Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection

OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated in summer/autumn (n=27). The 25OHD levels were measured by radioimmunoassay. Disease activity was assessed by the Birmingham Vasculitis Activity Score Modified for Wegener's Granulomatosis (BVAS/WG) and antineutrophil cytoplasmic antibody (ANCA) positivity. Respiratory infection was defined according the Centers for Disease Control and Prevention criteria. RESULTS: 25OHD levels were lower among patients in winter/spring than in summer/autumn (32.31±13.10 vs. 38.98±10.97 ng/mL, p=0.04). Seven patients met the criteria for respiratory infection: 5 in winter/spring and 2 in summer/autumn. Patients with respiratory infection presented lower 25OHD levels than those without infection (25.15±11.70 vs. 36.73±12.08 ng/mL, p=0.02). A higher frequency of low vitamin D levels (25OHD<20 ng/mL) was observed in patients with respiratory infection (37.5% vs. 7.8, p=0.04). Serum 25OHD levels were comparable between patients with (BVAS/WG≥1 plus positive ANCA) and without disease activity (BVAS/WG=0 plus negative ANCA) (35.40±11.48 vs. 35.34±13.13 ng/mL, p=0.98). CONCLUSIONS: Lower 25OHD levels were associated with respiratory infection but not disease activity in granulomatosis with polyangiitis patients. Our data suggest that hypovitaminosis D could be an important risk factor for respiratory infection in granulomatosis with polyangiitis patients.

Relapsing polychondritis: prevalence of cardiovascular diseases and its risk factors, and general disease features according to gender / Policondrite recidivante: prevalência de doenças cardiovasculares e seus fatores de risco e características gerais da doença de acordo com o gênero

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.

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.

Doença sistêmica relacionada à IgG4 com linfopenia: relato de caso e breve revisão de literatura / IgG4-related systemic disease associated with lymphopenia: case report and brief literature review

A doença sistêmica relacionada à IgG4 é uma doença emergente, recentemente descrita, caracterizada clinicamente por aumento parcial ou total de um órgão, e, por isso, com amplo espectro de manifestações clínicas. Esta é uma doença sistêmica fibroinflamatória, patologicamente provocada pela infiltração de plasmoblastos IgG4 positivos que levam à inflamação eosinofílica do tecido e, consequentemente, fibrose estoriforme. Quando o diagnóstico é precoce, a melhora clínica e resposta sustentada com corticosteroides sistêmicos é impressionante. O diagnóstico é baseado em critérios patológicos, mas, recentemente, alguns trabalhos têm descrito que plasmoblastos no soro podem servir como um fator independente para auxiliar no diagnóstico da doença. Este artigo descreve uma apresentação atípica da doença relacionada à IgG4, em um paciente linfopênico com medição inconclusiva de plasmoblastos no soro.

IgG4-related systemic disease is a recently described, emerging condition, clinically characterized by partial or total enlargement of an organ, with a broad spectrum of clinical manifestations. It is a systemic fibroinflammatory condition caused by the infiltration of IgG4-positive plasmablasts that lead to eosinophilic inflammation of tissues and consequently storiform fibrosis. When diagnosis is early, clinical improvement and maintained response achieved with systemic corticosteroids is impressive. Diagnosis is based on pathological criteria, but recent papers have described that serum plasmablasts may serve as an independent factor to aid in diagnosis. This paper describes an atypical presentation of IgG4-related disease in a lymphopenic patient with inconclusive serum plasmablast measurement.

Vascular surgery: the main risk factor for mortality in 146 Takayasu arteritis patients.

