Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study.

Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers. Patients and methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan). Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups. Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution.

Clinical manifestations, outcomes, and antibody profile of Brazilian adult patients with dermatomyositis: a single-center longitudinal study.

BACKGROUND: Currently, only a few retrospective cohort or cross-sectional studies have described the general characteristics of Brazilian patients with classical dermatomyositis (DM). In contrast, we aimed to longitudinally assess a large sample of these patients, and several myositis autoantibodies. METHODS: This single-center longitudinal study included 91 Brazilian adults with defined DM (EULAR/ACR 2017) who underwent follow-up appointments in our tertiary center from 2012 to 2021. Myositis autoantibody analysis was performed using a commercial kit. RESULTS: The mean age of the patients was 47.3 ± 15.4 years, with a predominance of female (67.0%) and White (81.3%) patients. As an initial treatment, 76.9% of the patients received methylprednisolone pulse therapy, 59.3% received intravenous immunoglobulin, and 54.9% received both drugs. The median follow-up duration was 44 months (interquartile 17-67) months. There were 92 severe episodes of infection, and neoplasms were identified in 20 patients (22.0%). Hypertension was identified in 46.2% of patients, whereas diabetes mellitus and myocardial infarction occurred in 19.8% and 4.4%, respectively. Nine patients died during the follow-up. At the last visit, one-third of the patients had disease activity, half had a complete clinical response, and one-fifth were in disease remission. In a univariate logistic regression, anti-aminoacyl-tRNA synthetase antibodies (n = 13) were associated with interstitial lung disease, "mechanic's hands", and anti-Ro-52, and had an inverse association with "V"-neck and "shawl" signs. Anti-MDA-5 (n = 10) were associated with male gender, digital ulcers, vasculitis, arthritis, anti-Ro-52, and active disease. Anti-Ro-52 (n = 26) were associated with "mechanics' hands", arthritis, interstitial lung disease, anti-tRNA synthetases, and anti-MDA-5. No association was found for anti-Mi-2 (n = 10). CONCLUSIONS: This study shows the general profile of a significant sample of Brazilian patients with DM as well as the association of some antibodies with clinical and laboratory manifestations of this myositis.

The relevance of anti-Jo-1 autoantibodies in patients with definite dermatomyositis.

BACKGROUND: To assess the prevalence and clinical relevance of anti-Jo-1 autoantibodies in a representative sample of patients with definite dermatomyositis (DM). METHODS: This retrospective cohort study took place from 2005 to 2020 and assessed 118 adult patients from a tertiary center who were diagnosed with definite DM. A commercial kit was used to detect anti-Jo-1 autoantibodies. RESULTS: The presence of anti-Jo-1 autoantibodies was observed in 10 out of 118 (8.5%) patients with definite DM. The following variables were comparable between individuals with and without anti-Jo-1 autoantibodies: age at diagnosis, sex, ethnicity, disease duration, follow-up period, recurrence rate, complete clinical response, death rate, and cancer incidence. There was no difference in clinical features between groups, except for an increased prevalence of "mechanic's hands," joint involvement, and lung disease, as well as a reduced occurrence of skin findings in patients positive for anti-Jo-1 autoantibodies. No anti-Jo-1-positive patients went into remission; they required greater use of glucocorticoids and immunosuppressive drugs. CONCLUSIONS: Anti-Jo-1 positivity was found in 8.5% of patients with definite DM. This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM.

A prospective cross-sectional study of serum IL-17A in antisynthetase syndrome.

