RESUMO Há escassez de estudos na literatura sobre as comorbidades na policondrite recidivante (PR). Além disso, exceto por alguns estudos epidemiológicos sobre a PR, não existem trabalhos que analisem especificamente a distribuição da PR de acordo com o gênero. Portanto, os objetivos do presente estudo foram: (a) analisar a prevalência de doenças cardiovasculares e seus fatores de risco em uma série de pacientes com PR; (B) determinar a influência do gênero na PR. Fez-se um estudo transversal unicêntrico que avaliou 30 casos de PR entre 1990 e 2016. Para comparar as comorbidades, foram recrutados 60 indivíduos saudáveis pareados por idade, gênero, etnia e índice de massa corporal. A idade média dos pacientes com PR foi de 49,0 ± 12,4 anos. A duração média da doença foi de 6,0 anos e 70% eram mulheres. Foi observada uma maior frequência de hipertensão arterial (53,3% vs. 23,3%, p = 0,008) e diabetes mellitus (16,7% vs. 3,3%; p = 0,039) no grupo PR em comparação com o grupo controle. Em uma análise adicional, os pacientes foram comparados de acordo com a distribuição de gênero (nove homens versus 21 mulheres). As características clínicas iniciais da doença foram comparáveis em ambos os sexos. No entanto, durante o período de seguimento, os pacientes do sexo masculino tiveram maior prevalência de perda auditiva, envolvimento vestibular e eventos de uveíte e também receberam mais tratamento com ciclofosfamida (p < 0,05). Houve uma alta prevalência de hipertensão arterial e diabetes mellitus e os pacientes do sexo masculino apresentaram pior prognóstico do que as pacientes do sexo feminino no seguimento.

ABSTRACT The comorbidities in relapsing polychondritis have been scarcely described in the literature. Moreover, apart from a few relapsing polychondritis epidemiological studies, no studies specifically addressing relapsing polychondritis distribution according to gender are available. Therefore, the objectives of the present study were: (a) to analyze the prevalence of cardiovascular diseases and its risk factors in a series of patients with relapsing polychondritis; (b) to determine the influence of gender on relapsing polychondritis. A cross-sectional tertiary single center study evaluating 30 relapsing polychondritis cases from 1990 to 2016 was carried out. To compare comorbidities, 60 healthy individuals matched for age-, gender-, ethnicity- and body mass index were recruited. The mean age of relapsing polychondritis patients was 49.0 ± 12.4 years, the median disease duration 6.0 years, and 70% were women. A higher frequency of arterial hypertension (53.3% vs. 23.3%; p = 0.008) and diabetes mellitus (16.7% vs. 3.3%; p = 0.039) was found in the relapsing polychondritis group, compared to the control group. As an additional analysis, patients were compared according to gender distribution (9 men vs. 21 women). The clinical disease onset features were comparable in both genders. However, over the follow-up period, male patients had a greater prevalence of hearing loss, vestibular disorder and uveitis events, and also received more cyclophosphamide therapy (p < 0.05). There was a high prevalence of arterial hypertension and diabetes mellitus, and the male patients seemed to have worse prognosis than the female patients in the follow up.

OBJECTIVE: To analyze the prevalence of myositis-specific and myositis-associated autoantibodies and their clinical correlations in a large series of patients with dermatomyositis/polymyositis. METHOD: This cross-sectional study enrolled 127 dermatomyositis cases and 95 polymyositis cases. The disease-related autoantibody profiles were determined using a commercially available blood testing kit. RESULTS: The prevalence of myositis-specific autoantibodies in all 222 patients was 34.4%, whereas myositis-associated autoantibodies were found in 41.4% of the patients. The most frequently found autoantibody was anti-Ro-52 (36.9%), followed by anti-Jo-1 (18.9%), anti-Mi-2 (8.1%), anti-Ku (4.1%), anti-SRP (3.2%), anti-PL-7 (3.2%), anti-PL-12 (2.7%), anti-PM/Scl75 (2.7%), and anti-PM/Scl100 (2.7%). The distributions of these autoantibodies were comparable between polymyositis and dermatomyositis, except for a higher prevalence of anti-Jo-1 in polymyositis. Anti-Mi-2 was more prevalent in dermatomyositis. Notably, in the multivariate analysis, anti-Mi-2 and anti-Ro-52 were associated with photosensitivity and pulmonary disorders, respectively, in dermatomyositis. Anti-Jo-1 was significantly correlated with pulmonary disorders in polymyositis. Moreover, anti-Ro-52 was associated with anti-Jo-1 in both diseases. No significant correlation was observed between the remaining autoantibodies and the clinical and/or laboratory findings. CONCLUSIONS: Our data are consistent with those from other published studies involving other populations, although certain findings warrant consideration. Anti-Ro-52 and anti-Jo-1 were strongly associated with one another. Anti-Ro-52 was correlated with pulmonary disorders in dermatomyositis, whereas anti-Jo-1 was correlated with pulmonary alterations in polymyositis.

