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https://www.readbyqxmd.com/read/28384112/inflammatory-myopathies-with-cutaneous-involvement-from-diagnosis-to-therapy
#1
Lyubomir A Dourmishev
The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Recently, numerous new antibodies defining the characteristic clinical phenotype have been described as anti-MDA5 antibodies associated with interstitial lung disease and amyopathic dermatomyositis or anti-TIF1γ antibodies as markers for paraneoplastic dermatomyositis...
March 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28346662/intravenous-immunoglobulin-contributes-to-control-anti-melanoma-differentiation-associated-protein-5-mda5-antibody-associated-dermatomyositis-with-palmar-violaceous-macules-papules
#2
H Koguchi-Yoshioka, N Okiyama, K Iwamoto, Y Matsumura, T Ogawa, S Inoue, R Watanabe, M Fujimot
Autoantibodies to melanoma differentiation-associated protein 5 (MDA5) are associated with a subset of dermatomyositis (DM) patients 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 3 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 immunoglobulins (IVIG)...
March 27, 2017: British Journal of 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/28127433/fibrocellular-non-specific-interstitial-pneumonia-with-favourable-prognosis-in-clinically-amyopathic-dermatomyositis
#4
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/28089977/antimelanoma-differentiation-associated-gene-5-antibody-expanding-the-clinical-spectrum-in-north-american-patients-with-dermatomyositis
#5
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/28040421/anti-mda5-dermatomyositis-mimicking-psoriatic-arthritis
#6
Iván Cabezas-Rodríguez, Isla Morante-Bolado, Anahy Brandy-García, Rubén Queiro-Silva, Lourdes Mozo, Francisco Javier Ballina-García
Dermatomyositis causes inflammation and damage of muscle and skin, and sometimes involves internal organs, especially lung parenchyma. Patients with dermatomyositis still represent a diagnostic challenge because of the rarity of this disease and the lack of specificity of some of its cutaneous manifestations. Herein, we describe the case of a patient with dermatomyositis, initially diagnosed as psoriatic arthritis, in which the performance of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was decisive to establish a definitive diagnosis...
December 28, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/28012490/association-of-anti-aminoacyl-transfer-rna-synthetase-antibody-and-anti-melanoma-differentiation-associated-gene-5-antibody-with-the-therapeutic-response-of-polymyositis-dermatomyositis-associated-interstitial-lung-disease
#7
Naomi Yoshida, Masaki Okamoto, Shinjiro Kaieda, Kiminori Fujimoto, Tomohiro Ebata, Morihiro Tajiri, Masayuki Nakamura, Masaki Tominaga, Daisuke Wakasugi, Tomotaka Kawayama, Masataka Kuwana, Tsuneyo Mimori, Hiroaki Ida, Tomoaki Hoshino
BACKGROUND: We attempted to clarify whether the presence of anti-aminoacyl-transfer RNA synthetase antibody (anti-ARS Ab) or anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is associated with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). METHODS: We retrospectively investigated 22 patients with PM/DM-ILD (10 positive for anti-ARS Ab and nine positive for anti-MDA5 Ab) for whom antibody analysis of conserved serum was possible...
January 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/27928683/specific-autoantibodies-in-dermatomyositis-a-helpful-tool-to-classify-different-clinical-subsets
#8
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
#9
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/27888997/comprehensive-assessment-of-myositis-specific-autoantibodies-in-polymyositis-dermatomyositis-associated-interstitial-lung-disease
#10
Hironao Hozumi, Tomoyuki Fujisawa, Ran Nakashima, Takeshi Johkoh, Hiromitsu Sumikawa, Akihiro Murakami, Noriyuki Enomoto, Naoki Inui, Yutaro Nakamura, Yuji Hosono, Yoshitaka Imura, Tsuneyo Mimori, Takafumi Suda
OBJECTIVES: Myositis-specific autoantibodies (MSAs) are associated with clinical phenotypes in polymyositis/dermatomyositis (PM/DM). No study has investigated the clinical features based on comprehensive MSA assessment in PM/DM-associated interstitial lung disease (ILD). We aimed to determine the practical significance of MSAs in PM/DM-ILD. METHODS: Sixty consecutive PM/DM-ILD patients were retrospectively analysed. Serum MSAs were comprehensively measured using immunoprecipitation assay...
December 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27851860/dermatopulmonary-syndrome-associated-with-anti-mda5-antibodies-after-allogeneic-hematopoietic-stem-cell-transplantation
#11
Clémence Lepelletier, Djaouida Bengoufa, Zeltni Lyes, Adèle de Masson, François Chasset, Marie Jachiet, David Michonneau, Marie Robin, Régis Peffault de Latour, Flore Sicre de Fontbrune, Yacine Tandjaoui-Lambiotte, Armand Bensussan, Michel Rybojad, Abdellatif Tazi, Martine Bagot, Gérard Socié, Anne Bergeron, Jean-David Bouaziz
Importance: Chronic graft-vs-host-disease (cGVHD) after allogeneic stem cell transplantation (AHSCT) may resemble autoimmune diseases. Anti-MDA5 (melanoma differentiation-associated gene 5) dermatopulmonary syndrome is a subset of dermatomyositis defined by specific clinical features and detection of anti-MDA5-antibodies in the serum. Objective: To characterize the clinical features of patients who underwent AHSCT and screened positively for anti-MDA5 antibodies...