OBJECTIVES: The pro-inflammatory interleukin (IL)-17A serum has been characterized in several systemic autoimmune diseases, but not in antisynthetase syndrome (ASS). Therefore, the present study aims firstly to assess the serum level of the IL-17A in patients with ASS, comparing with healthy individuals, and secondly to analyze prospectively this IL in patients with refractory ASS undergoing rituximab treatment. MATERIALS AND METHODS: A cross-sectional, single-center study that included 64 patients with ASS who were age-, gender-, and ethnicity-matched to 64 healthy individuals. Disease status was measured by the International Myositis Assessment and Clinical Studies Group (IMACS) set scores. Secondarily, the patients with refractory disease treated with rituximab were prospectively followed for 12 months. The IL-17A was assessed by the ELISA method. RESULTS: The mean age of the patients was 44.8 ± 11.8 years, with a predominance of female gender and Caucasian. The median serum IL-17A level was higher in ASS patients compared with healthy individuals: 9.7 (9.1-10.4) vs. 7.7 (5.7-9.0) pg/mL, respectively, and P < 0.001. However, the demographical, clinical, and laboratory data indicates that disease status did not correlate with serum levels of the IL-17A in ASS patients. Prospectively, 16 patients received rituximab, and there was a drop of IL-17A serum level over the first year of treatment in these patients: from 9.7 (9.1-10.6) to 9.0 (8.2-9.7) pg/mL (P = 0.01). CONCLUSIONS: Our study demonstrated that patients with ASS have increased serum levels of the IL-17A compared with healthy controls. In addition, the patients with refractory ASS treated with rituximab showed a reduction of the serum levels of the IL-17A. Key Points • Patients with ASS have increased serum levels of the IL-17A. • Patients with refractory ASS treated with rituximab showed a reduction of the serum levels of the IL-17A.

The Brazilian Society of Rheumatology recommendations on investigation and diagnosis of systemic autoimmune myopathies.

BACKGROUND: This research is recommended by the Myopathy Committee of the Brazilian Society of Rheumatology for the investigation and diagnosis of systemic autoimmune myopathies. BODY: A systematic literature review was performed in the Embase, Medline (PubMed) and Cochrane databases, including studies published until October 2018. PRISMA was used for the review, and the articles were evaluated, based on the Oxford levels of evidence. Ten recommendations were developed addressing different aspects of systemic autoimmune myopathy investigation and diagnosis. CONCLUSIONS: The European League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification stands out for the diagnosis of systemic autoimmune myopathies. Muscular biopsy is essential, aided by muscular magnetic resonance images and electroneuromyography in complementary research. Analysis of the factors related to prognosis with the evaluation of extramuscular manifestations, and comorbidities and intense investigation regarding differential diagnoses are mandatory.

Nailfold capillary changes in adult new-onset dermatomyositis: a prospective cross-sectional study.

OBJECTIVES: It is to prospectively analyze nailfold capillaroscopy (NC) findings in new-onset dermatomyositis (DM) and to correlate NC findings with serum angiogenic cytokines and DM clinical and laboratory features. MATERIALS AND METHODS: Twenty-three patients with DM who experienced < 12 months of symptoms were included in the study. To assess serum cytokine levels, 23 age-, sex-, and ethnicity-matched healthy volunteers were used. NC characteristics and DM activity parameters were analyzed. RESULTS: Significantly higher serum angiogenin (ANG) and vascular endothelial growth factor-1 (VEGF1) levels were observed in DM patients than in controls. Capillary density and avascular areas correlated positively and negatively, respectively, with serum levels of ANG. Moreover, the capillary density correlated inversely with the number of enlarged and giant capillaries and avascular areas. The number of enlarged capillaries correlated positively with patient and physician visual analogue scales (VAS), the presence of a facial rash, giant capillaries, and microhemorrhages. Giant capillaries had a positive correlation with physician and cutaneous VAS, enlarged capillaries, avascular areas, microhemorrhages and bushy capillaries, and a negative correlation with capillary density. Microhemorrhages correlated positively with the "V-neck" sign and physician VAS. VEGF1 showed no relationship with the NC parameters with DM-related clinical and laboratory features. Additionally, 15 out of 23 patients were assessed prospectively after 3.21 years. All patients had a major clinical response with significant improvement in all NC parameters, except for enlarged and bushy capillaries. CONCLUSIONS: The NC may be a useful tool to assess disease activity in recent-onset DM, and it can also reinforce the role of ANG in the angiogenesis of this myopathy.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies.

BACKGROUND: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). MAIN BODY: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. CONCLUSIONS: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strength-building and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies.

