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MDA5 antibody Interstitial lung disease

Wenhan Huang, Feifeng Ren, Qian Wang, Lei Luo, Jun Zhou, Dongmei Huang, Zhuma Pan, Lin Tang
OBJECTIVES: To investigate the clinical characteristics of patients positive for anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, and to analyse the potential pathogenesis of anti-MDA5 antibodies. METHODS: The clinical manifestations, serological tests, imaging features, treatments, and prognoses of 32 anti-MDA5 antibody-positive patients diagnosed in the Rheumatology and Immunology Department of the Second Affiliated Hospital of Chongqing Medical University from September 2015 to August 2018 were analysed...
February 11, 2019: Clinical and Experimental Rheumatology
Lili Jiang, Youlian Wang, Qinglin Peng, Xiaoming Shu, Guochun Wang, Xiaomu Wu
OBJECTIVE: We aimed to investigate the clinical value of checking serum chitinase-3-like-1 protein (YKL-40) levels in anti-MDA5 antibody-positive dermatomyositis (anti-MDA5+ DM) patients. METHODS: One hundred and five consecutive anti-MDA5+ DM patients and 44 healthy controls were enrolled in this study. Baseline and follow-up serum YKL-40 were detected by ELISA. We evaluated the association of YKL-40 with rapidly progressive interstitial lung disease (RPILD), severity of interstitial lung disease (ILD), and ILD-related survival...
February 9, 2019: Clinical Rheumatology
Junichi Aoyama, Hiroki Hayashi, Chika Yajima, Hiroyuki Takoi, Toru Tanaka, Takeru Kashiwada, Nariaki Kokuho, Yasuhiro Terasaki, Ayumi Nishikawa, Takahisa Gono, Masataka Kuwana, Yoshinobu Saito, Shinji Abe, Masahiro Seike, Akihiko Gemma
A 47-year-old man was referred to our hospital with a 1-month history of fever and dyspnea after inhalation of insecticide in a confined space. We diagnosed rapidly progressive interstitial pneumonia. High-dose methylprednisolone, tacrolimus, and intermittent infusion of cyclophosphamide were administered. His condition rapidly deteriorated; therefore, extracorporeal membrane oxygenation therapy was performed. Unfortunately, he died 69 days after admission. Although typical skin findings suggestive of dermatomyositis were absent, anti-melanoma differentiation-associate gene (anti-MDA5) antibody was positive...
2019: Respiratory Medicine Case Reports
Manami Kato, Kei Ikeda, Takahiro Kageyama, Tadamichi Kasuya, Takashi Kumagai, Hiroki Furuya, Shunsuke Furuta, Tomohiro Tamachi, Akira Suto, Kotaro Suzuki, Hiroshi Nakajima
No abstract text is available yet for this article.
January 4, 2019: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Nobuyuki Ono, Keita Kai, Akihito Maruyama, Mariko Sakai, Yuri Sadanaga, Shuichi Koarada, Takuya Inoue, Yoshifumi Tada
Objectives: Based on the antibody profiles of inflammatory myositis patients, we investigated the type 1 IFN (T1-IFN) signature in serum and DM skin to determine the relationship between T1-IFN and vasculopathy in anti-melanoma differentiation-associated 5 gene (MDA5) antibody-positive DM patients. Methods: We examined 47 patients with new-onset inflammatory myositis. We divided them into three groups: the anti-MDA5 antibody-positive patients (MDA5 group, n = 16), the anti-aminoacyl-tRNA synthetase antibody-positive patients (aminoacyl-tRNA synthetase group, n = 12), and the double-negative patients (n = 19)...
December 11, 2018: Rheumatology
Bradley J Peters, Mikaela Hofer, Craig E Daniels, Jeffrey L Winters
PURPOSE: The effect of therapeutic plasma exchange (TPE) on antifactor Xa activity in a patient treated with enoxaparin and levetiracetam is reported. SUMMARY: A 52-year-old woman was treated with levetiracetam and prophylactic enoxaparin while receiving TPE to manage respiratory failure due to anti-MDA5 antibody-associated interstitial lung disease (ILD) with dermatomyositis. Due to a scant amount of evidence regarding the management of these medications in TPE, therapeutic monitoring principles were used to assess the effect TPE had on these medications...
December 1, 2018: American Journal of Health-system Pharmacy: AJHP
Li Huang, Li Wang, Yang Yang, Huan Chen, Yanjuan Liu, Ke Liu, Meidong Liu, Yizhi Xiao, Xiaoxia Zuo, Yisha Li, Hui Luo, Huali Zhang, Xianzhong Xiao
Myositis-specific autoantibodies are important diagnostic and prognostic markers. The aim of our study is to detect anti-3-hydroxy 3-methylutaryl coenzyme A reductase (anti-HMGCR) antibody using novel unlabeled immunoprecipitation (IP) assay and immunoblotting in Chinese patients with myositis and to clarify the features of anti-HMGCR-positive patients. In the present study, we established novel unlabeled IP assay and immunoblotting of HMGCR C-terminus for anti-HMGCR detection. The presence of anti-HMGCR was screened in 181 Chinese patients with myositis...