OBJECTIVES: Herpes zoster has been widely described in the context of different systemic autoimmune diseases but not dermatomyositis/polymyositis. Therefore, we analyzed the prevalence, risk factors and herpes zoster outcomes in this population. METHOD: A retrospective cohort study of herpes zoster infections in dermatomyositis/polymyositis patients was performed. The patients were followed at a tertiary center from 1991 to 2012. For the control group, each patient with herpes zoster was paired with two patients without herpes zoster. Patients were matched by gender and the type of myositis, age at myositis onset and disease duration. RESULTS: Of 230 patients, 24 (10.4%) had a histories of herpes zoster (19 with dermatomyositis and five with polymyositis, two-thirds female). The mean age of the patients with herpes zoster was 44.6±16.8 years. No difference between the groups was found regarding cumulative clinical manifestations. Disease activity, autoantibody, muscle and leukogram parameters were also comparable between the groups. No differences in immunosuppressive (alone or in association with other immunosuppressive therapies) or glucocorticoid (current use, medium dose and cumulative dose in the last two months) therapies were found between patients with and without herpes zoster. However, a higher proportion of patients in the herpes zoster group received chloroquine diphosphate compared to the control group. All of the patients received acyclovir; 58.3% of patients had postherpetic neuralgia and no cases of recurrence were reported. Furthermore, individuals who were taking high prednisone doses at the time of the herpes zoster diagnosis had reduced levels of postherpetic neuralgia. CONCLUSIONS: These data suggest that chloroquine diphosphate could predispose patients with dermatomyositis/polymyositis to developing herpes zoster, particularly women and dermatomyositis patients.

OBJETIVOS: Relatamos os resultados de um estudo de coorte retrospectivo envolvendo 139 pacientes com dermatomiosite, conduzido de 1991 a 2011. MÉTODOS: Todos os pacientes preenchiam pelo menos quatro dos cinco critérios de Bohan and Peter (1975). RESULTADOS: A média de idade dos pacientes no início da doença foi de 41,7 ± 14,1 anos, e a duração da doença foi de 7,2 ± 5,2 anos. A amostragem constitui-se de 90,2% de indivíduos brancos, 79,9% do gênero feminino. Sintomas constitucionais foram detectados em menos da metade dos casos. Envolvimentos cutâneo e articular ocorreram em 95,7% e 41,7% dos pacientes, respectivamente. Em 48,2% dos pacientes foram apresentadas pneumopatia incipiente, opacidades em "vidro fosco" e/ou fibrose pulmonar. Todos os pacientes receberam prednisona (1 mg/kg/dia) e 51,1% receberam também metilprednisolona intravenosa (1 g/dia por três dias). Vários imunossupressores foram usados como poupadores de corticosteroide de acordo com tolerância, efeitos colaterais e/ou refratariedade. Houve recidiva de doença (clínica e/ou laboratorial) em 53,2% dos casos; 76,3% permaneceram em remissão no momento do estudo. A taxa de infecção grave foi de 35,3%, com o predomínio de herpes zoster. Houve 15 (10,8%) casos de câncer, dos quais 12 foram confirmados em um período de um ano após o diagnóstico da doença. Houve ainda 16 óbitos (11,5%) cujas causas principais foram sepse/choque séptico (27,5%), pneumopatia atribuída à doença (31,3%), neoplasias (31,3%) e eventos cardiovasculares (12,5%). CONCLUSÕES: No presente trabalho, os dados clínico-laboratoriais foram semelhantes aos de outros grupos populacionais descritos na literatura, com diferenças mínimas quanto à frequência e às características das manifestações extramusculares.