Takayasu arteritis (TA) is an idiopathic chronic inflammatory disease that affects the aorta and its main branches. According to disease involvement, patients may require surgical treatment mainly due ischemic lesions in association with medical therapy. We evaluated the impact of vascular interventions in a cohort of TA patients. Medical records from 146 TA patients were reviewed. Clinical features, medical, and surgical treatment were revised and disease activity was determined by clinical, laboratorial, and imaging parameters. Clinical parameters associated with mortality alongside vascular procedures were evaluated and their impact on mortality in our cohort was estimated. Ninety-four vascular interventions were performed in 61 patients (41.8%). A third of them were of endovascular procedures. The overall mortality was 4.1%, all due to early postoperative complications, which resulted in a rate of surgery-related mortality of 9.8%. All deaths occurred in patients with active disease. Clinical parameters known to be associated with mortality (aneurysm, secondary hypertension, aortic insufficiency, and cerebrovascular accident) were not found related with death. Patients whose disease began before age 20 years had an OR 3.54 of undergoing a vascular surgical intervention. The observed impact of vascular procedures on mortality in patients with Takayasu arteritis, especially during disease activity, supports the notion that such interventions should be performed with caution and preferably during periods of remission.

Serum 25-hydroxyvitamin D levels in patients with Granulomatosis with Polyangiitis: association with respiratory infection.

OBJECTIVES: To determine the possible association of serum 25-hydroxyvitamin D (25OHD) levels with disease activity and respiratory infection in granulomatosis with polyangiitis patients during two different periods: winter/spring and summer/autumn. METHODS: Thirty-two granulomatosis with polyangiitis patients were evaluated in the winter/spring, and the same patients (except 5) were evaluated in summer/autumn (n=27). The 25OHD levels were measured by radioimmunoassay. Disease activity was assessed by the Birmingham Vasculitis Activity Score Modified for Wegener's Granulomatosis (BVAS/WG) and antineutrophil cytoplasmic antibody (ANCA) positivity. Respiratory infection was defined according the Centers for Disease Control and Prevention criteria. RESULTS: 25OHD levels were lower among patients in winter/spring than in summer/autumn (32.31±13.10 vs. 38.98±10.97 ng/mL, p=0.04). Seven patients met the criteria for respiratory infection: 5 in winter/spring and 2 in summer/autumn. Patients with respiratory infection presented lower 25OHD levels than those without infection (25.15±11.70 vs. 36.73±12.08 ng/mL, p=0.02). A higher frequency of low vitamin D levels (25OHD<20 ng/mL) was observed in patients with respiratory infection (37.5% vs. 7.8, p=0.04). Serum 25OHD levels were comparable between patients with (BVAS/WG≥1 plus positive ANCA) and without disease activity (BVAS/WG=0 plus negative ANCA) (35.40±11.48 vs. 35.34±13.13 ng/mL, p=0.98). CONCLUSIONS: Lower 25OHD levels were associated with respiratory infection but not disease activity in granulomatosis with polyangiitis patients. Our data suggest that hypovitaminosis D could be an important risk factor for respiratory infection in granulomatosis with polyangiitis patients.

Determinants of arterial stiffness in female patients with Takayasu arteritis.

OBJECTIVE: The assessment of pulse wave velocity (PWV) in Takayasu arteritis (TA) is complex because of many confounding factors. We evaluated PWV in female patients with TA and controls with comparable anthropometric and clinical variables and assessed a possible association of TA with disease variables. METHODS: We evaluated 27 patients with TA consecutively. Exclusion criteria were menopause, smoking, diabetes, renal insufficiency, poorly controlled hypertension, cardiac arrhythmias, obesity, inflammatory comorbidities, pregnancy, and surgical procedures involving the aorta. Disease activity was determined by clinical and laboratory variables. As healthy controls, 27 subjects with comparable age, blood pressure, height, and weight were selected. Carotid-femoral PWV measurements were obtained using the Complior system. RESULTS: The mean PWV in patients with TA was higher than in healthy controls (9.77 ± 3.49 vs 7.83 ± 1.06 m/s; p = 0.009). Despite our strict selection criteria, patients with TA had an average systolic blood pressure (SBP) 8 mmHg higher than controls (p = NS), and significantly higher pulse pressure values. The multivariate linear regression model shows that 93.8% of the PWV variability is explained by the variables age, mean BP, and the disease itself (adjusted R(2) = 0.938). Stepwise logistic analysis using the PWV cutoff value established by the receiver-operator characteristic curve (> 8.34 m/s) as dependent variable, and measures with significance in univariate analysis as independent variables revealed that TA (OR 4.69; 95% CI 1.31-16.72; p = 0.017) and mean BP (OR 1.06; 95% CI 1.00-1.12; p = 0.048) were independently associated with higher PWV. Further analysis of disease variables revealed that PWV values were not correlated with erythrocyte sedimentation rate, C-reactive protein, cumulative dose of glucocorticoid, or ejection fraction (p > 0.05). CONCLUSION: In our cohort of female patients with TA, the disease itself and mean BP were the strongest determinants associated with arterial stiffness.

