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amyopathic dermatomyositis

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https://www.readbyqxmd.com/read/28274528/predictive-factors-for-long-term-outcome-in-polymyositis-dermatomyositis-associated-interstitial-lung-diseases
#1
Tomoyuki Fujisawa, Hironao Hozumi, Masato Kono, Noriyuki Enomoto, Yutaro Nakamura, Naoki Inui, Ran Nakashima, Yoshitaka Imura, Tsuneyo Mimori, Takafumi Suda
BACKGROUND: Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28210382/paraneoplastic-dermatomyositis-with-cutaneous-and-myopathic-disease-responsive-to-adrenocorticotropic-hormone-therapy
#2
Marisa Wolff, Christopher Mancuso, Karan Lal, Damian Dicostanzo, Charles Gropper
Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy...
January 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/28154280/an-atypical-clinical-course-of-anti-mda5-antibody-positive-interstitial-lung-disease-in-a-patient-with-three-deteriorations-in-9-years
#3
Yuki Sato, Kojiro Otsuka, Koji Tamai, Yuichiro Ono, Yasuhito Hamaguchi, Keisuke Tomii
Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28129490/the-cutaneous-and-systemic-findings-associated-with-nuclear-matrix-protein-2-antibodies-in-adult-dermatomyositis-patients
#4
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
https://www.readbyqxmd.com/read/28127433/fibrocellular-non-specific-interstitial-pneumonia-with-favourable-prognosis-in-clinically-amyopathic-dermatomyositis
#5
Takahiro Yoshizawa, Keishi Sugino, Kazutoshi Shibuya, Toshimasa Uekusa, Sakae Homma
We report the case of a 49-year-old man who experienced dry cough and itching eruption for 2 months. The patient had Gottoron's sign, mechanic's hand, and high levels of serum Krebs von den Lungen-6 and surfactant protein D, but was negative for anti-aminoacyl transfer RNA synthetase antibodies. High-resolution chest computed tomography revealed subpleural band-like or patchy consolidation with predominance in the bilateral lower lobe. Bronchoalveolar lavage fluid showed increased total cell counts with lymphocytosis...
September 2016: Respirology Case Reports
https://www.readbyqxmd.com/read/28099216/a-rare-case-of-amyopathic-juvenile-dermatomyositis-associated-to-psoriasis-successfully-treated-with-ustekinumab
#6
Claudia L Montoya, Martha L Gonzalez, Fabio E Ospina, Gabriel J Tobón
Coexistence between two or more immunological-mediated diseases implies a clinical and therapeutic challenge that is uncommon in the clinical field. We report a rare case of a young patient with amyopathic juvenile dermatomyositis and erythrodermic psoriasis, with an excellent response to ustekinumab.
January 18, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28089977/antimelanoma-differentiation-associated-gene-5-antibody-expanding-the-clinical-spectrum-in-north-american-patients-with-dermatomyositis
#7
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
https://www.readbyqxmd.com/read/27995057/rapidly-progressive-interstitial-lung-disease-due-to-anti-mda-5-antibody-positive-clinically-amyopathic-dermatomyositis-complicated-with-cervical-cancer-successful-treatment-with-direct-hemoperfusion-using-polymyxin-b-immobilized-fiber-column-therapy
#8
Hidenori Ichiyasu, Yasumiko Sakamoto, Chieko Yoshida, Kazuhiko Sakamoto, Ryosuke Fujita, Go Nakayama, Hiroko Okabayashi, Sho Saeki, Shinichiro Okamoto, Hirotsugu Kohrogi
The anti-melanoma differentiation-associated gene 5 (MDA-5) antibody is a marker of clinically amyopathic dermatomyositis (CADM) and rapidly progressive interstitial lung disease (ILD) with acute respiratory failure. A 35-year-old woman with cervical cancer showed Gottron's papules, severe hypoxemia, and diffuse ground-glass opacities on chest computed tomography. She was diagnosed with rapidly progressive ILD associated with CADM. Her serum was positive for the anti-MDA-5 antibody. Combination therapy with corticosteroids, immunosuppressants, and direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP) improved her respiratory dysfunction...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27928683/specific-autoantibodies-in-dermatomyositis-a-helpful-tool-to-classify-different-clinical-subsets