OBJECTIVE: To report the results of a retrospective cohort involving 139 patients with dermatomyositis, conducted from 1991 to 2011. METHODS: All patients met at least four of the five Bohan and Peter criteria (1975). RESULTS: The patients' mean age at disease onset was 41.7 ± 14.1 years, and mean disease duration was 7.2 ± 5.2 years. The sample comprised 90.2% white patients and 79.9% female patients. Constitutional symptoms occurred in less than half of the patients. Cutaneous and joint involvements occurred in 95.7% and 41.7% of the patients, respectively. Incipient pneumopathy, ground glass opacities and/or pulmonary fibrosis were present in 48.2% of the patients. All patients received prednisone (1 mg/kg/day) and 51.1% also received intravenous methylprednisolone (1 g/day for three days). Several immunosuppressants were used as corticosteroid sparing agents according to tolerance, side effects and/or refractoriness. Although disease relapse (clinical and/or laboratory) occurred in 53.2% of the patients, 76.3% were in disease remission at the end of the study. The rate of severe infection was 35.3%, and herpes zoster predominated. There were 15 (10.8%) cases of cancer, 12 within one year after the diagnosis. There were 16 deaths (11.5%), and their major causes were sepsis/septic shock (27.5%), pneumopathy attributed to the disease (31.3%), neoplasms (31.3%), and cardiovascular events (12.5%). CONCLUSIONS: In this study, the clinical and laboratory data were similar to those of other population groups described in the literature, with minimal differences regarding the frequency and characteristics of the extramuscular manifestations.

A associação entre a síndrome antifosfolípide e as miopatias inflamatórias idiopáticas tem sido raramente descrita na literatura. No presente trabalho relatamos dois pacientes com síndrome antifosfolípide diagnosticados com dermatomiosite ou polimiosite. Realizamos também uma revisão da literatura acerca dessa sobreposição de duas entidades autoimunes sistêmicas.

The association between antiphospholipid syndrome and idiopathic inflammatory myopathies has been rarely reported in the literature. In this paper we report two patients with antiphospholipid syndrome diagnosed with concomitant dermatomyositis or polymyositis. We also reviewed the literature on this overlapping of two systemic autoimmune entities.

OBJETIVO: Analisar a distribuição e a influência do gênero na polimiosite (PM), quanto às manifestações clínico-laboratoriais, evolução e comorbidades. MÉTODOS: Estudo de coorte retrospectivo, unicêntrico, em que foram avaliados 75 pacientes consecutivos com PM (Bohan e Peter, 1975) entre 1990 e 2010. Os exames complementares referem-se ao início do diagnóstico da PM. RESULTADOS: Este estudo avaliou 52 mulheres e 23 homens (razão 2,3:1), a maioria de cor branca (84,0%), com média de idade de 42,7 ± 13,7 anos (16 a 67 anos), e duração média de doença de 6,9 ± 5,5 anos (0 a 20 anos). Aproximadamente 50% apresentaram recidiva da doença durante o acompanhamento, com 4,0% de óbitos. Apesar disso, dois terços encontravam-se em remissão no desfecho do estudo. Não houve diferença entre os gêneros quanto à distribuição das características demográficas, clínico-laboratoriais, evolução clínica e terapia medicamentosa instituída. Com relação às comorbidades, houve alta prevalência de hipertensão arterial sistêmica (38,7%) e diabetes mellitus (17,3%), igualmente distribuídas entre os gêneros. Verificou-se alta prevalência de depressão e fibromialgia, porém apenas no gênero feminino. CONCLUSÕES: A prevalência de PM entre mulheres foi maior (razão 2,3:1). A prevalência de comorbidades foi alta na casuística estudada, cabendo-nos priorizar seus controles e, assim, oferecer melhor qualidade de vida aos pacientes.

