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dermatomyositis dysphagia

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]...
March 2, 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 anti-NXP-2 antibodies. METHODS: We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. 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...
January 27, 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...
January 15, 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
Yao-Fan Fang, Yeong-Jian Jan Wu, Chang-Fu Kuo, Shue-Fen Luo, Kuang-Hui Yu
This study aims to investigate the prevalence and predictive risk factors of malignancy in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 192 PM/DM patients followed up in a medical center between January 2000 and December 2013 were reviewed. Among the 192 patients, 33 patients (17.2 %) had associated cancer. Both PM and DM are significantly associated with cancer, although the risk of cancer appears to be somewhat higher among patients with DM (23.0 %) than among those with PM (8...
August 2016: Clinical Rheumatology
Naoki Mugii, Minoru Hasegawa, Takashi Matsushita, Yasuhito Hamaguchi, Sacihe Oohata, Hirokazu Okita, Tetsutarou Yahata, Fujiko Someya, Katsumi Inoue, Shigeyuki Murono, Manabu Fujimoto, Kazuhiko Takehara
OBJECTIVE: Dysphagia develops with low frequency in patients with dermatomyositis. Our objective was to determine the clinical and laboratory features that can estimate the development of dysphagia in dermatomyositis. METHODS: This study included 92 Japanese patients with adult-onset dermatomyositis. The associations between dysphagia and clinical and laboratory features including disease-specific autoantibodies determined by immunoprecipitation assays were analyzed...
2016: PloS One
Mariano A Menezes, Fernando A M Herbella, Marco G Patti
Different connective tissue diseases (CTDs), such as dermatomyositis, mixed CTD, rheumatoid arthritis, polymyositis, lupus, and Behçet's, may affect the esophagus, impairing its motor function. The muscular atrophy and fibrosis caused by the autoimmune vasculitis and neuronal dysfunction affect the esophageal body and the lower esophageal sphincter, leading to a clinical presentation of dysphagia and gastroesophageal reflux disease (GERD). The belief that the impaired esophageal motility may negatively affect surgical outcome has led to the common recommendation of avoiding laparoscopic antireflux surgery (LARS) for fear of creating or worsening dysphagia...
April 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
Takeshi Kusunoki, Hirotomo Homma, Yoshinobu Kidokoro, Aya Yanai, Katsuhisa Ikeda, Ryo Wada
We experienced a rare case of laryngeal cancer associated with dermatomyositis. The patient was a 63-year-old male and Japanese. He was admitted to our department of Otorhinolaryngology with dysphagia for a day as a chief complaint. He became aware of hoarseness with bloody sputum and then face edema with redness a half year before. At first physical examination, he had bilateral eyelid edema with erythema, finger edema with keratinizing erythema and limb extensor erythema. Serous creatine phosphokinase was 850 IU/mL (normal range: 40-200 IU/mL)...
November 5, 2015: Clinics and Practice
Angeles Shunashy Galindo-Feria, Jorge Rojas-Serrano, Andrea Hinojosa-Azaola
BACKGROUND: Factors associated with survival in patients with idiopathic inflammatory myopathies are heterogeneous. OBJECTIVE: This study aimed to describe clinical and prognostic factors associated with survival in Mexican patients with idiopathic inflammatory myopathies. METHODS: Patients with dermatomyositis (DM) and polymyositis (PM) seen at a tertiary care center from 1985 to 2012 were included. Demographic and clinical characteristics, comorbidities, treatment, and the time to death were recorded...
March 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
R Goussot, C Wettlé, C Le Coz, B Cribier, D Lipsker
INTRODUCTION: Edematous dermatomyositis is a rare entity with localized or generalized subcutaneous edema and only 21 cases have been reported in the literature. It is considered to be a severe form of dermatomyositis which needs quick therapeutic decision. We report 2 cases with difficult therapeutic decisions. OBSERVATIONS: Two patients aged 23 and 80 years were admitted in hospital for DM with typical cutaneous and muscular involvement without any sign of gravity and which have been treated by steroids: methylprednisolone bolus and prednisone...
March 2016: Annales de Dermatologie et de Vénéréologie
Ilaria Cavazzana, Micaela Fredi, Carlo Selmi, Angela Tincani, Franco Franceschini
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of myositis, characterised by chronic muscle weakness, cutaneous features, different extra-muscular manifestations and circulating autoantibodies. IIMs included classical polymyositis (PM), dermatomyositis (DM) and other different types of myositis with a wide range of muscle involvement. A complete autoantibody profile and a muscle biopsy are mandatory to correctly diagnose different clinical entities and to define their different prognosis...
February 2017: Clinical Reviews in Allergy & Immunology
Sudhir Babu Karri, Meena Anga Muthu Kannan, Liza Rajashekhar, Megha S Uppin, Sundaram Challa
AIMS: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM), applying the European Neuromuscular center (ENMC) criteria. MATERIALS AND METHODS: Adult patients who fulfilled clinical, laboratory, and muscle biopsy findings according to ENMC criteria for DM during the period 2010-2013 were included in the study. Cryostat sections of muscle biopsy were reviewed with emphasis on Perifascicular atrophy (PFA), perivascular/endomysial inflammation...
April 2015: Annals of Indian Academy of Neurology
Florenzo Iannone, Margherita Giannini, Giovanni Lapadula
No abstract text is available yet for this article.
June 2015: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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