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dermatomyositis dysphagia

Rita de Sousa Gameiro, Ana Isabel Alves Reis, Ana Cristina Grilo, Carla Noronha
Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis...
March 5, 2018: BMJ Case Reports
Divya Challa, Cynthia S Crowson, Timothy B Niewold, Ann M Reed
Determinants of changes in disease activity among patients with juvenile dermatomyositis (JDM) are unknown. Our objective was to develop predictive models to predict changes in disease activity using the CARRA Legacy Registry. The CARRA Legacy Registry included 658 subjects with definite or probably JDM with 297 subjects with a one follow-up visit after baseline, and we studied the 65 subjects with active disease at baseline. Linear regression models were used to build risk scores for changes in disease activity adjusted for baseline disease activity, age, sex, and disease duration...
November 23, 2017: Clinical Rheumatology
E Hendren, O Vinik, H Faragalla, R Haq
A 49-year-old woman presents with an extensive violaceous rash, rapidly progressive proximal muscle weakness, and dysphagia to solids, consistent with a diagnosis of dermatomyositis. Two weeks later, she palpates a mass in her left breast and is diagnosed with her2-positive metastatic invasive ductal carcinoma of the breast. There is a well-established association between dermatomyositis and malignancy. However, the specific association between breast cancer and dermatomyositis has not been well characterized...
October 2017: Current Oncology
Shota Kurushima, Yoshiro Horai, Masataka Umeda, Atsushi Kawakami
No abstract text is available yet for this article.
November 1, 2017: Internal Medicine
Emmanuel Ofori, Daryl Ramai, Mel Ona, Madhavi Reddy
Dermatomyositis (DM) is a rare autoimmune condition which predominantly affects females in the fifth and sixth decades of life. DM presents acutely or progressively as painless proximal skeletal muscle weakness and can be associated with a heliotropic rash around the eyes, and Gottron's papules on extensor surfaces of the hands. While the pathophysiology of DM is still unclear, abnormal T- and B-cell immune activity has been reported. DM has been associated with malignancy and has been shown to appear before, concurrently with, or after diagnosis of cancer...
August 2017: Gastroenterology Research
James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E Betteridge, Paul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy
AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. RESULTS: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%)...
August 30, 2017: Annals of the Rheumatic Diseases
Jen-Wei Chou, Yin-Lan Lin, Ken-Sheng Cheng, Po-Yuan Wu, Teressa Reanne Ju
Dermatomyositis or polymyositis as a paraneoplastic syndrome of hepatocellular carcinoma (HCC) is an uncommon event. Few cases have been reported in the literature. We herein report the case of a 55-year-old man with chronic hepatitis B and alcoholism who presented with skin rash. Abdominal computed tomography revealed multiple hypervascular liver tumors consistent with HCC. He subsequently developed dysphagia with proximal limb weakness. Laboratory tests and electromyography demonstrated inflammatory myopathy...
2017: Internal Medicine
Sulaiman M Al-Mayouf, Nora AlMutiari, Mohammed Muzaffer, Rawiah Shehata, Adel Al-Wahadneh, Reem Abdwani, Safia Al-Abrawi, Mohammed Abu-Shukair, Zeyad El-Habahbeh, Abdullah Alsonbul
This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Clinical and laboratory features as well as the long-term outcomes including accrual disease damage were collected at the last follow-up visit...
September 2017: Rheumatology International
Naomi Fei, Sarah Sofka
Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM...
2017: Case Reports in Hematology
Yongpeng Ge, Xin Lu, Xiaoming Shu, Qinglin Peng, Guochun Wang
This study aimed to analyze the clinical features of anti-SAE antibodies in Chinese myositis patients in comparison with different cohorts. The anti-SAE antibodies were tested in myositis patients and in control subjects. Long-term follow-up was conducted on the antibody-positive patients. Anti-SAE antibodies were exclusively present in 12 out of 394 (3.0%) adult dermatomyositis (DM) patients. Of the anti-SAE-positive DM patients, 75% had distinctive diffuse dark-red or pigment-like skin rashes, and 67% of these patients experienced mild muscle weakness...
March 15, 2017: Scientific Reports
Jerry R Mendell, Zarife Sahenk, Samiah Al-Zaidy, Louise R Rodino-Klapac, Linda P Lowes, Lindsay N Alfano, Katherine Berry, Natalie Miller, Mehmet Yalvac, Igor Dvorchik, Melissa Moore-Clingenpeel, Kevin M Flanigan, Kathleen Church, Kim Shontz, Choumpree Curry, Sarah Lewis, Markus McColly, Mark J Hogan, Brian K Kaspar
Sporadic inclusion body myositis, a variant of inflammatory myopathy, has features distinct from polymyositis/dermatomyositis. The disease affects men more than women, most commonly after age 50. Clinical features include weakness of the quadriceps, finger flexors, ankle dorsiflexors, and dysphagia. The distribution of weakness is similar to Becker muscular dystrophy, where we previously reported improvement following intramuscular injection of an isoform of follistatin (FS344) by AAV1. For this clinical trial, rAAV1...
