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amyopathic dermatomyositis

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https://www.readbyqxmd.com/read/29750110/non-specific-interstitial-pneumonia-associated-with-clinically-amyopathic-dermatomyositis-showing-crazy-paving-appearance-on-thin-section-lung-ct
#1
Yuya Aono, Tatsuru Eifuku, Tomohiro Uto, Jun Sato, Shiro Imokawa, Takafumi Suda
The "crazy paving" appearance consists of ground-glass opacity superimposed on a network of linear opacities on thin-section computed tomography (CT) images of the lung. This finding has been described in a variety of diseases but is extremely rare in patients with non-specific interstitial pneumonia (NSIP). We describe a 45-year-old woman with biopsy-proven NSIP associated with clinically amyopathic dermatomyositis that showed a "crazy paving" appearance on thin-section CT of the lung. Clinicians should include NSIP in the differential diagnosis in patients presenting with "crazy paving" appearance on thin-section chest CT...
July 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29737058/-risk-factors-of-respiratory-failure-in-the-dermatomyositis-patients-with-interstitial-lung-disease
#2
Su-Han Zhang, Yun Peng, Qi-Bing Xie, Geng Yin, Bing Yan
OBJECTIVE: To reveal the clinical features of respiratory failure (RF) in dermatomyositis (DM) patients with interstitial lung disease (ILD),and to explore risk factors of RF in these patients. METHODS: The medical data of 122 DM patients with ILD were retrospectively reviewed: 40 developed RF (RF group),82 did not develope RF (Non RF group). Clinical,laboratory and radiological variables were compared between RF patients and Non RF patients. Multivariate Logistic regression was used to analyze risk factors of RF...
March 2018: Sichuan da Xue Xue Bao. Yi Xue Ban, Journal of Sichuan University. Medical Science Edition
https://www.readbyqxmd.com/read/29713969/rituximab-for-refractory-rapidly-progressive-interstitial-lung-disease-related-to-anti-mda5-antibody-positive-amyopathic-dermatomyositis
#3
Ho So, Victor Tak Lung Wong, Virginia Weng Nga Lao, Hin Ting Pang, Ronald Man Lung Yip
To report our experience in using rituximab (RTX) for treating refractory rapidly progressive interstitial lung disease (RP-ILD) complicating anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab)-positive amyopathic dermatomyositis (ADM). Medical records of four ADM patients with refractory RP-ILD treated with RTX therapy were reviewed retrospectively. All four patients were tested positive for anti-MDA5 Ab and failed to respond to high-dose systemic steroid and other intensive immunosuppressive therapies...
April 30, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29707197/case-report-acute-amyopathic-dermatomyositis-presenting-with-isolated-facial-edema
#4
Efthymia Pappa, Marina Gkeka, Asimina Protogerou, Leonidas Marinos, Chariclia Loupa, Constantinos Christopoulos
A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation...
2018: F1000Research
https://www.readbyqxmd.com/read/29657141/association-of-serum-soluble-cd163-with-polymyositis-and-dermatomyositis-especially-in-anti-mda5-antibody-positive-cases
#5
Hidenaga Kawasumi, Yasuhiro Katsumata, Akira Nishino, Shinya Hirahara, Yasushi Kawaguchi, Masataka Kuwana, Hisashi Yamanaka
OBJECTIVE: We elucidated the association of serum soluble CD163 (sCD163) with rapidly progressive interstitial lung disease (RP-ILD), autoantibody profiles, and serum ferritin in patients with polymyositis (PM), classic dermatomyositis (DM), and clinical amyopathic dermatomyositis (CADM). METHODS: Serum sCD163 levels were retrospectively measured by ELISA in patients with PM, classic DM, and CADM, as well as in healthy controls (HC). Repeat sera samples were obtained posttreatment from available patients...
April 15, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29579373/calcinosis-of-the-mandible-in-dermatomyositis
#6
Andrea Fava, Lisa Christopher-Stine
A 52-year-old woman presented to the Rheumatology clinic complaining of new tender nodules along the inferior border of her jaw. She has an 8-year history of amyopathic dermatomyositis with anti-MDA5 antibodies complicated by interstitial lung disease. Her current treatments include tacrolimus, prednisone and hydroxychloroquine. She noticed the nodules during the most recent flare of her skin disease one month prior. On exam, she had 2 tender, hard, mobile nodules measuring approximately 5mm each located at 4cm and 2...
