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https://www.readbyqxmd.com/read/29301801/clinically-amyopathic-dermatomyositis-associated-with-anti-mda5-antibody
#1
Konstantinos Parperis, Amirali Kiyani
Clinically amyopathic dermatomyositis (CADM) is a rare entity that presents with cutaneous manifestations of classic dermatomyositis but without muscle weakness or abnormal muscle enzymes. It is more common in young white and Asian females. A subset of patients with CADM has a specific antibody known as anti-MDA5. These patients have a more aggressive course with distinct cutaneous features, pulmonary involvement and early death. Here, we present the case of a 64-year-old Caucasian male with no significant medical history who was admitted with marked weight loss and a painful rash for 6 months...
January 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29289262/clinical-heterogeneity-of-interstitial-lung-disease-in-polymyositis-and-dermatomyositis-patients-with-or-without-specific-autoantibodies
#2
Fang Chen, Shanshan Li, Tao Wang, Jingli Shi, Guochun Wang
BACKGROUND: The aim of this study was to compare the heterogeneity of interstitial lung disease (ILD) in patients with polymyositis and dermatomyositis (PM/DM) according to serological type. METHODS: A total of 182 patients with PM/DM-ILD were observed retrospectively. Antiaminoacyl-tRNA synthetase (ARS) and antimelanoma differentiation-associated gene5 (MDA5) antibodies were screened using immunoblotting approach. The patients with ILD were divided into 3 groups: MDA5 (with anti-MDA5 antibody), ARS (with anti-ARS antibody) and MSN (without anti-MDA5 or anti-ARS antibody) group...
January 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29287518/anti-mda5-positive-dermatomyositis-complicated-with-rapidly-progressive-interstitial-lung-disease-a-case-report
#3
Eva De Backer, Félix Gremonprez, Guy Brusselle, Pieter Depuydt, Jo Van Dorpe, Carole Van Haverbeke, Pieter C Goeminne, Eric Derom
CASE PRESENTATION:  We present a case of a 55-year-old Caucasian male with manifestations of dermatomyositis complicated with rapidly progressive interstitial lung disease (RP-ILD). Diagnosis of anti-MDA5 positive dermatomyositis was made. DISCUSSION:  Myositis specific antibodies (MSA) can be used for diagnosis and predicting prognosis in patients with polymyositis and dermatomyositis. Anti-MDA5 positive dermatomyositis should be considered in patients presenting with dermatomyositis and a disease course resembling antisynthetase syndrome in the absence of antisynthetase autoantibodies, especially if a remarkably high ferritin is noted...
December 29, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29225988/effective-administration-of-rituximab-in-anti-mda5-antibody-positive-dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-and-refractory-cutaneous-involvement-a-case-report-and-literature-review
#4
Yuka Ogawa, Dai Kishida, Yasuhiro Shimojima, Koichi Hayashi, Yoshiki Sekijima
We describe the case of a 48-year-old man with dermatomyositis (DM) who demonstrated rapidly progressive interstitial lung disease (RP-ILD) and refractory cutaneous involvement together with high levels of anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab). Even after combination immunosuppressive therapy including a corticosteroid, cyclosporine A, and intravenous cyclophosphamide, his respiratory insufficiency and cutaneous involvement progressively worsened. However, the administration of rituximab (RTX) resulted in clinical remission as well as a visible reduction in anti-MDA5-Ab levels, suggesting that RTX could be a useful remedy in cases refractory to conventional immunosuppressive agents, especially those of RP-ILD related to anti-MDA5-Ab-positive DM...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29186193/rig-i-like-receptor-activation-by-dengue-virus-drives-follicular-t-helper-cell-formation-and-antibody-production
#5
Joris K Sprokholt, Tanja M Kaptein, John L van Hamme, Ronald J Overmars, Sonja I Gringhuis, Teunis B H Geijtenbeek
Follicular T helper cells (TFH) are fundamental in orchestrating effective antibody-mediated responses critical for immunity against viral infections and effective vaccines. However, it is unclear how virus infection leads to TFH induction. We here show that dengue virus (DENV) infection of human dendritic cells (DCs) drives TFH formation via crosstalk of RIG-I-like receptor (RLR) RIG-I and MDA5 with type I Interferon (IFN) signaling. DENV infection leads to RLR-dependent IKKε activation, which phosphorylates IFNα/β receptor-induced STAT1 to drive IL-27 production via the transcriptional complex ISGF3...