Nefropatia por IgA e polimiosite: uma rara associação / IgA nephropathy and polymyositis: a rare association

A polimiosite é uma miopatia inflamatória idiopática sistêmica que, além da manifestação muscular, pode eventualmente cursar com acometimento respiratório, do trato gastrintestinal e, raramente, renal. Neste último caso, há descrição de apenas dois casos de nefropatia por IgA em pacientes com miopatia, ambos em dermatomiosite. Em contrapartida, relatamos pela primeira vez esta rara associação em polimiosite.

Polymyositis is a systemic and idiopathic inflammatory myopathy that, besides muscle manifestation, may occur with respiratory involvement, gastrointestinal tract and rarely renal involvement. In this latter, there are only two cases of IgA nephropathy, but both in dermatomyositis. On the other hand, we reported, for the first time, a case of IgA nephropathy in polymyositis.

[Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ]. / Síndrome antissintetase: anti-PL-7, anti-PL-12 e anti-EJ.

OBJECTIVES: Due to the scarcity of studies in the literature, we conducted an analysis of a series of patients with the anti-PL-7, PL-12 and EJ types of antisynthetase syndrome (ASS). METHODS: We conducted a retrospective cohort study of 20 patients with ASS (8 with anti-PL-7, 6 with PL-12, 6 with EJ) monitored in our department between 1982 and 2012. RESULTS: The mean patient age at disease onset was 38.5 ± 12.9 years, and the disease duration was 4.5 ± 6.4 years. Of all the patients, 70% were white and 85% were female. Constitutional symptoms occurred in 90% of cases. All patients presented objective muscle weakness in the limbs; in addition, 30% were bedridden and 65% demonstrated high dysphagia at diagnosis. Joint and pulmonary involvement and Raynaud's phenomenon occurred in 50%, 40% and 65% of cases, respectively, with more than half of the patients presenting incipient pneumopathy, ground-glass opacity and/or pulmonary fibrosis. There were no cases of neurological and/or cardiac involvement. All patients received prednisone or other immunosuppressants depending on tolerance, side effects and/or disease refractoriness. Importantly, patients with the anti-EJ type of ASS demonstrated higher rates of recurrence. Two patients died during follow-up, and 1 patient had breast cancer at the time of diagnosis. CONCLUSIONS: ASS (anti-PL-7, PL-12 and EJ) was found to predominantly affect white women. Although the autoantibodies described in the present study are more related to pulmonary than joint involvement, our patients showed a significant percentage of both types of involvement and a high percentage of myopathy. We also observed a low mortality rate.

[Inclusion body myositis: series of 30 cases from a Brazilian tertiary center]. / Miosite de corpos de inclusão: série de 30 casos de um centro terciário brasileiro.

OBJECTIVES: To describe a series of 30 consecutive patients with inclusion body myositis (IBM) from our tertiary center, from 1982 to 2012. MATERIALS AND METHODS: All patients fulfilled the criteria of Griggs et al. (1995) for IBM. RESULTS: The mean age of patients at disease onset was 60.8 � 11.9 years with disease duration of 8.0 � 5.2 years. Eighty % of patients were Caucasian, with similar distribution between genders. Weight loss in early disease was present in less than a quarter of cases. The main symptom was proximal weakness of the lower limbs followed by weakness of the upper (proximal and/or distal) limbs. One third of patients had dysphagia, whereas dysphonia was present in 16.7%, arthralgias in 6.7%, moderate dyspnea symptoms in 3.3% of cases. All patients received prednisone (1mg/kg/day). Several immunosuppressives were used as corticosteroid-sparing according to tolerance, side effects and/or refractoriness. Half of the patients still in follow-up remained stable according to clinical and laboratory data during the study. There were four cases of cancer, four cases associated with viral infections (HIV and hepatitis C virus) and three deaths (two because of sepsis secondary to community bronchopneumonia, and one because of congestive heart failure). CONCLUSIONS: This is the first Brazilian series of cases involving large sample of IBM. The profile of the patients analyzed in this study was comparable to those profiles described in literature, except that of IBM cases of our population are equally distributed in both genders and the interval between symptoms onset and diagnosis of the disease was relatively short. It is relevant to note the high frequency of neoplastic diseases and chronic viral infections in our population, reinforcing the need for specific epidemiological studies to verify these associations, once it is poorly described in the literature.