BACKGROUND: Interpretation of rituximab efficacy for refractory idiopathic inflammatory myopathies (IIM) is hampered by the absence of a uniform definition of refractory myositis and clinical response. Therefore, rigorous criteria of refractoriness, together with a homogenous definition of clinical improvement, were used to evaluate rituximab one-year response. METHODS: A retrospective cohort study including 43 IIM (15 antisynthetase syndrome, 16 dermatomyositis, 12 polymyositis) was conducted. All patients had refractory disease (inadequate response to at least two immunosuppressives/immunomodulatories and no less than three months sequentially or concomitantly glucocorticoid tapering) criteria. Clinical/laboratory improvement at one-year was based on modified International Myositis Assessment & Clinical Studies Group (IMACS) core set measures. The patients received two infusions of rituximab (1 g each) at baseline, followed by repeated dose after 6 months. Baseline immunosuppressive therapy was maintained and glucocorticoid dose was tapered according to clinical/laboratory parameters. RESULTS: Five patients had side effects at the first rituximab application and were excluded. Therefore, 38 out of 43 patients completed the one-year follow up. Almost 75% of the patients attained clinical and laboratory response after one-year. A significant reduction in median glucocorticoid dose (18.8 vs. 6.3 mg/day) was achieved and 42% patients were able to discontinue prednisone. In contrast, young individuals and patients with dysphagia had a tendency to be non-responders to rituximab. No severe infections were observed. CONCLUSION: This study provides convincing evidence that rituximab is an effective and safe therapy for refractory IIM.

Mycophenolate mofetil in patients with refractory systemic autoimmune myopathies: case series.

BACKGROUND: Currently, there are only few studies (mostly case reports or case series) on mycophenolate mofetil (MMF) in patients with systemic autoimmune myopathies (SAM). Therefore, the goal of the present study was to evaluate the safety and efficacy of MMF (monotherapy or coadjuvant drug) in a specific sample of patients with refractory SAM: dermatomyositis, polymyositis, anti-synthetase syndrome or clinically amyopathic dermatomyositis. METHODS: A case series including 20 consecutive adult patients with refractory SAM from 2010 to 2016 was conducted. After the introduction of MMF, associated or not with other drugs, the patients were followed for 6 consecutive months. RESULTS: In 17 out of 20 patients MMF was introduced without any intolerance. The clinical symptoms evaluated in these patients were muscular, cutaneous and/or pulmonary activity. During the 6-month follow-up, 11 out of 17 patients had clinical and laboratory activities response with MMF, allowing significant tapering of the prednisone median dose (15 vs. 5 mg/day, P=0.005). On the other hand, in three out of 20 patients; MMF was discontinued in less than two months, because of gastrointestinal intolerance. There were no cases of serious infection or death. CONCLUSIONS: MMF was relatively well-tolerated, safe and effective in patients with refractory SAM. Further studies are needed to confirm the data found.

Necrotising myopathy associated with anti-signal recognition particle (anti-SRP) antibody.

OBJECTIVES: Prompted by the few studies available in the literature, we analysed patients with necrotising myopathy associated with anti-signal recognition particle (anti-SRP). METHODS: We conducted a retrospective, single-centre cohort study involving 14 patients with anti-SRP antibody followed between 2001 and 2016. RESULTS: Patients had a mean age at disease onset of 40.7 years and were predominantly female and of white ethnicity. At disease onset, all patients had limb muscle weakness with median serum of creatine phosphokinase level of 8080U/L, 64.3% had constitutional symptoms, 50% dysphagia, 42.9% myalgia, 21.4% and 14.3% pulmonary and articular involvement, respectively. There were no cases of cutaneous, neurological or cardiac involvements. Notably, 21.4% of patients had previous exposure to statins. Moreover, with the exception of one patient, all received methylprednisolone pulse therapy and/or human intravenous immunoglobulin (IVIg), as well as prednisone and different immunosuppressive drugs or rituximab. Relapse occurred in 64.3% of the cases. However, most patients had significant recovery of muscle strength, with half no longer using glucocorticoids and the remainder on a weaning regimen with low dose prednisone. CONCLUSIONS: Unlike the cases described in the literature, there was a high frequency of extra-muscular symptoms in the patients studied. Moreover, one fifth of patients had previous exposure to statin use. There was a high relapse rates, but with good clinical and laboratory recovery, especially with pulse therapy regimen of methylprednisolone and/or IVIg.

Electrocardiographic changes in dermatomyositis and polymyositis.

