Resumo As doenças reumatológicas, que incluem lúpus eritematoso sistêmico, artrite reumatoide, doença de Behçet, esclerodermia e espondilite anquilosante, são caracterizadas por envolvimento do tecido conjuntivo, com múltiplas manifestações. Nessas doenças, o envolvimento do sistema nervoso central ou periférico pode ser primário, apresentando-se como uma das principais características clínicas, ou secundárias, como efeito das drogas usadas para seu controle. O diagnóstico, especialmente nas fases iniciais, depende do conhecimento de grande variedade de achados em métodos de imagem, permitindo um tratamento eficaz, causando menores deficiências. Este ensaio, com casos de um arquivo didático de dois hospitais terciários, engloba pacientes com diagnóstico de doenças reumatológicas e ilustra os achados neurorradiológicos de ressonância magnética e tomografia computadorizada, a fim de enfatizar a importância desses métodos para o diagnóstico adequado.

Abstract The rheumatic diseases, which include systemic lupus erythematosus, rheumatoid arthritis, Behçet's disease, scleroderma, and ankylosing spondylitis, are characterized by involvement of connective tissue, with multiple manifestations. In those diseases, there can be involvement of the peripheral or central nervous system, and that involvement can be primary, presenting as a major feature of the clinical presentation, or secondary, as an effect of the drugs used in order to control a given disease or its complications. Knowledge of the wide variety of imaging findings is crucial to the diagnosis of a rheumatic disease, especially in the early stages, enabling effective treatment and minimizing disability. This pictorial essay, presenting cases from the records of two tertiary teaching hospitals, encompasses cases of patients diagnosed with rheumatic disease and illustrates the neuroradiological findings on magnetic resonance imaging and computed tomography, in order to emphasize the importance of these methods for properly diagnosing rheumatic diseases.

OBJECTIVES: Rheumatoid arthritis is a polygenically controlled systemic autoimmune disease. Rheumatoid vasculitis is an important extra-articular phenotype of rheumatoid arthritis that can result in deep cutaneous ulcers. The objective of this study was to establish a correlation between the frequency of major histocompatibility complex class I/II alleles and killer immunoglobulin-like receptor genotypes in patients with cutaneous rheumatoid vasculitis. METHODS: Using the Scott & Bacon 1984 criteria to diagnose rheumatoid vasculitis and after excluding any other causes such as diabetes, atherosclerosis, adverse drug reactions, infection, and smoking, patients who met the criteria were selected. All of the selected rheumatoid vasculitis patients presented deep cutaneous ulcers. Identification of the major histocompatibility complex class I/II and killer immunoglobulin-like receptor genotypes was performed by polymerase chain reaction assays of samples collected from the 23 rheumatoid vasculitis patients as well as from 80 controls (40 non-rheumatoid vasculitis RA control patients and 40 healthy volunteers). RESULTS: An association between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and cutaneous lesions in rheumatoid vasculitis patients and a correlation between the inhibitor KIR2DL3 and the HLA-C*0802 ligand in rheumatoid vasculitis patients were found. CONCLUSION: An association was found between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and the development of cutaneous lesions in rheumatoid vasculitis patients. Additionally, the HLA-C*0802 ligand protects these individuals from developing cutaneous lesions.

Introdução: As manifestações otorrinolaringológicas de doenças reumáticas representam um grande desafio não só ao médico generalista, mas também ao otorrinolaringologista e ao reumatologista. Frequentemente representam manifestações iniciais de uma desordem autoimune que exige um tratamento imunossupressor imediato e agressivo. Sintomas auditivos, nasais, laríngeos e oculares podem ser a primeira manifestação de doenças reumáticas, e sua correta avaliação auxilia o médico a identificar sinais de atividade da doença. O objetivo deste trabalho foi identificar as manifestações otorrinolaringológicas em pacientes com doenças reumáticas em um hospital de alta complexidade, no que se refere a facilitar diagnóstico e tratamento precoces. Métodos: Foram realizadas avaliações clínicas e otorrinolaringológicas completas em pacientes selecionados no ambulatório de reumatologia, no segundo semestre do ano de 2010, de forma padronizada e com utilização de um formulário de preenchimento normatizado. Resultados: No grupo estudado, pacientes com LES apresentaram predominantemente manifestações laríngeas, enquanto pacientes com síndrome de Sjögren apresentaram predomínio das manifestações otológicas (100% dos casos). As alterações de exames audiométricos são encontradas em 53% dos casos portadores de GW, 80% de PR, 33% de LES e 50% de SCS. Quanto às alterações nasais, estas foram encontradas de forma prevalente em todas as patologias, principalmente a síndrome de Churg-Strauss. Discussão e conclusão: Este estudo demonstrou que a maioria dos pacientes em seguimento em nosso serviço apresenta os sinais e sintomas otorrinolaringológicos comumente relacionados em trabalhos prévios sobre doenças reumáticas, porém novos estudos com um número maior de pacientes devem ser feitos para comprovar tais relações.

