Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small. .

Comparative study of ophthalmological and serological manifestations and the therapeutic response of patients with isolated scleritis and scleritis associated with systemic diseases / Estudo comparativo entre as manifestações oftalmológicas, sorológicas e resposta terapêutica de pacientes com esclerite isolada e esclerite associada a doenças sistêmicas

INTRODUCTION: Scleritis is a rare, progressive and serious disease, the signs of which are inflammation and edema of episcleral and scleral tissues and is greatly associated with systemic rheumatoid diseases. PURPOSE: To perform a prospective and comparative study between ophthalmologic manifestations, serologic findings and therapeutic response of patients with isolated scleritis and scleritis associated with systemic rheumatoid disease. METHODS: Thirty-two outpatients with non-infectious scleritis were studied, from March 2006 to March 2008. The treatment was corticoid eye drops associated with anti-inflammatory agents, followed by systemic corticoids and immunosuppressive drugs if necessary, was considered successful after six months without scleritis recurrence. RESULTS: Fourteen of 32 patients had scleritis associated with systemic rheumatoid disease, of which nine had rheumatoid arthritis, two systemic lupus erythematosus, one Crohn's disease, one Behçet's disease and one gout. There were no difference in relation to involvement and ocular complications, there was predominance of nodular anterior scleritis and scleral thinning was the most frequent complication. The scleritis associated with systemic rheumatoid disease group had 64.3 percent of autoantibodies, versus 27.8 percent among those with isolated scleritis and this difference was statistically significant. In the isolated scleritis group 16.7 percent used anti-inflammatory, 33.3 percent corticosteroids, 27.8 percent corticosteroids with one immunosuppressive drug, 5.5 percent two immunosuppressive drugs, 16.7 percent corticosteroids with two immunosuppressive drugs and 33.3 percent pulse of immunosuppressive drugs, there was remission in 88.9 percent. In the scleritis associated with systemic rheumatoid disease group 7.1 percent used anti-inflammatory, 7.1 percent corticosteroids, 50 percent corticosteroids with one immunosuppressive drug, 7.1 percent two immunosuppressive drugs and 22.2 percent pulse of immunosuppressive drugs, 100 percent had treatment success. CONCLUSION: Prevalence of unilateral nodular scleritis was noted in both groups and higher rates of all the parameters tested were noted in the scleritis associated with systemic rheumatoid disease group. There were no differences between the groups with respect to the use of immunosuppressive drugs and therapeutic response, which was fully satisfactory in the scleritis associated with systemic rheumatoid disease group and satisfactory in the isolated scleritis group.

INTRODUÇÃO: Esclerite é uma doença grave, rara e progressiva, que envolve inflamação e edema dos tecidos episcleral superficial, profundo e escleral e está associada com doenças sistêmicas reumatológicas em muitos casos. OBJETIVOS: Realizar um estudo prospectivo comparativo entre as manifestações oftalmológicas, achados sorológicos e resposta terapêutica de pacientes com esclerite isolada e com esclerite associada a doenças sistêmicas reumatológicas. MÉTODOS: Trinta e dois pacientes com esclerite não infecciosa participaram do estudo, de março de 2006 a março de 2008. O tratamento realizado baseou-se no uso de colírios de corticoides associados aos anti-inflamatórios não-hormonais, seguidos de corticoides sistêmicos e imunossupressores, se necessário. O sucesso do tratamento foi considerado como seis meses sem crises de esclerite. RESULTADOS: Quatorze dos 32 pacientes apresentaram esclerite associada à doença sistêmica, dos quais nove com artrite reumatóide, dois com lúpus eritematoso sistêmico, um com doença de Crohn, um com doença de Behçet e um com gota. Não houve diferenças em relação ao envolvimento ocular e suas complicações, predominando a esclerite anterior nodular e o afinamento escleral, respectivamente. O grupo com esclerite associada a doenças sistêmicas apresentou 64,3 por cento de positividade de autoanticorpos contra 27,8 por cento no grupo com esclerite isolada, sendo tal diferença estatisticamente significante. No grupo com esclerite isolada, 16,7 por cento fez uso de apenas anti-inflamatórios, 33,3 por cento de corticoide sistêmico, 27,8 por cento de corticoide com um imunossupressor, 5,5 por cento dois imunossupressores, 16,7 por cento corticoide com dois imunossupressores e 33,3 por cento pulsoterapia com imunossupressor; sendo que houve sucesso do tratamento em 88,9 por cento. No grupo com esclerite associada à doença sistêmica, 7,1 por cento fez uso de anti-inflamatórios, 7,1 por cento corticoide sistêmico, 50 por cento corticoide com um imunossupressor, 7,1 por cento dois imunossupressores e 22,2 por cento pulsoterapia com imunossupressor; com 100 por cento de sucesso no tratamento nesse grupo. CONCLUSÃO: Em ambos os grupos houve predomínio da esclerite nodular unilateral e o grupo com esclerite associada a doença sistêmica apresentou taxas maiores de todos os autoanticorpos testados. Não houve diferença entre os grupos em relação ao uso de imunossupressores e à resposta terapêutica, a qual foi totalmente satisfatória no grupo com esclerite associada à doença sistêmica e satisfatória no grupo com esclerite isolada.

Curso de reumatologia: aula 7: manifestações neurológicas nas colagenoses / Course of rheumatology: class 7: neurologic manifestations in collagenosis

O propósito deste artigo é focar as principais manifestações neurológicas das doenças difusas do tecido conjuntivo, com ênfase nas suas manifestações clínicas. Os autores discutem as várias complicações do sistema nervoso central e periférico na doença muscular inflamatória (polimiosite e dermatomiosite), policondrite recidivante, esclerose sistêmica, artrite reumatoide, síndrome de Sjõgren, doença mista do tecido conjuntivo (doença de Sharp), lúpus eritematoso sistêmico e síndrome do anticorpo antifosfolipide.

The purpose of this article is to focus on the major nervous system manifestations in connective tissue diseases, with emphasis on their clinica findings. Authors discuss several complications in inflammatory muscle disease (polymyositis an dermatomyositis), relapsing polychondritis, systemic sclarosis, rheumatoid arthritis, Sjõgren syndorme, mixed connective tissue (Sharp disease), systemic lupus erythematosus and antiphospholipid syndrome.

Magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders

OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis.METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. Meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behçet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.

Diagnóstico da esclerodermia sistêmica baseado em alteraçöes orofaciais: revisäo da literatura / Diagnosis of Scleroderma based on orofacial alterations: review of the literature

A Esclerose Sistêmica Progressiva (Esclerodermia) é uma doença crônica caracterizada por esclerose difusa do tecido conjuntivo. Os tecidos cutâneos e viscerais podem estar envolvidos e há manifestaçöes orofaciais clínicas e radiográficas que os dentistas devem conhecer. Através do estudo radiológico dos dentes e maxilares, o dentista poderá contribuir para o diagnóstico da doença, encaminhando o paciente ao médico especialista e o conscientizando em relaçäo à higiene oral adequada

Colagenosis perforante reactiva y artritis reumatoidea juvenil / Reactive perforating collagenosis and juvenile rheumatoid arthritis

Se presenta la asociación en una paciente de 16 años, de colagenosis perforante reactiva y artritis reumatoide juvenil, de 5 y 3 años de evolución, respectivamente, sin relación en la actividad de cada una. Se analiza la vinculación de ambas entidades, llegándose a la conclusión de que probablemente se deba al azar, no encontrándose un factor etiopatogénico común ni antecedentes bibliográficos. Se incluye una revisión del cuadro clínico e histológico de las entidades perforantes y su diagnóstico diferencial