OBJECTIVE: To assess gender distribution in polymyositis (PM) and its influence on disease, regarding clinical and laboratory manifestations, outcome and comorbidities. METHODS: Retrospective single-center cohort study assessing 75 consecutive patients with PM (Bohan and Peter, 1975) from 1990 to 2010. Complementary tests were related to early diagnosis of PM. RESULTS: The study assessed 52 women and 23 men (ratio 2.3:1), most of whom white (84.0%), with a mean age of 42.7 ± 13.7 years (16 to 67 years), and mean disease duration of 6.9 ± 5.5 years (0 to 20 years). Approximately 50% experienced disease relapse during follow-up. Nevertheless, two thirds were in remission at the end of this study, with 4.0% of deaths. There was no difference between genders regarding demographic, clinical and laboratory characteristics, clinical outcome and the drug therapy instituted. Regarding comorbidities, there was a high prevalence of hypertension (38.7%) and diabetes mellitus (17.3%), equally distributed between genders. There was also a high prevalence of depression and fibromyalgia, which were only observed among females. CONCLUSIONS: The prevalence of PM was higher among women than among men (2.3:1). Because the prevalence of comorbidities was high in the case series studied, it is worth emphasizing the need for their control to provide better quality of life for patients with PM.

A linfadenopatia no lúpus eritematoso sistêmico (LES) é um achado benigno encontrado comumente em jovens, com atividade cutânea e sintomas constitucionais, apresentando boa resposta à corticoterapia. O achado mais frequente à biópsia é a hiperplasia folicular reacional. Relatamos o caso de um paciente que, desde os 13 anos de idade, apresentava surtos recorrentes de linfadenopatia, acompanhados de hepatoesplenomegalia, febre e emagrecimento. Na evolução, apareceram artrite, hipertensão arterial, proteinúria, miocardiopatia e neuropatia periférica. Foi amplamente investigado sem esclarecimento diagnóstico e submetido a tratamento empírico de tuberculose. Somente após cinco anos de evolução firmou-se o diagnóstico de LES e recebeu tratamento específico. O diagnóstico precoce nestes casos é difícil, pois a investigação laboratorial pode ainda não demonstrar presença de autoanticorpos ou hipocomplementemia.

Lymphadenopathy is a benign finding in systemic lupus erythematosus (SLE), commonly seen in young patients with cutaneous involvement and constitutional symptoms, with good response to corticosteroids. Reactive follicular hyperplasia is the most frequent finding in biopsies. We report the case of a patient with recurrent episodes of lymphadenopathy associated with hepatosplenomegaly, fever, and weight loss since the age of 13 years. The patient also developed arthritis, hypertension, proteinuria, cardiomyopathy, and peripheral neuropathy. His condition was investigated extensively without diagnostic clarification; he was treated, empirically, for tuberculosis. The patient received a diagnosis of SLE only five years after the original presentation and received the specific treatment. Early diagnosis in those cases is difficult because laboratorial exams may not show the presence of auto-antibodies and low complement levels.

Descrevemos o caso de uma paciente com retocolite ulcerativa que apresentou gangrena cutânea secundária à trombose microvascular. Esta rara complicação da doença inflamatória intestinal é decorrente do estado de hipercoagulabilidade observado na sua fase de atividade inflamatória. Foi necessário estabelecer diagnóstico diferencial com poliarterite nodosa pelo acometimento abdominal e cutâneo. As alterações intestinais foram demonstradas pela colonoscopia com biópsia e as vasculares por ultrasonografia com Doppler de membros inferiores e biópsia da lesão cutânea. A paciente foi tratada com prednisona (1 mg/kg), sulfassalazina e anticoagulação plena e evoluiu com remissão da doença cutânea e intestinal.

We reported a patient with ulcerative colitis who developed microvascular thrombosis with secondary cutaneous gangrene. This is an uncommon complication of bowel inflammatory disease due to the hypercoagulability state that occurs in the active inflammatory phase. Differential diagnosis with polyarteritis nodosa had to be performed due to the presence of abdominal and cutaneous manifestations. The diagnosis was confirmed by colonoscopy and biopsies of various segments of the bowel, Doppler ultrasonography of the legs and biopsy of the cutaneous lesion. The patient was treated with prednisone (1 mg/kg), sulfasalazine and anticoagulation, with remission of cutaneous and bowel manifestations.

