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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...
October 20, 2016: Clinical Rheumatology
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
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
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
T Matsushita, K Mizumaki, M Kano, N Yagi, M Tennichi, A Takeuchi, Y Okamoto, Y O Hamaguchi, A Murakami, M Hasegawa, M Kuwana, M Fujimoto, K Takehara
BACKGROUND: Anti-MDA5 antibodies are associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). OBJECTIVE: 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 or DM (CADM, 10; DM, 2) accompanied by RP-ILD were included. Baseline characteristics and outcomes were recorded...
July 25, 2016: British Journal of Dermatology
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
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...
June 27, 2016: Journal of General Internal Medicine
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
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
Takeo Abe, Shinichiro Tsunoda, Aki Nishioka, Kouta Azuma, Kazuyuki Tsuboi, Chie Ogita, Yuichi Yokoyama, Tetsuya Furukawa, Momo Maruoka, Masao Tamura, Takahiro Yoshikawa, Atsushi Saito, Masahiro Sekiguchi, Naoto Azuma, Masayasu Kitano, Kiyoshi Matsui, Yuji Hosono, Ran Nakashima, Koichiro Ohmura, Tsuneyo Mimori, Hajime Sano
  Anti-aminoacyl-tRNA synthetase (ARS) antibody is one of the myositis-specific autoantibodies to make a diagnosis of polymyositis (PM) and dermatomyositis (DM). Recently a new enzyme-linked immunosorbent assay (ELISA) kit of concurrently detected anti-ARS antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-KS) have become to measure in the clinical setting. To evaluate the reliability of this ELISA kit, we measured anti-ARS antibodies in 75 PM and DM patients using by this ELISA assay and compared them with the results by RNA immunoprecipitation assay...
2016: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Li Zhang, Guoqin Wu, Di Gao, Guijian Liu, Lin Pan, Liyan Ni, Zheng Li, Qiang Wang
OBJECTIVES: Interstitial lung disease (ILD) is an extramuscular manifestation that results in increased morbidity and mortality from polymyositis (PM) and dermatomyositis (DM). The aim of this study was to systematically evaluate risk factors associated with the development of ILD in PM/DM. METHODS: Observational studies were identified from searching PubMed, Medline, Embase, and the Cochrane Library. Pooled odds ratios (ORs) or standardized mean differences (SMDs) and corresponding 95% confidence intervals (CIs) were obtained for the relationships between risk factors and ILD in PM/DM using either fixed- or random-effects models, whichever were appropriate...
2016: PloS One
Shinji Sato, Akihiro Murakami, Akiko Kuwajima, Kazuhiko Takehara, Tsuneyo Mimori, Atsushi Kawakami, Michiaki Mishima, Takafumi Suda, Mariko Seishima, Manabu Fujimoto, Masataka Kuwana
OBJECTIVE: Autoantibodies to melanoma differentiation-associated gene 5 (MDA5) are specifically expressed in patients with dermatomyositis (DM) and are associated with a subset of DM patients with rapidly progressive interstitial lung disease (RP-ILD). Here, we examined the clinical utility of a newly developed enzyme-linked immunosorbent assay (ELISA) system for detecting these antibodies. METHODS: Here we developed an improved ELISA for detecting anti-MDA5 antibodies...
2016: PloS One
Hajime Yoshifuji
Various autoantibodies are seen in idiopathic inflammatory myopathies. Among myositis-specific antibodies, anti-aminoacyl-tRNA synthetase and anti-melanoma differentiation-associated protein 5 (MDA5) antibodies are associated with interstitial lung disease (ILD). Anti-MDA5 antibodies are associated with dermatomyositis (DM) or clinically amyopathic DM complicated with rapidly progressive ILD. In anti-MDA5-positive patients, a random ground-glass attenuation pattern is a characteristic finding of ILD in chest high-resolution computed tomography...
