Morphological alterations of upper gastrointestinal tract in patients with new onset-dermatomyositis: correlation with demographic, clinical and laboratory features / Alterações morfológicas do trato intestinal superior em portadores de dermatomiosite de instalação recente: correlações com características demográficas, clínicas e laboratoriais

OBJECTIVE: To endoscopically assess the upper digestive tract of adult patients with newly diagnosed dermatomyositis; to correlate possible changes in the gastrointestinal tract with demographic, clinical and laboratory features in this population. METHOD: A cross-sectional study evaluating 65 newly diagnosed dermatomyositis cases from 2004 to 2015 was carried out. We excluded patients with clinically amyopathic dermatomyositis, overlap dermatomyositis, polymyositis, liver diseases, prior gastric surgery, upper gastrointestinal tract symptoms (except for upper dysphagia), systemic infections, alcohol consumption and smoking. RESULTS: Mean age of patients was 44.9 years, with disease duration of four months. Endoscopic findings were observed in 70.8% of patients. (1) Esophageal disease/gastric distress was documented in 18.5% of patients: erosive distal esophagitis (16.9%) and non-erosive distal esophagitis distal (1.5%); (2) gastric distress in 63.1% of cases: antral gastritis (42.3%) and pangastritis (27.8%); (3) duodenal involvement in 15.4% of patients: bulbar duodenitis (10.9%) and duodenal ulcers (7.7%). There were no neoplasic lesions. On multivariate analysis, erosive distal esophagitis was less associated with older patients. Males had a higher prevalence of erosive gastritis. Enanthematous pangastritis was less associated with lesions with "V-neck" sign lesions. CONCLUSIONS: This study provides the first estimates of the prevalence of high endoscopic findings in adult patients with newly diagnosed dermatomyositis. The results may be relevant to guide conduct in digestive disorders with upper digestive endoscopy, and point to the need for pharmacological prevention of digestive tract lesions in these patients. Further studies are needed to validate this data and evaluate patients with dyspeptic symptoms.

OBJETIVOS: Avaliar os exames de endoscopia digestiva alta (EDA) de pacientes adultos com DM (dermatomiosite) recém-diagnosticados; correlacionar eventuais alterações do trato gastrintestinal com dados demográficos, clínicos, e medicamentosos desta população. MÉTODO: Estudo transversal, em que foram avaliados 65 casos de DM recém-diagnosticados, no período entre 2004 a 2015. Foram excluídos casos de DM clinicamente amiopática, sobreposição com DM, hepatopatias, cirurgia gástrica prévia, sintomas do trato gastrointestinal (exceto disfagia alta), quadros infecciosos sistêmicos, etilismo e tabagismo. RESULTADOS: A média idade dos pacientes foi de 44,9 anos, com um tempo de sintomas atribuídos a DM de quatro meses. Alterações endoscópicas foram encontradas em 70,8% dos pacientes. O acometimento esofágico/gástrico foi documentado em 18,5% dos pacientes: esofagite distal erosiva (16,9%) e esofagite distal não-erosiva (1,5%); alterações gástricas em 63,1% dos casos: gastrite antral (42,3%) e pangastrite (27,8%); o acometimento duodenal em 15,4% dos pacientes: bulboduodenite (10,9%) e úlcera duodenal (7,7%). Não foram detectadas lesões malignas. Em análise multivariada, a esofagite distal erosiva esteve menos associada a indivíduos de idade maior. Sexo masculino apresentava mais diagnóstico de gastrite erosiva. A pangastrite enantemática esteve menos associada a lesões em "V" do decote. CONCLUSÕES: O presente estudo estima, pela primeira vez, a prevalência de alterações endoscópicas altas em pacientes adultos com DM recém-diagnosticada. Os resultados podem ser relevantes para guiar potenciais alterações digestivas com exame de EDA, bem como apontar para necessidade de prevenção medicamentosa de lesões do trato digestivo nestes pacientes.

Intravenous immunoglobulin contributes to the control of antimelanoma differentiation-associated protein 5 antibody-associated dermatomyositis with palmar violaceous macules/papules.

Autoantibodies to melanoma differentiation-associated protein 5 (MDA5) are associated with a subset of patients with dermatomyositis (DM) who have rapidly progressive interstitial lung disease (RP-ILD) with poor prognosis. Intensive immunosuppressive therapy is initiated before irreversible lung damage can occur; however, there are few lines of evidence for the treatment of RP-ILD. Here, we report three cases of anti-MDA5 antibody-associated DM with RP-ILD in which the patients were treated with combined-modality therapy, including high-dose prednisolone, tacrolimus, intravenous cyclophosphamide and intravenous immunoglobulin (IVIG). In all three cases, serum ferritin levels, which are known to represent the disease activity of RP-ILD, were decreased after IVIG administration. IVIG might contribute to the control of the disease activity of anti-MDA5 antibody-positive DM. Moreover, palmar violaceous macules/papules around the interphalangeal joints, which was observed in all three cases in the incipient stage, might be a useful sign in suggesting a diagnosis of anti-MDA5 antibody-associated DM.

[Clinical features of patients with juvenile and adult dermatomyositis]. / A juvenilis és felnottkori dermatomyositises betegek klinikai jellemzoi.

