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amyopathic dermatomyositis

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...
December 2, 2016: Journal of Autoimmunity
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
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
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
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...
September 23, 2016: Journal of Dermatological Science
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
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
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
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
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
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
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
Naomi Yoshida, Shinjiro Kaieda, Kumi Tomozoe, Morihiro Tajiri, Daisuke Wakasugi, Masaki Okamoto, Masaki Tominaga, Hiroaki Ida, Tomoaki Hoshino
A 62-year-old man presented with heliotrope rash, Gottron's sign, and mild muscle weakness. Both of his lung fields showed interstitial changes that worsened rapidly. He was diagnosed with clinical amyopathic dermatomyositis with rapidly progressive interstitial lung disease. The patient died of respiratory failure, despite the administration of immunosuppressive therapy. Autopsy revealed diffuse alveolar damage. An antibody analysis, which was performed postmortem, detected the presence of anti-melanoma differentiation-associated gene (MDA)-5 antibodies...
2016: Internal Medicine
Eun Jin Doh, Jungyoon Moon, Sue Shin, Soo Hyun Seo, Hyun Sun Park, Hyun-Sun Yoon, Soyun Cho
Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin lesions which were erythematous scaly patches on the knuckles and elbows, since her childhood. When skin biopsy was performed from a left knee nodule, liquid chalky discharge was observed...
June 2016: Annals of Dermatology
Jeffrey D McBride, Richard D Sontheimer
IMPORTANCE: Many patients present with cutaneous signs and symptoms that suggest a diagnosis on the autoimmune disease spectrum. During the "acute phase" of disease activity, patients with systemic sclerosis (SSc) and dermatomyositis (DM) have characteristic nailfold findings, including dilated capillaries, microhemorrhages, and hemosiderin deposits. OBJECTIVE: To review the literature on the presentation of microhemorrhages and to highlight the differences (in terms of terminology, characterization, and clinical relevance) between proximal microhemorrhage events and the distal products, often thought of as "hemosiderin deposits" located in the cuticle (eponychium)...
2016: Dermatology Online Journal
Jeremy Udkoff, Philip R Cohen
Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron's sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. A literature review of amyopathic dermatomyositis was performed using the PubMed medical database...
October 2016: American Journal of Clinical Dermatology
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
J Callander, Y Robson, J Ingram, V Piguet
Clinically amyopathic dermatomyositis (CADM) affects a subset of 5-20% of patients with dermatomyositis and is defined as the presence of cutaneous features of dermatomyositis without clinical muscle weakness for ≥ 6 months. There is no consensus on first-line treatment for CADM and whether treatment should differ from treatment of classic dermatomyositis with muscle weakness. We carried out a systematic review of published literature about treatment of adult patients with CADM, via the Embase, Medline, CINAHL and ClinicalTrials...
May 11, 2016: British Journal of Dermatology
Cheilonda Johnson, Iago Pinal-Fernandez, Radhika Parikh, Julie Paik, Jemima Albayda, Andrew L Mammen, Lisa Christopher-Stine, Sonye Danoff
PURPOSE: Among patients with autoimmune myositis, associated interstitial lung disease (MA-ILD) is a known contributor of excess morbidity and mortality. Recent data on survival in idiopathic inflammatory myopathies originate primarily in Asia and Europe and vary widely. We sought to examine mortality in a large U.S. myositis cohort focusing in particular on the impact of associated ILD. METHODS: A cross-sectional analysis of participants from the Johns Hopkins Myositis Center with autoimmune myositis (polymyositis [PM], dermatomyositis [DM], or clinically amyopathic dermatomyositis [CADM]) was conducted...
October 2016: Lung
Lúcia Maria Arruda Campos, Adriana M E Sallum, Cintia Z Camargo, Luís Eduardo C Andrade, Cristiane Kayser
Clinically amyopathic dermatomyositis (CADM) is an extremely rare entity in childhood and only 75 cases have been described in the literature. These patients present mild or no muscle involvement, while skin involvement is similar to that seen in classic dermatomyositis (DM). Systemic inflammatory vasculopathy is a hallmark of juvenile dermatomyositis (JDM), especially affecting the microcirculation. Nailfold capillaroscopy (NFC) is a non-invasive method that allows direct visualization of nailfold capillary loops around the proximal edge of the finger nails...
April 18, 2016: Revista Brasileira de Reumatologia
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