INTRODUCTION: Cardiac involvement is frequent in inflammatory myopathies. Electrocardiogram (ECG) may show evidence of this involvement and its changes should be well-known and described. OBJECTIVES: Due to the lack of studies in the literature, we conducted an analysis of the ECG findings in patients with dermatomyositis (DM) and polymyositis (PM), comparing them with a control group. METHODS: This cross-sectional study compared the ECG of 86 individuals with no rheumatic disorders (controls) with 112 patients (78 DM and 34 PM), during 2010-2013. The ECG findings between DM and PM were also compared. RESULTS: Demographic characteristics, comorbidities and ECG abnormalities were similar between controls and patients (p>0.05), except for a higher frequency of left ventricular hypertrophy (LVH) in patients (10.7% vs. 1.2%, p=0.008). Demographic characteristics, comorbidities, clinical and laboratory manifestations, were also similar between the groups PM and DM, except for the presence of cutaneous lesions only in DM. One-third of the patients had ECG abnormalities, which were more prevalent in PM than DM (50% vs. 24.4%, p=0.008). LVH, left atrial enlargement, rhythm and conduction abnormalities were more frequent in PM than DM (p<0.05 for all), especially the left anterior fascicular block. CONCLUSIONS: We showed distinct ECG changes between DM and PM and a higher frequency of LVH in patients compared to controls. Investigation of cardiac involvement should be considered even in asymptomatic patients, especially PM. Further studies are necessary in order to determine the correlation of ECG findings with other complementary tests, clinical manifestations, disease activity and progression to other cardiac diseases.

Reduced ovarian reserve in patients with adult polymyositis.

Polymyositis (PM) affects female gender during reproductive age; however, there is no study assessing ovarian reserve in these patients to evaluate ovarian reserve markers in PM. Eight female patients with PM (Bohan and Peter criteria, 1975) with aged 18-40 years, followed at our tertiary centre from March 2011 to May 2014, were invited to participate. They were age-matched with 16 healthy individuals (control group). All were evaluated at early follicular phase of menstrual cycle. Follicle stimulating hormone (FSH), estradiol, inhibin B, anti-Müllerian hormone (AMH) serum levels (ELISA) and sonographic antral follicle count (AFC) were determined. PM patients and controls had comparable mean age (31.4 ± 6.5 vs. 30.7 ± 6.2 years, P = 0.946), ethnicity and socioeconomic class (P > 0.05). PM mean age of onset was 27.3 ± 6.5 years and disease duration of 6.5 ± 4.1 years. Menstrual cycles were alike in both groups with a similar frequency of age at menarche, gynaecological age, duration and length of menstrual cycle (P > 0.05). The median serum level of AMH was significantly lower in PM compared to controls [0.7(0.3-3.4) vs. 3.1(1.4-4.0), P = 0.021]. AMH levels ≤1 ng/mL (50 vs. 6.3 %, P = 0.024) and very low AFC (37.5 vs. 6.3 %, P = 0.037) were significantly in PM patients versus controls. The other hormones (FSH, inhibin B and estradiol levels) were similar between both groups (P > 0.05). The present study was the first to identify subclinical ovarian dysfunction in PM patients during reproductive ages. Further study is necessary to assess the possible role of PM-related factors that may influence the ovarian function of these patients.

IgA nephropathy and polymyositis: a rare association.

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.

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. .

Dermatomyositis and polymyositis: from immunopathology to immunotherapy (immunobiologics).

Idiopathic inflammatory myopathies (IIM), which include dermatomyositis (DM) and polymyositis (PM), are chronic systemic diseases associated with high morbidity and functional disability. Current treatment is based on the use of glucocorticoids and immunosuppressive drugs, but a considerable number of patients is refractory to traditional therapy. That has led to the attempted use of biologics based on the physiopathogenesis of IIM. From the immunopathological viewpoint, PM and DM differ: the former is more related to cellular immunity, while the latter, to humoral immunity. In both, however, elevated concentrations of proinflammatory interleukins (TNF, IL-1, IL-6) and increased expression of molecules related to costimulation of T lymphocytes have been described; thus, the use of biologics in those conditions seems reasonable. Considering the biologics available, open-label studies are scarce, comprising mainly case reports and series. TNF blockers have yielded conflicting results, with no evidence of good response to treatment. The anti-CD20 therapy has the most promising results. Data on T lymphocyte costimulation blockade and anti-IL-6 therapy are extremely scarce, preventing any consideration. Thus, the use of biologics in IIM still remains an unconquered frontier. Biologics may have an important role in the management of IIM refractory to conventional therapy, but further prospective studies based on objective parameters of response to treatment are needed. So far, anti-CD20 therapy seems to be the most promising treatment for refractory IIM.

