacientes con lupus eritematoso sistémico / Complications in patients with systemic lupus erythematosus

RESUMEN Introducción: el lupus eritematoso sistémico (LES) es una enfermedad autoinmune de origen desconocido, que cursa con episodios de brotes alternados con periodos de remisión. Durante los brotes pueden aparecer complicaciones que motivan la hospitalización. Objetivo: identificar complicaciones en pacientes con LES que ingresaron al Servicio de Clínica Médica del Hospital Central "Dr. Emilio Cubas" del Instituto de Previsión Social en el año 2013. Metodología: estudio observacional, descriptivo, retrospectivo, de corte transversal. Se excluyeron a pacientes no conocidos portadores de LES y que debutaron con complicaciones, así como también aquellos con LES que ingresaron al Servicio de Clínica Médica provenientes de la Unidad de Terapia Intensiva (UTI). Resultados: 40 pacientes con LES fueron internados en el periodo estudiado. Se observó predominio del sexo femenino (92,5%), con un promedio de edad de 38,8 años. Los síntomas de ingreso más importantes en orden de frecuencia fueron: sintomatología respiratoria (35,0%), neurológica (25,0%) y sensación febril (20,0%). Las comorbilidades presentes con mayor frecuencia fueron: hipertensión arterial (40,0%), otra enfermedad autoinmune (18,0%) y enfermedad renal crónica (15,0%). Las complicaciones más frecuentes fueron: infecciones (67,5%), renales (22,5%), hematológicas (20,0%) y neurológicas (20,0%). Con respecto a la evolución de los pacientes, el 87,5% fueron dados de alta, 7,5% fallecieron y 5,0% fueron trasladados a la UTI. Los 3 fallecimientos correspondieron a causas infecciosas (choque séptico). Conclusión: las complicaciones infecciosas constituyen motivo frecuente de ingreso en pacientes con LES, ya que debido a la enfermedad y al tratamiento inmunosupresor se encuentran más propensos a desarrollar este tipo de complicación.

ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown origin that causes outbreaks episodes alternating with periods of remission. During outbreaks complications may occur and cause admission hospitalization. Objective: To identify complications in SLE patients admitted to the Medical Clinic of the Central Hospital "Dr. Emilio Cubas" Institute of Social Welfare in 2013. Methodology: observational, descriptive, retrospective and cross-sectional. Were excluded patients not known with SLE and who developed complications, as well as those with SLE admitted to the Medical Clinic from the Intensive Care Unit (ICU). Results: 40 patients with SLE were admitted during the study period. predominance of females (92.5%) were observed, with an average age of 38.8 years. Symptoms more frecuent on admission were: respiratory symptoms (35.0%), neurological (25.0%) and feeling feverish (20.0%). The most common comorbidities were present: hypertension (40.0%), another autoimmune disease (18.0%) and chronic renal disease (15.0%). The most common complications were infection (67.5%), renal (22.5%), hematologic (20.0%) and neurological (20.0%). Regarding the evolution of patients, 87.5% were discharged, 7.5% died and 5.0% were transferred to the ICU. The 3 patients died from infectious causes (septic shock). Conclusion: Infectious complications are frequent cause of admission in patients with SLE, due to the disease itself and immunosuppressive therapy are wich more prone to develop this complication.

Complicaciones en pacientes con lupus eritematoso sistémico / Complications in patients with systemic lupus erythematosus

Introducción: el Lupus Eritematoso Sistémico (LES) es una enfermedad autoinmune de origen desconocido, que cursa con episodios de brotes alternados con periodos de remisión. Durante los brotes pueden aparecer complicaciones que motivan la hospitalización. Objetivo:identificar complicaciones en pacientes con LES que ingresaron al Servicio de Clínica Médica del Hospital Central "Dr. Emilio Cubas" del Instituto de Previsión Social en el año 2013. Metodología: estudio observacional descriptivo, retrospectivo, de corte transversal. Se excluyeron a pacientes no conocidos portadores de LES y que debutaron con complicaciones, así como también aquellos con LES que ingresaron al Servicio de Clínica Médica provenientes de la Unidad de Terapia Intensiva (UTI). Resultados: 40 pacientes afectados fueron internados en el periodo estudiado, se observó predominio del sexo femenino (92,5%), con un promedio de edad de 38,8 años. Los síntomas de ingreso más importantes en orden de frecuencia fueron: sintomatología respiratoria (35,0%), neurológica (25,0%) y sensación febril (20,0%). Las comorbilidades presentes con mayor frecuencia fueron: hipertensión arterial (40,0%), otra enfermedad autoinmune (18,0%) y enfermedad renal crónica (15,0%). Las complicaciones más frecuentes fueron: infecciosas (67,5%), renales (22,5%), hematológicas (20,0%) y neurológicas (20,0%). Con respecto a la evolución de los pacientes, el 87,5% fueron dados de alta, 7,5% fallecieron y 5,0% fueron trasladados a la UTI. Los 3 fallecimientos correspondieron a causas infecciosas (shock séptico).Conclusión: las complicaciones infecciosas constituyen motivo frecuente de ingreso en pacientes con LES, ya que debido a la enfermedad y al tratamiento inmunosupresor se encuentran más propensos a desarrollar este tipo de complicación.

Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease of unknown origin that causes outbreaks episodes alternating with periods of remission. During outbreaks motivating hospitalization complications can occur. Objective: To identify complications in SLE patients admitted to the Internal Medicine Service of Central Hospital "Dr. Emilio Cubas" Institute for Social Welfare in 2013. Methods: Descriptive observational, retrospective, cross-sectional research. Patients not known carriers of SLE and who developed complications at the onset of the disease were excluded, as well as those with SLE who were admitted to the Internal Medicine Service from the Intensive Care Unit (ICU). Results: 40 patients affected were hospitalized during the study period, predominantly female (92.5%), with an average age of 38.8 years. Main symptoms in order of frequency were: respiratory (35.0%), neurological (25.0%) and febrile sensation (20.0%). The most common comorbidities present were hypertension (40.0%), another autoimmune disease (18.0%) and chronic kidney disease (15.0%). The most common complications were infection (67.5%), renal (22.5%), hematologic (20.0%) and neurological (20.0%). About the evolution of patients, 87.5% were discharged, 7.5% died and 5.0% were transferred to the ICU. The 3 deaths corresponded to infectious causes (septic shock). Conclusion: Infectious complications are a frequent reason for hospitalization in patients with SLE, because of the disease and the immunosuppressive therapy they are more likely to develop this complication.

Lúpus eritematoso sistêmico e terapia de reposição hormonal: atualização / Lupus erythematosus systemic and hormonal replacement therapy: update

O Lúpus Eritematoso Sistêmico (LES) é uma doença inflamatória crônica, de maior prevalência no sexo feminino, multissistêmica e de natureza autoimune, caracterizada pela presença de diversos autoanticorpos que evolui com manifestações clínicas polimórficas, com períodos de exacerbações e remissões Este artigo consistiu de revisão da literatura realizada pela consulta de seis bases de dados em busca de artigos nacionais e internacionais que relataram o evento tromboembólico e cardíaco nas pacientes lúpicas usuárias de terapia hormonal (TH), buscou relatos sobre o efeito da TH na prevenção da osteoporose nestas pacientes, e saber se o estrogênio pode ser fator etiológico no aparecimento da doença ou causando exacerbação de mulheres lúpicas expostas à TH. Como resultado, conclui-se que terapia hormonal pode ser usada na ausência de atividade da doença e sem agudizações por alguns anos, em mulheres com anticorpo anticardiolipina e/ou anticoagulante lúpico negativo e com doses terapêuticas de glicorticóide baixa e que se deve preferir baixa dose de estrogênio transdérmico combinado com progesterona natural, micronizada ou derivados pregnanos, por apresentar menor potencial trombogênico. E, naquelas pacientes com atividade da doença em controle e ainda com sintomas vasomotores, um agente não-estrogênico (antidepressivo ou progesterona) deve ser a primeira linha de tratamento.(AU)

Systemic Lupus Erythematosus (SLE) is a chronic multisystemic inflammatory disease, more prevalent in women. It is considered an autoimmune disease, characterized by the presence of several autoantibodies. Its clinical presentation is polymorphic, characterized by exacerbation and remissions periods. It was performed an extensive literature research in six databases searching for articles related to the thromboembolic and cardiac events in female SLE patients exposed to replacement hormone therapy (HT). Furthermore, it was searched articles on the effects of HT in the prevention of osteoporosis, as well as if estrogen therapy could be an etiologic factor in the emergence or exacerbation of LES in female patients. HT can be safely used in the absence of disease activity, in patients without exacerbation for some years, in women with no evidence of anticardiolipin or lupus anticoagulant antibodies, and using low corticoid doses. When the use of HT is necessary it is preferable to use low dose of transdermical estrogen combined with natural progesterone, micronised or pregnane derivatives that have less thrombogenic potential. In addition, in patients with LES activity controlled that maintain hot flashes a non-estrogenic therapy (antidepressant or progesterone) should be the first treatment line.(AU)

Pericarditis constrictiva como presentación grave e infrecuente de lupus eritematoso sistémico: reporte de un caso y revisión de la literatura / Constrictive pericarditis as a serious and rare presentation of systemic lupus erythematosus: Report of one case

Constrictive Pericarditis (CP) is an unusual disease. Its most common causes are idiopathic or secondary to cardiac surgery. Less frequently it is caused by connective tissue diseases. We report a 30 years old woman hospitalized due to progressive dyspnea, chest pain and signs of right sided heart failure. During her stay, a Systemic Lupus Erythematosus (SLE) was diagnosed. The echocardiogram suggested a CP and the diagnosis was confirmed by cardiac catheterization. Pericardiectomy was successfully performed. The biopsy confirmed a nonspecific chronic pericarditis, with extensive fibrosis and absence of caseating granulomas. The patient had a satisfactory recovery.