2015: Clinical Medicine Insights. Circulatory, Respiratory and Pulmonary Medicine
Claire Theillac, Nicolas Poulalhon, Sébastien Debarbieux, Luc Thomas
No abstract text is available yet for this article.
June 1, 2016: European Journal of Dermatology: EJD
L G Rider, K Nistala
The aim of this review was to summarize recent advances in the understanding of the clinical and autoantibody phenotypes, their associated outcomes and the pathogenesis of the juvenile idiopathic inflammatory myopathies (JIIMs). The major clinical and autoantibody phenotypes in children have many features similar to those in adults, and each has distinct demographic and clinical features and associated outcomes. The most common myositis autoantibodies in JIIM patients are anti-p155/140, anti-MJ and anti-MDA5...
July 2016: Journal of Internal Medicine
Hidenaga Kawasumi, Takahisa Gono, Yasushi Kawaguchi, Masataka Kuwana, Hirotaka Kaneko, Yasuhiro Katsumata, Sayuri Kataoka, Masanori Hanaoka, Hisashi Yamanaka
OBJECTIVE: The physical function of PM/DM patients after remission induction therapy remains unknown adequately. The aim of our study was to evaluate the present status of physical dysfunction and to clarify the clinical manifestations and myositis-specific autoantibodies (MSAs) associated with physical dysfunction after treatment in PM/DM. METHODS: We obtained clinical data including the age at disease onset, gender, disease duration, laboratory data prior to initial treatment, and the specific treatment administered...
2016: BioMed Research International
Ilaria Cavazzana, Micaela Fredi, Angela Ceribelli, Cristina Mordenti, Fabio Ferrari, Nice Carabellese, Angela Tincani, Minoru Satoh, Franco Franceschini
OBJECTIVE: To analyze the performance of a line blot assay for the identification of autoantibodies in sera of patients affected by myositis, compared with immunoprecipitation (IP) as gold standard. METHODS: 66 sera of patients with myositis (23 polymyositis, 8 anti-synthetase syndromes, 29 dermatomyositis and 6 overlap syndromes) were tested by commercial LB (Euroimmun, Lubeck, Germany); 57 sera were analyzed also by IP of K562 cell extract radiolabeled with (35)S-methionine...
June 2016: Journal of Immunological Methods
Yves Allenbach, Gaëlle Leroux, Xavier Suárez-Calvet, Corinna Preusse, Eduard Gallardo, Baptiste Hervier, Aude Rigolet, Miguel Hie, Debora Pehl, Nicolas Limal, Peter Hufnagl, Norman Zerbe, Alain Meyer, Jessie Aouizerate, Yurdagul Uzunhan, Thierry Maisonobe, Hans-Hilmar Goebel, Olivier Benveniste, Werner Stenzel
The anti-melanoma differentiation-associated gene 5 (MDA5) autoantibody is specifically associated with dermatomyositis (DM). Nevertheless, anti-MDA5(+)-patients experience characteristic symptoms distinct from classic DM, including severe signs of extramuscular involvement; however, the clinical signs of myopathy are mild or even absent. The morphological and immunological features are not yet described in adulthood. Data concerning the pathophysiology of anti-MDA5 DM are sparse; however, the importance of the interferon (IFN) type I pathway involved in DM has been shown...
March 2016: American Journal of Pathology
Y Xu, C S Yang, Y J Li, X D Liu, J N Wang, Q Zhao, W G Xiao, P T Yang
Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD...
January 2016: Clinical Rheumatology
Z Betteridge, N McHugh
The idiopathic inflammatory myopathies are characterized by muscle weakness, skin disease and internal organ involvement. Autoimmunity is known to have a role in myositis pathogenesis, and myositis-specific autoantibodies, targeting important intracellular proteins, are regarded as key biomarkers aiding in the diagnosis of patients. In recent years, a number of novel myositis autoantibodies including anti-TIF1, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR and anti-cN1A have been identified in both adult and juvenile patients...
July 2016: Journal of Internal Medicine
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