INTRODUCTION: Juvenile and adult dermatomysitis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. AIM: To compare the symptoms, laboratory and serological findings, treatment and disease course in children and adults suffering from dermatomyositis. METHOD: In this retrospective study, juvenile and adult dermatomyositis groups were formed. There were 27 patients with juvenile dermatomyositis (mean age, 8.7 years; mean follow-up time: 104.6 months) and 30 adult patients (mean age, 50.3; mean follow-up time: 58.1 months). RESULTS: In patients with juvenile dermatomyositis, treatment with intravenous immunoglobulin and cyclosporine A were more frequent as compared to adult patients. Acute onset of the disease was more frequent in adult patients than in those with juvenile disease. In children symptoms of the disease developed gradually. CONCLUSIONS: The findings confirm previously published data showing that there are differences between juvenile and adult patients with dermatomyositis. The authors recommend to follow the patients regularly after reaching remission to avoid bad patient compliance and decrease the number and severity of relapses.

Dermatomiositis juvenil / Dermatomiositis juvenil / Juvenile dermatomyositis

La dermatomiositis juvenil, la miopatia inflamatòria més freqüent en pediatria, és una vasculopatia sistèmica que afecta habitualment la pell i el teixit musculoesquelètic, però que també pot afectar el tracte gastrointestinal i altres òrgans. El diagnòstic es basa en els criteris de Bohan i Peter i l'objectiu del tractament inclou el control dels símp-tomes i la prevenció de les complicacions. Un tractament escalonat precoç disminueix l'activitat de la malaltia i mi-llora el pronòstic a llarg termini

La dermatomiositis juvenil, la miopatía inflamatoria más frecuente en pediatría, es una vasculopatía sistémica que afecta habitualmente la piel y el tejido musculoesquelético, pero que también puede afectar el tracto gastrointestinal y otros órganos. El diagnóstico se basa en los criterios de Bohan y Peter y el objetivo del tratamiento incluye el control de los síntomas y la prevención de las complicaciones. Un tratamiento escalonado precoz disminuye la actividad de la enfermedad y mejora el pronóstico a largo plazo (AU)

Juvenile dermatomyositis, the most common inflammatory myopathy in children, is a systemic vasculopathy that usually affects skin and musculoskeletal tissue, but can also affect the gastrointestinal tract and other organs. Diagnosis is based on the criteria of Bohan and Peter and the treatment goal includes controlling symptoms and preventing complications. Staggered early treatment reduces disease activity and improves long-term prognosis (AU)

[Dermatomyositis with tonsillar carcinoma. Rare presentation as paraneoplastic syndrome]. / Dermatomyositis bei Tonsillenkarzinom. Seltener Zusammenhang als paraneoplastisches Syndrom.

BACKGROUND: Malignant tumors occur in up to 15 % of patients with paraneoplastic syndromes. The temporal association between malignancy and paraneoplasia is variable. Dermatomyositis belongs to the facultative cutaneous paraneoplasia. CASE REPORT: A patient presented with a cervical swelling and preexisting dermatomyositis. Staging revealed a tonsillar carcinoma with cervical, mediastinal and bone metastasis, and meningeal carcinomatosis. Systemic intrathecal chemotherapy was initiated. CONCLUSION: Dermatomyositis has only been described four times worldwide as a paraneoplastic disease with tonsillar carcinoma. Upon occurrence of a paraneoplastic syndrome, an intensive search for tumours is required at regular intervals until the primary tumor is diagnosed.

Genetic risk and protective factors for idiopathic inflammatory myopathy in Koreans and American whites: a tale of two loci.

OBJECTIVE: To better understand genetic contributions to autoimmunity, immunogenetic markers were studied in two racially discrete and geographically isolated populations of patients with idiopathic inflammatory myopathy (IIM). METHODS: Clinical characteristics, as well as clinical and autoantibody subsets, were defined in 151 American white patients and 50 Korean patients with IIM. HLA-DRB1 and DQA1 genotyping was performed on patients and racially matched controls by standard molecular techniques. Gm allotypes and phenotypes were determined by the hemagglutination-inhibition method. RESULTS: HLA-DRB1*0301, the linked allele DQA1*0501, and DRB1 alleles sharing the first hypervariable region motif 9EYSTS13 were major genetic risk factors for the development of myositis in whites (corrected P [Pcorr] < 0.0004, odds ratio [OR] 11.2, 4.5, and 3.1, respectively, for each factor versus controls). Although both the white and Korean patients had a similar distribution of clinical characteristics, autoantibody profiles, and clinical groups, no HLA-DRB1 nor DQA1 allele or motif was found to be a risk factor for IIM in the Korean patients. However, DRB1*14 was a protective factor in Korean patients without myositis-specific autoantibodies (Pcorr = 0.004, OR 0.046). In addition, although no Gm phenotype or allotype was identified as a risk factor in whites, Gm 21 was a protective factor for the development of IIM in Koreans (Pcorr = 0.024, OR 0.3). CONCLUSION: Although myositis patients in the US and Korea share similar clinical and serologic features, the immune response genes predisposing to and protecting from myositis in each of these ethnic groups differ at two chromosomal loci. These data suggest that multiple genetic loci should be studied to identify risk and protective factors for some autoimmune diseases in various ethnic populations.

Dermatopolimiositis / Dermatopolimyositis

Se presenta un caso de dermatopolimiositis de 3 meses de evolucion en un paciente de sexo masculino, de 55 anos de edad. Se comenta la evolucion, resultados de los examenes paraclinicos y la respuesta al tratamiento durante la hospitalizacion