Os componentes de matriz extracelular têm sido amplamente pesquisados nos últimos anos quanto ao seu papel na patogênese de diversas doenças difusas do tecido conjuntivo e síndromes vasculíticas. No caso da arterite de Takayasu, muito pouco se sabe sobre o assunto e são propostas teorias relacionadas com a participação da imunidade celular ou humoral para explicar a causa desta doença. Neste sentido, avaliamos a imunidade humoral nesta patologia. OBJETIVO: Pesquisar anticorpos contra componentes da matriz extracelular, incluindo a identificação de anticorpos anticolágeno e antiaorta. MÉTODOS: Soros de 13 pacientes com arterite de Takayasu e de 8 pacientes normais foram utilizados para pesquisa de auto-anticorpos anticolágeno tipos I, III, IV e V e antiaorta pelo método ELISA. RESULTADOS: Soros dos pacientes com arterite de Takayasu revelaram-se negativos para colágenos dos tipos III e IV e apenas um paciente apresentou positividade para os tipos I e V, enquanto todos os soros de pacientes com arterite de Takayasu revelaram-se negativos para anticorpos anti-aorta. CONCLUSÕES: Nossos dados demonstraram que as freqüências de anticorpos anticolágenos dos tipos I, III, IV e V não estiveram significativamente aumentadas no soro de pacientes com arterite de Takayasu, assim como os anticorpos antiextrato de aorta, sugerindo que a etiopatogenia desta vasculite esteja possivelmente relacionada com distúrbio da imunidade celular.

The pathogenic role of the components of extracellular matrix as causative factor of diffuse connective diseases and vasculitis has been studied extensively. The pathogenesis of Takayasu´s arteritis is unknown, and many theories have been proposed to explain the etiology of this disease, involving disturbances of the humoral or cellular immune responses. OBJECTIVE: To research antibodies directed to components of the extracellular matrix and aorta extract. METHODS: Serum from thirteen patients with Takayasu´s arteritis and from eight normal patients was tested to show the presence of autoantibodies directed to anti types I, III, IV and V collagen and anti-aorta employing ELISA methodology. RESULTS: Serum from patients with Takayasu´s arteritis didn't show the presence of antibodies directed to types III and IV collagen and only one patient was positive for type I and V collagen. No serum was reactive with aorta extract in patients with Takayasu´s arteritis as well. CONCLUSIONS: Our results didn't show the presence of antibodies directed to types I, III, IV and V collagen, as well as against aorta extract, suggesting that cell immune mediated disturbance should be the mechanism involved in the etiopathogenesis of this form of vasculitis.

São registrados três casos de feo-hifornicose subcutânea em transplantados renais provocados pela Exophiala jeanselmei (Langeron) McGinnis et Padhye 1977, fungo demácio capaz, também, de produzir raramente eumicetoma de grãos pretos. Este fungo, segundo KWON-CHUNG & BENNETT, 1992(27) é antigenicamente muito heterogêneo, sendo identificados até o presente momento três sorotipos com subgrupos dentro de cada um deles. A feo-hifomicose subcutânea vem se tornando cada vez mais freqüente em transplantados renais, submetidos a terapêutica imunodepressora. Como a Exophiala jeanselmei já foi isolada do meio ambiente, torna-se dificil explicar a patogenia desses casos por um despertar ou reativação de processos quiescentes. Os Autores fizeram ampla revisão da literatura, registrando principalmente os casos de feo-hifomicose publicados no Brasil. Sugerem também, eventual ação fungistática da ciclosporina A sobre a Exophiala jeanselmei.

We report three cases of subcutaneous phaeohy-phomycosis due to Exophiala jeanselmei (Langeron) McGinnis et Padhye 1977, in kidney transplant patients. Exophiala jeanselmei is a dematious fungus having also ability to rarely procedure eumycetoma (black grains). According to KWON-CHUNG & BENNETT (1992)27 such fungus is antigenically very heterogeneous, since so far three serotypes have been identified; each serotype including subgroups. Subcutaneous phaeohyphomycosis is becoming more and more frequent in kidney transplant patients submitted to an immunosupressive treatment. As Exophiala jeanselmei has already been isolated from the environment it becomes difficult to explain the pathogenicity of these cases by a reactivation of quiescent processes. The authors suggest an occasional fungistatic action of cyclosporine A upon Exophiala jeanselmei. A review of the literature was carried out with the report mainly of cases published in Brazil.