High prevalence of metabolic syndrome in Takayasu arteritis: increased cardiovascular risk and lower adiponectin serum levels.

OBJECTIVE: The prevalence of metabolic syndrome (MetS) tends to be high among rheumatic patients, and cardiovascular disease is the leading cause of death in these conditions. We aimed to determine the prevalence of MetS in patients with Takayasu arteritis (TA) and its association with risk factors and adipokine and cytokine levels. METHODS: A cross-sectional study was conducted in 45 consecutive women with TA and 47 healthy controls matched by age and body mass index. RESULTS: The prevalence of MetS (International Diabetes Federation/American Heart Association criteria) was higher in TA compared to controls (33.34 vs 8.51%, p = 0.003). Patients with TA had a higher frequency of hypertension (p < 0.001) and dyslipidemia (p = 0.001) and higher levels of insulin (p = 0.021), homeostasis model assessment index (p = 0.024), apolipoprotein E (p = 0.029), resistin (p = 0.018), and C-reactive protein (CRP, p < 0.001) compared to healthy subjects, with similar levels of adiponectin and plasminogen activator inhibitor-1 (PAI-1; p > 0.05). Further analysis of patients with TA with and without MetS revealed a higher frequency of overweight/obesity (66.66 vs 26.66%, p = 0.022), higher Framingham score ≥ 1 (p = 0.032), and lower adiponectin levels (20.37 ± 21.16 vs 38.64 ± 22.62 µg/ml, p = 0.022) in the patients with MetS. No differences were found regarding disease duration, activity, glucocorticoid use, resistin, and PAI-1 levels in the 2 groups of patients with TA (p > 0.05). Patients with and without MetS showed no differences in cytokine levels [interleukin 12 (IL-12, IL-1a, IL-6) and tumor necrosis factor-α]. IL-6 had a positive Pearson correlation with CRP only in TA patients with MetS (r = 0.57; p = 0.050). CONCLUSION: A high prevalence of MetS was observed in patients with TA and this comorbidity seems to identify a subgroup of overweight/obese patients with high cardiovascular risk without a significant association with disease status. Further longitudinal studies are necessary to observe the effects of controlling this modifiable risk factor in the quality of life and survival of patients with TA.

Síndrome antissintetase: anti-PL-7, anti-PL-12 e anti-EJ / Anti-synthetase syndrome: anti-PL-7, anti-PL-12 and anti-EJ

OBJETIVOS: Devido à escassez de trabalhos na literatura, realizamos análise de uma série de pacientes com síndrome antissintetase (SAS) do tipo anti-PL-7, PL-12 e EJ. MÉTODOS: Estudo de coorte, retrospectivo, envolvendo 20 pacientes com SAS (8 com anti-PL-7, 6 com PL-12, 6 com EJ), em acompanhamento em nosso serviço, entre 1982 e 2012. RESULTADOS: A média de idade dos pacientes ao início da doença foi de 38,5 ± 12,9 anos, e a duração da doença de 4,5 ± 6,4 anos. Setenta por cento dos pacientes eram brancos e 85% eram mulheres. Sintomas constitucionais ocorreram em 90% dos casos. Todos apresentavam fraqueza muscular objetiva dos membros; ao diagnóstico, 30% encontravam-se acamados e 65% com disfagia alta. Envolvimento articular, pulmonar e fenômeno de Raynaud ocorreram, respectivamente, em 50%, 40% e 65% dos casos; mais da metade dos pacientes apresentava pneumopatia incipiente, opacidade em vidro-fosco e/ou fibrose pulmonar. Não houve casos de envolvimento neurológico e/ou cardíaco. Todos receberam prednisona e, como poupadores dessa medicação, diferentes imunossupressores, dependendo da tolerância, efeitos colaterais e/ou refratariedade da doença. De relevância, os pacientes com anti-EJ apresentaram maiores taxas de recidiva. Dois pacientes evoluíram para óbito ao longo do seguimento, e um paciente teve neoplasia mamária na ocasião do diagnóstico da doença. CONCLUSÕES: A SAS (anti-PL-7, PL-12 e EJ) afetou predominantemente mulheres brancas. Embora os autoanticorpos descritos no presente estudo estejam mais relacionados com o acometimento pulmonar comparativamente ao articular, nossos pacientes apresentaram uma porcentagem significativa de ambos e com percentagem alta de miopatia. Além disso, houve menor taxa de mortalidade.