November 16, 2016: JAMA Dermatology
https://www.readbyqxmd.com/read/27761751/myositis-specific-autoantibodies-and-their-association-with-malignancy-in-italian-patients-with-polymyositis-and-dermatomyositis
#12
Angela Ceribelli, Natasa Isailovic, Maria De Santis, Elena Generali, Micaela Fredi, Ilaria Cavazzana, Franco Franceschini, Luca Cantarini, Minoru Satoh, Carlo Selmi
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis...
February 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/27588444/clinical-analysis-and-outcome-of-interstitial-lung-disease-complicated-with-juvenile-dermatomyositis-and-juvenile-polymyositis
#13
Satoshi Sato, Yoji Uejima, Meika Nanbu, Eisuke Suganuma, Tadamasa Takano, Risa Tanaka, Tomoyuki Kabuki, Eiji Oguma, Tsutomu Oh-Ishi, Yutaka Kawano
OBJECTIVES: The aim of this study was to determine the clinical phenotype and outcome of interstitial lung disease (ILD) complicated with juvenile dermatomyositis (JDM) or juvenile polymyositis (JPM). METHODS: This was a single-center retrospective study. From 1984 to 2015, we retrospectively reviewed 29 patients who were diagnosed with JDM/JPM, among whom eight cases were ILD and 21 were non-ILD. The clinical features and laboratory findings included chest computed tomography (CT) images that were compared between the patients with ILD and non-ILD...
September 20, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27533321/recent-advances-in-dermatomyositis-specific-autoantibodies
#14
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/27523002/radiological-and-pathological-correlation-in-anti-mda5-antibody-positive-interstitial-lung-disease-rapidly-progressive-perilobular-opacities-and-diffuse-alveolar-damage
#15
Haruka Chino, Akimasa Sekine, Tomohisa Baba, Tae Iwasawa, Koji Okudela, Tamiko Takemura, Harumi Itoh, Shinji Sato, Yasuo Suzuki, Takashi Ogura
We herein present the first case of rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody evaluated by surgical lung biopsy (SLB). High-resolution CT scan revealed perilobular opacities, which rapidly became thicker and formed consolidation, resulting in remarkable loss of lung volume. Specimens taken from SLB revealed membranous organization with alveolar occlusion, dilation of alveolar ducts, and sacs with collapsed alveoli, which are typical features of diffuse alveolar damage (DAD)...
2016: Internal Medicine
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
#16
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/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
#17
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
https://www.readbyqxmd.com/read/27350281/anti-mda5-positive-dermatomyositis-presenting-as-fever-of-unknown-origin
#18
Lori W Lee, Neera S Narang, Anna Postolova, Nicole Seminara, Molly A Kantor
Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations...
December 2016: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/27307530/gottron-papules-and-gottron-sign-with-ulceration-a-distinctive-cutaneous-feature-in-a-subset-of-patients-with-classic-dermatomyositis-and-clinically-amyopathic-dermatomyositis
#19
Hua Cao, Qunli Xia, Meng Pan, Xiaoqing Zhao, Xia Li, Ruofei Shi, Min Zhou, Xiaoyi Ding, Masataka Kuwana, Jie Zheng
OBJECTIVE: Gottron papules and Gottron sign are characteristic and possibly pathognomonic cutaneous features of classic dermatomyositis and clinically amyopathic dermatomyositis (DM/CADM). However, the Gottron papules/Gottron sign with cutaneous ulceration (ulcerative Gottron papules/Gottron sign) are less common. We aimed to clarify the clinical characteristics of patients with DM/CADM who have ulcerative Gottron papules/Gottron sign. METHODS: Clinical features, laboratory findings, and prognosis of patients with DM/CADM who had Gottron papules/Gottron sign with or without ulceration were analyzed and compared...
September 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27252271/clinical-significance-and-new-detection-system-of-autoantibodies-in-myositis-with-interstitial-lung-disease
#20
REVIEW
R Nakashima, Y Hosono, T Mimori
Anti-aminoacyl-tRNA synthetase (ARS) and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are closely associated with interstitial lung disease in polymyositis and dermatomyositis. Anti-ARS-positive patients develop common clinical characteristics termed anti-synthetase syndrome and share a common clinical course, in which they respond well to initial treatment with glucocorticoids but in which disease tends to recur when glucocorticoids are tapered. Anti-MDA5 antibody is associated with rapidly progressive interstitial lung disease and poor prognosis, particularly in Asia...
July 2016: Lupus
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