#9
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
https://www.readbyqxmd.com/read/27919567/splicing-factor-proline-glutamine-rich-is-a-novel-autoantigen-of-dermatomyositis-and-associated-with-anti-melanoma-differentiation-associated-gene-5-antibody
#10
Yuji Hosono, Ran Nakashima, Satoshi Serada, Kosaku Murakami, Yoshitaka Imura, Hajime Yoshifuji, Koichiro Ohmura, Tetsuji Naka, Tsuneyo Mimori
OBJECTIVE: Anti-MDA5 antibody positive dermatomyositis (DM) and clinically amyopathic DM (CADM) often develop into rapidly progressive interstitial lung disease, but their pathogenesis remains unclear. We observed that sera from DM/CADM patients immunoprecipitated a common 110 kDa polypeptide. We investigated this autoantigen and its clinical significance. METHODS: Autoantibodies were screened in 333 patients with various connective tissue diseases (CTDs) and 20 healthy controls (HCs) by immunoprecipitation with [(35)S]methionine-labeled HeLa cells...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27856478/prognostic-factors-of-idiopathic-inflammatory-myopathies-complicated-with-interstitial-lung-disease-protocol-for-a-systematic-review-and-meta-analysis
#11
Hiroyuki Kamiya, Ogee Mer Panlaqui, Shinyu Izumi, Takashi Sozu
INTRODUCTION: Idiopathic inflammatory myopathies may be an overlapping disease complex. Although interstitial lung disease affects the mortality and the morbidity of the disease, a clinical course and the prognosis of the disease complicated with interstitial lung disease are diverse among individuals and prognostic factors have yet to be clarified. This article aims to report the rationale and the methodology of a future intended systematic review and meta-analysis of prognostic factors of idiopathic inflammatory myopathies complicated with interstitial lung disease...
November 17, 2016: BMJ Open
https://www.readbyqxmd.com/read/27813167/how-often-are-pediatric-patients-with-clinically-amyopathic-dermatomyositis-truly-amyopathic
#12
Edward J Oberle, Michelle L Bayer, Yvonne E Chiu, Dominic O Co
BACKGROUND: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation. OBJECTIVE: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution. METHODS: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin...
November 4, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27784516/dermatomyositis-part-1-definition-epidemiology-etiology-and-pathogenesis-and-clinics
#13
REVIEW
Marcia Ramos-E-Silva, Ana Paula Frade Lima Pinto, Rodrigo Pirmez, Tullia Cuzzi, Sueli Coelho da Silva Carneiro
Dermatomyositis is an inflammatory autoimmune disorder that affects the skin, muscles, and blood vessels. The prevailing clinical manifestations are intense erythema, typically around the eyes and dorsum of the hands, accompanied by proximal muscle weakness. There is one clinical form, called amyopathic dermatomyositis, which lacks muscular symptoms and laboratory evidence of muscle damage.
2016: Skinmed
https://www.readbyqxmd.com/read/27693019/enzyme-linked-immunosorbent-assays-for-detection-of-anti-transcriptional-intermediary-factor-1-gamma-and-anti-mi-2-autoantibodies-in-dermatomyositis
#14
Manabu Fujimoto, Akihiro Murakami, Shunsuke Kurei, Naoko Okiyama, Atsushi Kawakami, Michiaki Mishima, Shinji Sato, Mariko Seishima, Takafumi Suda, Tsuneyo Mimori, Kazuhiko Takehara, Masataka Kuwana
BACKGROUND: Autoantibodies against transcriptional intermediary factor 1 (TIF1) and Mi-2 are selectively detected in patients with dermatomyositis (DM). To measure these antibodies readily, the development of reliable ELISA systems has been needed. OBJECTIVE: This study aimed to establish enzyme-linked immunosorbent assays (ELISAs) for anti-TIF1γ and anti-Mi-2β antibodies (Abs) and to assess their utility. METHODS: Serum samples were obtained from 104 patients with classic DM, 68 with clinically amyopathic DM (CADM) and 70 with polymyositis, who were followed up at 8 medical centers across Japan...