OBJECTIVES: Due to the scarcity of studies in the literature, we conducted an analysis of a series of patients with the anti-PL-7, PL-12 and EJ types of antisynthetase syndrome (ASS). METHODS: We conducted a retrospective cohort study of 20 patients with ASS (8 with anti-PL-7, 6 with PL-12, 6 with EJ) monitored in our department between 1982 and 2012. RESULTS: The mean patient age at disease onset was 38.5 ± 12.9 years, and the disease duration was 4.5 ± 6.4 years. Of all the patients, 70% were white and 85% were female. Constitutional symptoms occurred in 90% of cases. All patients presented objective muscle weakness in the limbs; in addition, 30% were bedridden and 65% demonstrated high dysphagia at diagnosis. Joint and pulmonary involvement and Raynaud's phenomenon occurred in 50%, 40% and 65% of cases, respectively, with more than half of the patients presenting incipient pneumopathy, ground-glass opacity and/or pulmonary fibrosis. There were no cases of neurological and/or cardiac involvement. All patients received prednisone or other immunosuppressants depending on tolerance, side effects and/or disease refractoriness. Importantly, patients with the anti-EJ type of ASS demonstrated higher rates of recurrence. Two patients died during follow-up, and 1 patient had breast cancer at the time of diagnosis. CONCLUSIONS: ASS (anti-PL-7, PL-12 and EJ) was found to predominantly affect white women. Although the autoantibodies described in the present study are more related to pulmonary than joint involvement, our patients showed a significant percentage of both types of involvement and a high percentage of myopathy. We also observed a low mortality rate.

Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis

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. .

Chloroquine diphosphate: a risk factor for herpes zoster in patients with dermatomyositis/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. .

Adult dermatomyositis: experience of a Brazilian tertiary care center.

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.

Dermatomiosite em adulto: experiência de um centro terciário brasileiro / Adult dermatomyositis: experience of a brazilian tertiary care center

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.

[Adalimumab induced-inflammatory myopathy in rheumatoid arthritis]. / Miopatia inflamatória induzida por adalimumab na artrite reumatóide.

The application of immunobiologics for the rheumatoid arthritis treatment may present as a rare complication the development of inflammatory myopathy. Until this moment, there have been described in literature only seven cases of inhibitors of tumor necrosis factor induced-myositis. In this paper, we report the case of the patient with 39 years-old with eight years of arthritis rheumatoid and that due to refractory to various immunosuppressive drugs, the adalimumab was introduced, and evolved to dermatomyositis status.

Síndrome antifosfolípide e dermatomiosite/polimiosite: uma rara associação / Antiphospholipid syndrome and dermatomyositis/polymyositis: a rare association

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.

Prevalência de manifestações clínico-laboratoriais e comorbidades na polimiosite segundo o gênero / Prevalence of clinical and laboratory manifestations and comorbidities in polymyositis according to gender

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 por cento), 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 por cento apresentaram recidiva da doença durante o acompanhamento, com 4,0 por cento 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 por cento) e diabetes mellitus (17,3 por cento), 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 percent), 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 percent experienced disease relapse during follow-up. Nevertheless, two thirds were in remission at the end of this study, with 4.0 percent 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 percent) and diabetes mellitus (17.3 percent), 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.

Prevalence of clinical and laboratory manifestations and comorbidities in polymyositis according to gender.

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.