November 29, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/29106035/genetic-association-of-hla-drb1-multiple-polymorphisms-with-dermatomyositis-in-chinese-population
#6
Lin Jinming, Zhang Yongbiao, Qinglin Peng, Hanbo Yang, Jingli Shi, Mingliang Gu, Wenming Zhao, Guochun Wang
Genetic variation in human leukocyte antigen (HLA) plays an important role in the pathogenesis of dermatomyositis (DM). The aim of this study was to investigate the association of HLA class II with DM in China. 224 DM patients and 300 healthy controls were randomly enrolled at China-Japan Friendship Hospital. High resolution typing of HLA-DRB1 alleles was performed by sequencing based typing (SBT). The HLA-DQA1 and HLA-DQB1 alleles were determined by PCR sequence-specific primers (SSP). The frequencies of HLA-DRB1*09:01 (28...
November 6, 2017: HLA
https://www.readbyqxmd.com/read/29100298/assessment-of-anti-mda5-antibody-as-a-diagnostic-biomarker-in-patients-with-dermatomyositis-associated-interstitial-lung-disease-or-rapidly-progressive-interstitial-lung-disease
#7
Liubing Li, Qian Wang, Xiaoting Wen, Chenxi Liu, Chanyuan Wu, Funing Yang, Xiaofeng Zeng, Yongzhe Li
Anti-melanoma differentiation-associated protein 5 (MDA5) antibody have been found in dermatomyositis (DM)-associated interstitial lung disease (DM-ILD) and DM-associated rapidly progressive ILD (DM-RPILD). Due to the conflicting results regarding the association between anti-MDA5 antibody and DM-ILD or DM-RPILD and the diagnostic value of this antibody for DM-ILD and DM-RPILD, we performed this meta-analysis. A systematic search was performed to identify studies published to January 14, 2017. Sixteen publications with 491 DM with ILD versus 605 DM without ILD, as well as eighteen publications with 186 DM with RPILD and 790 DM without RPILD were included...
September 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/29093381/the-serum-ferritin-level-is-associated-with-the-treatment-responsivity-for-rapidly-progressive-interstitial-lung-disease-with-amyopathic-dermatomyositis-irrespective-of-the-anti-mda5-antibody-level-a-case-report
#8
Takeshi Osawa, Kozo Morimoto, Yuka Sasaki, Shuichi Matsuda, Kazunari Yamana, Ryozo Yano, Takashi Uchiyama, Hajime Goto
We herein report he case of a 61-year-old woman with rapidly progressive interstitial lung disease caused by clinically amyopathic dermatomyositis. Both the serum ferritin and anti-MDA5 antibody levels were elevated at the time of admission. Despite intensive treatment with corticosteroids, immunosuppressants, immunoglobulins and polymyxin B direct hemoperfusion, the patient died 75 days after symptom onset. Over the course of treatment, the anti-MDA5 antibody level continually decreased, while the serum ferritin level increased, suggesting that sequential measurements of the serum ferritin level might be useful for evaluating the treatment responsivity, irrespective of the anti-MDA5 antibody level...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29065773/describing-and-expanding-the-clinical-phenotype-of-anti-mda5-associated-rapidly-progressive-interstitial-lung-disease-case-series-of-nine-canadian-patients-and-literature-review
#9
S Hoa, Y Troyanov, M J Fritzler, I N Targoff, S Chartrand, A M Mansour, E Rich, H Boudabbouz, J Bourré-Tessier, M Albert, J R Goulet, M Landry, J L Senécal
OBJECTIVE: To describe and expand the phenotype of anti-MDA5-associated rapidly progressive interstitial lung disease (MDA5-RPILD) in Canadian patients. METHOD: All proven cases of MDA5-RPILD hospitalized in the University of Montreal's affiliated centres from 2004 to 2015 were selected for inclusion. RESULTS: Of nine consecutive patients, RPILD was the presenting manifestation in seven, whereas two patients developed RPILD 2 years after the onset of arthritis and of chronic interstitial lung disease...