Introduction: Otorhinolaryngological manifestations of rheumatologic diseases represent a great challenge not only to the generalistphysician but also to the ENT doctor andrheumatologist. They often represent early manifestations of an autoimmune disorder which requires prompt and aggressive immunosuppressive treatment. Auditory, nasal, laryngeal and eye symptoms can be the first manifestation of rheumatic diseases and their proper assessment helps the doctor to identify signs of disease activity. The objective of this study is to identify the ENT manifestations in patients with rheumatic diseases in a high complexity hospital, regarding facilitating an early diagnosis and treatment. Methods: We performed clinical and complete otorhinolaryngological evaluations in patients selected from the outpatient rheumatology in a standardized manner by the use of a standardized form filling during the secondhalf of 2010. Results: In the study group, systemic lupus erythematosus (SLE) patients had predominantly laryngeal manifestations, while patients with Sjögren's syndrome showed a higher prevalence of otologic manifestations. Changes in audiometric tests were found in 53% of Wegener's granulomatosis (WG) patients, 80% of relapsing polychondritis (RP), 33% of systemic lupus erythematosus (SLE) and 50% of Churg-Strauss syndrome (SCS). Regarding nasal alterations, these were found so prevalent in all conditions, especially Churg-Strauss syndrome. Discussion and conclusion: This study demonstrated that most patients treated in our hospital has the ENT signs and symptoms commonly associated in previous studies on rheumatic diseases, but further studies with a larger number of patients must be made to establish such relations.

O objetivo deste trabalho é avaliar as características demográficas, clínicas, laboratoriais e histopatológica de pacientes com diagnóstico de síndrome de Churg Strauss (SCS) acompanhados no ambulatório de vasculites do Hospital das Clínicas da Universidade Estadual de Campinas (HC/Unicamp). Foi realizado estudo retrospectivo dos prontuários dos pacientes com vasculite primária, classificados como SCS pelos critérios do American College of Rheumathology (ACR). Foram observados cinco pacientes com diagnóstico de SCS (M/F:3/2, caucasóides). A média de idade do início da doença foi 46 anos (40-55). A média de tempo de seguimento foi de 2,37 anos (0,25-6). Sintomas sistêmicos, asma, neuropatia periférica e lesão cutânea foram observados em todos os pacientes. Envolvimento cardiovascular, renal e de trato gastrintestinal estiveram presentes cada qual em um paciente. Todos os pacientes apresentaram eosinofilia (>10%),e o p-ANCA foi positivo nos três casos investigados. A radiografia de tórax revelou infiltrado pulmonar intersticial em dois casos; em um, associado à derrame pleural. Na histopatologia, evidências de eosinófilos extravascular foram encontradas nas biópsias cutâneas de 3/4 pacientes. Todos os pacientes foram tratados com prednisona. Em 3, houve a necessidade de associar-se imunossupressor. Nenhum paciente evoluiu a óbito. A SCS é uma patologia rara, com acometimento sistêmico, sendo a asma uma das principais e mais precoce manifestação. Todos os pacientes apresentaram sintomas sistêmicos, acometimento de pele e sistema nervoso periférico. Envolvimento renal, cardíaco e gastrointestinal foi observado, cada qual em um único paciente, diferindo de outras séries nas quais esses acometimentos são mais freqüentes. Embora seja uma patologia grave e de prognóstico reservado, observamos evolução favorável com tratamento.

The aim of this report is to verify the demographic, clinical, laboratorial and histopathological findings of patients with CSS followed in a University Clinical Hospital. We reviewed the medical records of all patients with the diagnosis of CSS. Only patients who fulfilled ACR criteria were included. Five Caucasian patients were found, with a sex ratio M/F: 3/2. The mean age at disease onset was 46 years old (40 to 55) and the mean time of follow-up was 2.37 years (0.25 to 6). General symptoms, asthma, peripheral neuropathy and skin lesions were the most common clinical findings in all patients. Cardiovascular, gastrointestinal and renal involvement was found in one patient each. All patients presented hypereosinophilia (> 10%) and p-ANCA was present in 3 patients. The chest x-ray showed pulmonary parenchymal infiltrate in 2 cases and in 1 of them an unilateral pleural effusion. Skin biopsies showed extravascular eosinophylia in 3 out of 4. All patients were treated with prednisone and in 3 it was added immunosuppressive drug. No patient died during the follow-up. CSS is a rare disease with a multivisceral involvement and asthma is the main and the earliest symptom. General symptoms and involvement of skin and peripheral nerves were found in all patients. Renal, cardiovascular and gastrointestinal involvement was rare, differently from the literature series where these findings are more frequent. In spite of the CSS being a serious and life-threatening disease, these patients presented adequate response to the treatment and a favorable outcome.