Analysis of metabolic syndrome in adult dermatomyositis with a focus on cardiovascular disease.

OBJECTIVE: To evaluate the frequency of metabolic syndrome in dermatomyositis (DM) patients and to analyze the possible association of metabolic syndrome with traditional cardiovascular disease (CVD) risk factors and DM-related clinical and laboratory features. METHODS: The present cross-sectional single-center study included 84 DM patients and 105 healthy controls. Metabolic syndrome was diagnosed according to the National Cholesterol Education Program Adult Treatment Panel III. RESULTS: The median age was similar in both the DM and control groups (41.5 and 42.0 years, respectively; P = 0.378), with a comparable predominance of women (P = 0.904) and white race (P = 0.623). The DM patients had a higher prevalence of metabolic syndrome (41.7% versus 7.0%; P < 0.001), diabetes mellitus (17.9% versus 1.0%; P < 0.001), stroke (4.8% versus 0%; P = 0.024), and family history of CVD (23.8% versus 8.6%; P = 0.004). However, the frequency of sedentarism, hypothyroidism, smoking, and alcohol intake was similar in both groups (P > 0.05). Further analysis of the DM patients with (n = 35) and without (n = 49) metabolic syndrome revealed that the patients with this complication were older (mean ± SD age 50.0 ± 14.5 years versus 40.9 ± 14.6 years; P = 0.006) and had a similar disease duration (P = 0.925) and higher prevalence of systemic arterial hypertension prior to the onset of disease symptoms (54.3% versus 10.2%; P < 0.001). In a multivariate analysis, only hypertension diagnosed prior to the disease was associated with metabolic syndrome (odds ratio 10.47 [95% confidence interval 2.62-44.81]). CONCLUSION: Metabolic syndrome is highly prevalent in DM, and prior hypertension seems to be a major determinant of its development, while disease- and therapy-related factors do not appear to play a relevant role.

[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.

Newly diagnosed dermatomyositis in the elderly as predictor of malignancy.

OBJECTIVE: Dermatomyositis (DM) symptoms may be a clue to the existence of a hidden cancer. Enhancing early detection is essential, but there are no studies evaluating short-term predictive factors in this disease. METHODS: This is a single-center retrospective study, including patients diagnosed with DM meeting at least four of the five Bohan and Peter's criteria (1975), from 1991 to 2011. This study assessed malignancies occurring in up to 12 months after the diagnosis of DM. RESULTS: Neoplasm was found in 12 out of 139 patients (skin, gastrointestinal tract, prostate, thyroid, breast, lungs, and genitourinary tract). Patients with neoplasm had a higher mean age than controls (56.8 ± 15.7 vs. 40.3 ± 13.1 years, respectively, P = 0.004, odds ratio 1.09; 95% confidence interval: 1.04-1.14). No statistical differences were observed regarding gender, ethnicity, frequency of constitutional symptoms, organ and systemic involvements, and/or laboratory alterations. CONCLUSION: In newly diagnosed DM, age at disease diagnosis was a predictive factor of malignancy.

[Autoimmune hepatitis and dermatomyositis: a rare association]. / Hepatite autoimune e dermatomiosite: uma rara associação.

The association between autoimmune hepatitis and idiopathic inflammatory myopathies has been rarely described in literature. To our knowledge, there are only five reports of autoimmune hepatitis, all coursing with polymyositis. In the present work, we describe a female patient at the age of 58 with cutaneous lesions (heliotrope), progressive proximal muscle weakness of four limbs and constitutional symptoms for 12 months, and worsened two months ago. She had also been episodes of jaundice for five months. During hospitalization, after intense clinical investigation, the diagnosis of dermatomyositis and autoimmune hepatitis were defined, and the patient had a good clinical and laboratory response to corticosteroids and immunosuppressive.