October 25, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28983955/associations-between-anti-melanoma-differentiation-associated-gene-5-antibody-and-demographics-clinical-characteristics-and-laboratory-results-of-patients-with-dermatomyositis-a-systematic-meta-analysis
#10
Ju Li, Yan Liu, Yongsheng Li, Fang Li, Kai Wang, Wenyou Pan, Deqian Meng
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody is a specific biomarker in patients with dermatomyositis (DM). Results from several studies that examined the relationship between anti-MDA5 antibody and the demographics, clinical characteristics and laboratory results of DM patients have been conflicting. The purpose of this study was to identify the relationship, if any, of anti-MDA5 antibody with demographics, clinical characteristics and laboratory results of DM patients. PubMed, Web of Science, Embase and the Cochrane Library databases were searched for studies without language restrictions conducted before 16 March 2017...
October 6, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28722667/assessment-of-anti-mda5-antibody-as-a-diagnostic-biomarker-in-patients-with-dermatomyositis-associated-interstitial-lung-disease-or-rapidly-progressive-interstitial-lung-disease
#11
Liubing Li, Qian Wang, Xiaoting Wen, Chenxi Liu, Chanyuan Wu, Funing Yang, Xiaofeng Zeng, Yongzhe Li
Anti-melanoma differentiation-associated protein 5 (MDA5) antibody have been found in dermatomyositis (DM)-associated interstitial lung disease (DM-ILD) and DM-associated rapidly progressive ILD (DM-RPILD). Due to the conflicting results regarding the association between anti-MDA5 antibody and DM-ILD or DM-RPILD and the diagnostic value of this antibody for DM-ILD and DM-RPILD, we performed this meta-analysis. A systematic search was performed to identify studies published to January 14, 2017. Sixteen publications with 491 DM with ILD versus 605 DM without ILD, as well as eighteen publications with 186 DM with RPILD and 790 DM without RPILD were included...
July 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28711882/hla-drb1-alleles-as-genetic-risk-factors-for-the-development-of-anti-mda5-antibodies-in-patients-with-dermatomyositis
#12
Zhiyong Chen, Yan Wang, Masataka Kuwana, Xue Xu, Wei Hu, Xuebing Feng, Hong Wang, Akinori Kimura, Lingyun Sun
OBJECTIVE: Patients with polymyositis/dermatomyositis (PM/DM) who express anti-melanoma differentiation associated protein 5 (anti-MDA5) antibodies frequently present with interstitial lung disease (ILD). The aim of this study was to investigate the association of HLA-DRB1 with anti-MDA5 expression in PM/DM. METHODS: The frequency of DRB1 alleles was compared among 70 patients with PM, 104 patients with DM, and 400 healthy controls in a Han Chinese population. RESULTS: Frequencies of DRB1*04:01 [17...
July 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28668260/-amyopathic-dermatomyositis-dm-with-anti-mda5-antibodies-associated-with-bullous-pemphigoid-sj%C3%A3-gren-syndrome-and-gastric-malt-lymphoma
#13
B Garcia, F Dabouz, L Pascal, M Gillard, P Modiano
BACKGROUND: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement. PATIENTS AND METHODS: A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption...
October 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28580698/spontaneous-pneumomediastinum-in-a-dermatomyositis-patient-with-anti-melanoma-differentiation-associated-gene-5-antibody-and-interstitial-lung-disease-despite-an-initial-response-to-immunosuppressant
#14
Chiu Wai Shirley Chan, Ho Yin Chung, Chak Sing Lau, Helen H L Tsang
We report a 24-year-old man with anti-melanoma differentiation-associated gene-5 (MDA5) antibody-positive dermatomyositis (DM) and interstitial lung disease (ILD) who developed spontaneous pneumomediastinum. By comparing serial thoracic high-resolution computed tomography scans, we demonstrated the distinct time course showing a paradoxical occurrence of pneumomediastinum despite a radiological improvement of ILD. Our case shows that pneumomediastinum in DM can occur regardless of associated ILD and it is a serious complication that should be considered in DM patients presenting with pulmonary manifestations...