December 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/27651398/thigh-muscle-mri-in-immune-mediated-necrotising-myopathy-extensive-oedema-early-muscle-damage-and-role-of-anti-srp-autoantibodies-as-a-marker-of-severity
#15
Iago Pinal-Fernandez, Maria Casal-Dominguez, John A Carrino, Arash H Lahouti, Pari Basharat, Jemima Albayda, Julie J Paik, Shivani Ahlawat, Sonye K Danoff, Thomas E Lloyd, Andrew L Mammen, Lisa Christopher-Stine
OBJECTIVES: The aims of this study were to define the pattern of muscle involvement in patients with immune-mediated necrotising myopathy (IMNM) relative to those with other inflammatory myopathies and to compare patients with IMNM with different autoantibodies. METHODS: All Johns Hopkins Myositis Longitudinal Cohort subjects with a thigh MRI (tMRI) who fulfilled criteria for IMNM, dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) or clinically amyopathic DM (CADM) were included in the study...
September 20, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27615411/pirfenidone-in-patients-with-rapidly-progressive-interstitial-lung-disease-associated-with-clinically-amyopathic-dermatomyositis
#16
Ting Li, Li Guo, Zhiwei Chen, Liyang Gu, Fangfang Sun, Xiaoming Tan, Sheng Chen, Xiaodong Wang, Shuang Ye
To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants...
September 12, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27533321/recent-advances-in-dermatomyositis-specific-autoantibodies
#17
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
https://www.readbyqxmd.com/read/27452897/antimelanoma-differentiation-associated-protein-5-antibody-level-is-a-novel-tool-for-monitoring-disease-activity-in-rapidly-progressive-interstitial-lung-disease-with-dermatomyositis
#18
T Matsushita, K Mizumaki, M Kano, N Yagi, M Tennichi, A Takeuchi, Y Okamoto, Y Hamaguchi, A Murakami, M Hasegawa, M Kuwana, M Fujimoto, K Takehara
BACKGROUND: Antimelanoma differentiation-associated protein (anti-MDA)5 antibodies are associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). OBJECTIVES: We aimed to evaluate the relevance of monitoring anti-MDA5 antibody levels for the management of RP-ILD in patients with CADM or DM. METHODS: Twelve patients with CADM (n = 10) or DM (n = 2) accompanied by RP-ILD were included...
February 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/27433359/a-curious-case-of-acute-respiratory-failure-is-it-antisynthetase-syndrome
#19
Gurveen Malhotra, Nitya Ramreddy, Serafin Chua, Mira Iliescu, Tanjeev Kaur
Antisynthetase (AS) syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Although it is usually associated with elevated creatine phosphokinase level, some patients may have amyopathic dermatomyositis (ADM) like presentation with predominant skin involvement. Interstitial lung disease (ILD) is the main pulmonary manifestation and may be severe thereby determining the prognosis. It may rarely present with a very aggressive course resulting in acute respiratory distress syndrome (ARDS)...
2016: Case Reports in Critical Care
https://www.readbyqxmd.com/read/27354955/lung-histopathological-pattern-in-a-survivor-with-rapidly-progressive-interstitial-lung-disease-and-anti-melanoma-differentiation-associated-gene-5-antibody-positive-clinically-amyopathic-dermatomyositis
#20
Atsushi Suzuki, Yasuhiro Kondoh, Hiroyuki Taniguchi, Kazuhiko Tabata, Tomoki Kimura, Kensuke Kataoka, Kenzo Ono, Mikiko Hashisako, Junya Fukuoka
Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are specific indicators of patients with dermatomyositis, particularly clinically amyopathic dermatomyositis (CADM). CADM is occasionally accompanied by fatal, treatment-resistant, rapidly-progressive interstitial lung disease (RP-ILD). All previous reports showed that histopathological findings in RP-ILD with anti-MDA5 antibody-positive CADM indicated diffuse alveolar damage (DAD). This is the first report describing a non-DAD pattern in RP-ILD with anti-MDA5 antibody-positive CADM, which was improved by immunosuppressive therapy...
2016: Respiratory Medicine Case Reports
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