November 2018: Medicine (Baltimore)
Noriho Sakamoto, Hiroshi Ishimoto, Shota Nakashima, Hirokazu Yura, Takuto Miyamura, Daisuke Okuno, Atsuko Hara, Takeshi Kitazaki, Tomoyuki Kakugawa, Yuji Ishimatsu, Minoru Satoh, Hiroshi Mukae
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody is associated with rapidly progressive interstitial lung disease (RP-ILD) in patients with clinically amyopathic dermatomyositis (CADM) or dermatomyositis (DM). We herein report three Japanese cases of anti-MDA5 antibody-positive RP-ILD without signs of CADM or DM. High-resolution computed tomography revealed patchy or subpleural distribution of consolidations and/or ground-glass opacities accompanied by traction bronchiectasis. All patients succumbed to respiratory failure within two months...
November 19, 2018: Internal Medicine
Aki Nishioka, Shinichiro Tsunoda, Takeo Abe, Takahiro Yoshikawa, Miki Takata, Masayasu Kitano, Kiyoshi Matsui, Ran Nakashima, Yuji Hosono, Koichiro Ohmura, Tsuneyo Mimori, Hajime Sano
OBJECTIVE: This study identified biomarkers that can be used to assess disease activity and response to therapy in patients with interstitial lung disease complicating anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive clinically amyopathic dermatomyositis (CADM). METHODS: In 15 patients with interstitial lung disease complicating anti-MDA5 Ab-positive CADM, anti-MDA5 Ab, neopterin, interleukin (IL)-18, ferritin, and soluble interleukin 2 receptor (sIL-2R) levels were measured in cryopreserved serum specimens before and at multiple times after remission induction therapy, and their correlations were assessed...
November 19, 2018: Modern Rheumatology
Siamak Moghadam-Kia, Chester V Oddis, Rohit Aggarwal
PURPOSE OF REVIEW: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) is a novel and highly specific myositis-associated autoantibody, which defines a unique phenotype among patients with dermatomyositis (DM). RECENT FINDINGS: Anti-MDA5 was originally characterized in Japan in DM patients with hallmark cutaneous features and no proximal muscle weakness and termed clinically amyopathic DM (CADM). Anti-MDA5 has characteristic cutaneous manifestations which include tender palmar papules and cutaneous ulcerations, along with an increased frequency of interstitial lung disease (ILD) that can be rapidly progressive (RPILD) and fatal...
October 31, 2018: Current Rheumatology Reports
Haruki Matsumoto, Shuzo Sato, Yuya Fujita, Makiko Yashiro-Furuya, Naoki Matsuoka, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Kiyoshi Migita
Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are frequently detected in amyopathic dermatomyositis with rapidly progressive interstitial lung disease (RP-ILD). However, the presence of anti-MDA5 antibodies in other connective tissue diseases is not well known. We herein report a case of rheumatoid arthritis complicated with refractory anti-MDA5 antibody-positive ILD. A 75-year-old Japanese woman was referred to our hospital for refractory ILD. Serological testing was positive for anti-MDA5 antibody without any muscle or skin lesions...
October 17, 2018: Internal Medicine
María Victoria Collado, María De Los Ángeles Gargiulo, Ramiro Gómez, Graciela Gómez, Nicolás Pérez, Lorena Suarez, Ana Lía Taratuto, Patricia Aruj
Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment...
2018: Medicina
Makiko Yashiro, Tomoyuki Asano, Shuzo Sato, Hiroko Kobayashi, Hiroshi Watanabe, Masayuki Miyata, Kiyoshi Migita
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) resulting in high mortality. Here we report a 51-year-old Japanese woman with anti-MDA5 antibody-positive hypomyopathic dermatomyositis (DM) who developed RP-ILD. She developed respiratory failure and pneumomediastinum, however her RP-ILD responded favorably to the combined immunosuppressive treatments consisting of steroids, intravenous cyclophosphamide and tacrolimus...
2018: Fukushima Journal of Medical Science
Satoshi Sakamoto, Masaki Okamoto, Shinjiro Kaieda, Kiminori Fujimoto, Shuji Nagata, Masaki Tominaga, Masayuki Nakamura, Yoshiaki Zaizen, Takashi Nouno, Takuma Koga, Tomotaka Kawayama, Masataka Kuwana, Hiroaki Ida, Tomoaki Hoshino
BACKGROUND: Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab) is associated with fatal rapidly progressive interstitial lung disease (RP-ILD) in patients with dermatomyositis (DM). We attempted to clarify whether anti-MDA5-Ab is associated with long-term outcomes in patients with DM-ILD. METHODS: Thirty-six patients with DM-ILD were retrospectively analyzed for their serum anti-MDA5-Ab by using an enzyme-linked immunosorbent assay. We analyzed the association between clinical parameters, including the serum levels of anti-MDA5-Ab and ferritin...