April 5, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
Sumantra Sarkar, Tanushree Mondal, Arpan Saha, Rakesh Mondal, Supratim Datta
OBJECTIVES: To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. METHODS: All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. RESULTS: Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]...
April 2017: Indian Journal of Pediatrics
Kazuhiro Nishikawa, Chu Matsuda, Junji Kawada, Kazumasa Fujitani, Shunji Endo, Motohiro Hirao, Kazuyoshi Yamamoto, Sakae Maeda, Mamoru Uemura, Masakazu Miyake, Naoki Hama, Atsushi Miyamoto, Masataka Ikeda, Shoji Nakamori, Mitsugu Sekimoto
We report a case of rectal metastasis of gastric cancer associated with dermatomyositis showing paraneoplastic syndrome. The patient was a 70-year-old man who had undergone curative total gastrectomy for Stage III Agastric cancer in March 2005. He was diagnosed with dermatomyositis and treated with prednisolone after gastrectomy. In April 2006, erythema of his face relapsed, and his serum CPK level was abnormally elevated. He experienced muscle weakness and dysphagia, and was treated with increased doses of prednisolone and gamma-globulin...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
Anna Rogers, Lorinda Chung, Shufeng Li, Livia Casciola-Rosen, David F Fiorentino
OBJECTIVE: To characterize the cutaneous and systemic clinical phenotype of dermatomyositis patients with antinuclear matrix protein 2 (anti-NXP-2) antibodies. METHODS: We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. An electronic chart review employing a keyword search strategy was performed to collect clinical and laboratory data. Anti-NXP-2 antibodies were assayed by immunoprecipitation using NXP-2 produced by in vitro transcription/translation...
December 2017: Arthritis Care & Research
Siamak Moghadam-Kia, Chester V Oddis, Shinji Sato, Masataka Kuwana, Rohit Aggarwal
OBJECTIVE: To determine the clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM. METHODS: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy...
March 2017: Journal of Rheumatology
Giulia Merlo, Andrea Clapasson, Emanuele Cozzani, Luigi Sanna, Giampaola Pesce, Marcello Bagnasco, Martina Burlando, Aurora Parodi
Autoantibodies are important in the diagnosis of dermatomyositis. They can be divided in two different groups: myositis-associated autoantibodies (MAA) prevailing in overlap syndromes, and myositis-specific autoantibodies (MSA), with diagnostic specificity exceeding 90%. Our purpose was to detect retrospectively the prevalence of the most common MSAs in a group of 19 adult DM patients (13 women, 6 men). A severe DM (SDM), with extensive cutaneous and muscular manifestations, dysphagia, and sometimes pneumopathy, was detected in ten cases...
March 2017: Archives of Dermatological Research
H Rachadi, K Bouayad, S Chiheb
Juvenile dermatomyositis (JDM) is a rare disease, with a mean age of onset of 7 years. We report a case of JDM in a 13-month-old infant. OBSERVATION: A 13-month-old infant presented with an edema of the upper lip, 4 days after receiving amoxicillin-clavulanate. The patient was treated with betamethasone and an antihistamine. Progression was marked by the appearance of a white edema of the periorbital area and the upper lip, with purpuric lesions, aphthoid ulcerations, and a drooping head...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Manabu Fujimoto, Rei Watanabe, Yosuke Ishitsuka, Naoko Okiyama
PURPOSE OF REVIEW: In dermatomyositis, disease-specific autoantibodies now cover more than 70% of patients. These autoantibodies closely correlate with distinct clinical manifestations. In the past few years, extensive evidence has been accumulated on clinical significance of dermatomyositis-specific autoantibodies including autoantibodies against melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), and small ubiquitin-like modifier activating enzyme (SAE)...
November 2016: Current Opinion in Rheumatology
Liguo Yin, Yongpeng Ge, Hanbo Yang, Qinglin Peng, Xin Lu, Yamei Zhang, Guochun Wang
The objectives of this study are to assess the levels of serum Interleukin-35 (IL-35) in patients with idiopathic inflammatory myopathies (IIMs) and to evaluate the association between IL-35 levels and IIM-related features. Serum IL-35 was detected in 76 patients with dermatomyositis (DM), 28 patients with polymyositis (PM), 98 disease controls (40 rheumatoid arthritis (RA), 34 systemic lupus erythematosus (SLE), 12 systemic sclerosis (SSc), and 12 sjogren syndrome (SS)), and 43 healthy controls by ELISA. Follow-up was conducted on 34 patients...
November 2016: Clinical Rheumatology
Hidekazu Yoshie, Ryuto Nakazawa, Wataru Usuba, Hiroya Kudo, Yuichi Sato, Hideo Sasaki, Tatsuya Chikaraishi
We report the first case in Japan of paraneoplastic dermatomyositis with pure seminoma, a tumor that extremely rarely accompanies dermatomyositis. The patient presented to the hospital with muscle weakness and erythema and was diagnosed with dermatomyositis from skin biopsy. Routine radiological screening revealed testicular tumor and massive lymph node metastases. We initially performed orchiectomy along with conventional immunotherapy. However, muscle weakness gradually worsened, and he eventually showed dysphagia and forced respiration and became bedridden...
2016: Case Reports in Urology
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