June 4, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28566605/dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-treated-with-rituximab-a-report-of-3-cases-in-japan
#15
Kenichiro Tokunaga, Noboru Hagino
We performed a retrospective chart review of three patients with hypomyopathic dermatomyositis and rapidly progressive interstitial lung disease. The patients were Japanese women of 71, 69, and 65 years of age. Two patients were anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and 1 was anti-aminoacyl-tRNA synthetase (anti-ARS) antibody-positive. Their respiratory statuses deteriorated despite the administration of glucocorticoid, calcineurin inhibitors, and intravenous cyclophosphamide therapy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28499006/clinical-characteristics-and-change-in-the-antibody-titres-of-patients-with-anti-mda5-antibody-positive-inflammatory-myositis
#16
Yoshiyuki Abe, Masakazu Matsushita, Kurisu Tada, Ken Yamaji, Yoshinari Takasaki, Naoto Tamura
Objective: The aim of this study was to evaluate the clinical characteristics of patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive inflammatory myositis, and the change in anti-MDA5 antibody titres before and after onset. Method: For 105 PM/DM patients, newly diagnosed in our hospital within the period 2008-2016, serum anti-MDA5 antibody levels were measured at diagnosis and after treatment by ELISA using the MESACUP anti-MDA5 test...
September 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28490218/evaluation-of-clinical-prognostic-factors-for-interstitial-pneumonia-in-anti-mda5-antibody-positive-dermatomyositis-patients
#17
Youhei Fujiki, Takuya Kotani, Kentaro Isoda, Takaaki Ishida, Takeshi Shoda, Shuzo Yoshida, Tohru Takeuchi, Shigeki Makino
OBJECTIVES: We retrospectively investigated clinical prognostic factors for interstitial pneumonia (IP) in anti-melanoma differentiation-associated gene 5 (MDA5) antibody (Ab)-positive dermatomyositis (DM) patients. METHODS: Subjects comprised 18 patients with anti-MDA5 Ab-positive DM-IP (9 survivors; 9 deaths). RESULTS: Initial serum albumin levels, ferritin levels, and ground-glass opacity (GGO) scores in the right middle lobes were significantly higher in the death group than in the survivor group (p = ...
May 11, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28480196/mda5-autoantibody-another-indicator-of-clinical-diversity-in-dermatomyositis
#18
REVIEW
Richard D Sontheimer
Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i...
April 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28460448/anti-mda5-antibody-as-a-potential-diagnostic-and-prognostic-biomarker-in-patients-with-dermatomyositis
#19
Liubing Li, Qian Wang, Funing Yang, Chanyuan Wu, Si Chen, Xiaoting Wen, Chenxi Liu, Yongzhe Li
The presence of anti-MDA5 antibodies in serum represents an important biomarker in the diagnosis and prediction of prognosis for patients with idiopathic inflammatory myopathies (IIMs). Due to conflicting results that have been reported regarding the detection of anti-MDA5 antibodies, the goal of this study was to assess a potential association between the presence of anti-MDA5 antibodies and dermatomyositis/polymyositis (DM/PM), as well as the diagnostic and prognostic values of anti-MDA5 antibodies for DM/PM...
April 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28451794/comparison-of-long-term-prognosis-and-relapse-of-dermatomyositis-complicated-with-interstitial-pneumonia-according-to-autoantibodies-anti-aminoacyl-trna-synthetase-antibodies-versus-anti-melanoma-differentiation-associated-gene-5-antibody
#20
Kentaro Isoda, Takuya Kotani, Tohru Takeuchi, Takao Kiboshi, Kenichiro Hata, Takaaki Ishida, Kenichiro Otani, Takao Kamimori, Hiroshi Fujiwara, Takeshi Shoda, Shigeki Makino
The aim of this study was to investigate long-term prognosis and relapse of dermatomyositis complicated with interstitial pneumonia (DMIP) according to anti-aminoacyl tRNA synthetase (ARS) antibodies and anti-melanoma differentiation-associated gene 5 (MDA5) antibody. This retrospective study comprised 36 patients with DMIP who were divided into the anti-ARS antibody-positive group (ARS+) (n = 12), anti MDA5 antibody-positive group (MDA5+) (n = 11), double-negative group (ARS-/MDA5-) (n = 11), and double-positive group (ARS+/MDA5+) (n = 1)...
August 2017: Rheumatology International
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