November 2018: Respiratory Investigation
M M Xie, X Yan, H Li, J J Ding, X H Gui, Y Liu, Y S Wang, Z Y Chen, H R Cai
Objective: To investigate the profile and clinical significance of myositis-specific antibody spectrum (MSAs) in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods: Sera from 74 patients with PM/DM-ILD, 29 patients with SLE and 32 healthy controls were collected and Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs . The clinical data of all patients were collected from medical records. Statistical analysis was performed using One-way ANOVA, t -test, rank sum test, χ(2) test or Fisher's exact test...
August 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
M Chen, C Quan, L Diao, F Xue, K Xue, B Wang, X Li, X Zhu, J Zheng, H Cao
BACKGROUND: Dermatomyositis (DM) is an autoimmune disease affecting primarily the skin, muscle and lung. Dysregulations of cytokines and chemokines are commonly found in inflammatory disorders. OBJECTIVES: To investigate the association between serum cytokines and chemokines and clinical severity, especially cutaneous lesions and interstitial lung disease (ILD) in patients with DM and clinically amyopathic DM (CADM). METHODS: Clinical features, laboratory findings and serum of 40 patients with DM or CADM were collected and analysed...
August 12, 2018: British Journal of Dermatology
Kazuhiro Kurasawa, Satoko Arai, Yumeko Namiki, Ayae Tanaka, Yuta Takamura, Takayoshi Owada, Masafumi Arima, Reika Maezawa
Objective: We aimed to determine the outcome of combination therapy with tofacitinib (TOF) in a case series of refractory rapidly progressive interstitial lung disease (ILD) associated with anti-melanoma differentiation-associated 5 gene (MDA5) antibody-positive (Ab+) DM. Patients who had poor prognostic factors and failed to respond to immunosuppressive therapy were selected for TOF treatment. Methods: Five patients with anti-MDA5 Ab+ DM-ILD who failed to respond to triple therapy with high dose glucocorticoids, CSA and CYC were given additional TOF (10 mg/day)...
December 1, 2018: Rheumatology
Yuya Fujita, Shoichi Fukui, Takahisa Suzuki, Midori Ishida, Yushiro Endo, Sousuke Tsuji, Ayuko Takatani, Takashi Igawa, Toshimasa Shimizu, Masataka Umeda, Remi Sumiyoshi, Ayako Nishino, Tomohiro Koga, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Kuniko Abe, Atsushi Kawakami
A 56-year-old Japanese woman with muscle weakness, increased creatine kinase and aldolase levels, and characteristic cutaneous lesions was diagnosed with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis. She also had interstitial lung disease (ILD). After corticosteroid and tacrolimus combination therapy was started, pancytopenia and elevated serum ferritin and transaminase emerged. Because the bone marrow tissues were hypoplastic with hemophagocytes, she was diagnosed with concomitant autoimmune-associated hemophagocytic syndrome (HPS)...
July 6, 2018: Internal Medicine
Koichi Yamaguchi, Aya Yamaguchi, Chiharu Kashiwagi, Yuri Sawada, Kohei Taguchi, Kazue Umetsu, Kazuma Oshima, Megumi Uchida, Masafumi Suzuki, Shunichi Kono, Masao Takemura, Hiroaki Masubuchi, Shinsuke Kitahara, Kenichiro Hara, Toshitaka Maeno, Sei-Ichiro Motegi, Yoshinao Muro, Toru Sakairi, Takeshi Hisada, Masahiko Kurabayashi
BACKGROUND: Anti-melanoma differentiation-associated gene 5 (MDA5) autoantibodies have been identified as myositis-specific autoantibodies that are often associated with clinically amyopathic dermatomyositis (CADM) and a poor prognosis due to rapidly progressive interstitial lung disease (RP-ILD) in East Asian patients. Besides anti-MDA5 autoantibodies, patients with CADM may have myositis-associated autoantibodies (MAAs), which characterize other connective tissue diseases such as rheumatoid arthritis and Sjögren's syndrome...
July 2018: Respiratory Medicine
Yushiro Endo, Tomohiro Koga, Midori Ishida, Yuya Fujita, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Remi Sumiyoshi, Takashi Igawa, Masataka Umeda, Shoichi Fukui, Ayako Nishino, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Masataka Kuwana, Yuji Hosono, Tsuneyo Mimori, Atsushi Kawakami
RATIONALE: Among all dermatomyositis (DM) patients, antimelanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) positive patients have significantly poor short-term mortality, whereas they experience less relapses over the long term after the remission. We report the case of a patient with anti-MDA5 Ab-positive clinically amyopathic dermatomyositis (CADM) with the recurrence of interstitial lung disease (ILD) after 7 years of remission. There has been no case report of an anti-MDA5 Ab-positive DM patient with the recurrence of ILD after 7 years of long-term remission...
June 2018: Medicine (Baltimore)
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