March 26, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29541951/the-prevalence-and-clinical-significance-of-anti-puf60-antibodies-in-patients-with-idiopathic-inflammatory-myopathy
#7
Ya-Mei Zhang, Han-Bo Yang, Jing-Li Shi, He Chen, Xiao-Ming Shu, Xin Lu, Guo-Chun Wang, Qing-Lin Peng
Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. In our study, 388 IIM patients, 301 disease controls, and 167 healthy controls (HCs) were involved. An enzyme-linked immunosorbent assay (ELISA) was developed to detect serum anti-PUF60 levels and was validated using immunoblotting methods...
March 15, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29532710/myositis-specific-autoantibodies-in-japanese-patients-with-juvenile-idiopathic-inflammatory-myopathies
#8
Masahiro Ueki, Ichiro Kobayashi, Shunichiro Takezaki, Yusuke Tozawa, Yuka Okura, Masafumi Yamada, Masataka Kuwana, Tadashi Ariga
OBJECTIVES: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs). METHODS: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district. RESULTS: Eighteen of 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), 5 with anti-transcriptional intermediary factor (TIF)-1γ (20%), 4 with anti-MJ/nuclear matrix protein (NXP)-2 (16%), 2 with anti-Jo-1 (8%), 1 with anti- HMG-CoA reductase, 1 with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each...
March 13, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29512806/a-severe-anti-mda-5-antibodies-associated-amyopathic-dermatomyositis
#9
Mounira El Euch, Wifak Bani, Madiha Mahfoudhi, Fatima Jaziri, Khaoula Ben Abdelghani, Sami Turki, Taïeb Ben Abdallah
BACKGROUND: Lung damage during amyopathic dermatomyositis (ADM) associated with auto antibodies anti MDA-5 is serious. We report a rare observation of a severe ADM associated to anti MDA-5 antibodies complicated with diffuse interstitial lung disease (ILD) rapidly extensive and fatal. OBSERVATION: We report the observation of a Tunisian adult who was admitted for ADM. The anti MDA-5 antibodies were detected. He developed an ILD rapidly extensive to fibrosis. Corticosteroids were unefficient and he deceded of pneumomediastin and refractory respiratory distress...
June 2017: La Tunisie Médicale
https://www.readbyqxmd.com/read/29491297/a-case-of-clinically-amyopathic-dermatomyositis-with-interstitial-pneumonia-that-was-successfully-treated-with-plasma-exchange
#10
Mizuki Yagishita, Yuya Kondo, Toshihiko Terasaki, Mayu Terasaki, Masaru Shimizu, Fumika Honda, Ayako Oyama, Hiroyuki Takahashi, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Hiroto Tsuboi, Isao Matsumoto, Takayuki Sumida
Patients with clinically amyopathic dermatomyositis (CADM), a subset of dermatomyositis characterized by a lack of muscle involvement, frequently develop rapidly progressive and treatment-resistant interstitial lung disease. We report the case of a 49-year-old man who was diagnosed with CADM. He developed interstitial pneumonia, which did not respond to combination therapy with methylprednisolone pulse therapy, cyclophosphamide, and cyclosporine. We therefore attempted plasma exchange. After 7 courses of therapeutic plasma exchange, the interstitial pneumonia gradually improved...
February 28, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29480875/evidence-supports-blind-screening-for-internal-malignancy-in-dermatomyositis-data-from-2-large-us-dermatology-cohorts
#11
MULTICENTER STUDY
Hayley Leatham, Courtney Schadt, Sarah Chisolm, Deborah Fretwell, Lorinda Chung, Jeffrey P Callen, David Fiorentino
The association between dermatomyositis and internal malignancy is well established, but there is little consensus about the methods of cancer screening that should be utilized.We wished to analyze the prevalence and yield of selected cancer screening modalities in patients with dermatomyositis.We performed a retrospective analysis of 2 large US dermatomyositis cohorts comprising 400 patients.We measured the frequency of selected screening tests used to search for malignancy. Patients with a biopsy-confirmed malignancy were identified...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29469754/clinical-improvement-of-a-patient-with-both-amyopathic-dermatomyositis-and-psoriasis-following-treatment-with-cyclosporine
#12
Megan S Inkeles, Daniel No, Jashin J Wu
Clinically amyopathic dermatomyositis (CADM) is an uncommon subtype of dermatomyositis that rarely presents simultaneously with psoriasis. There are subsequently few reports discussing the management of concurrent CADM and psoriasis. Furthermore, skin lesions of CADM are often recalcitrant to first line dermatomyositis interventions. We present a case of a 45-year-old woman with both CADM and psoriasis whose lesions were resistant to multiple therapies; she eventually achieved disease control and remission with cyclosporine...
August 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29441299/livedo-racemosa-reticulated-ulcerations-panniculitis-and-violaceous-plaques-in-a-46-year-old-woman
#13
Alfredo Agulló, Brian Hinds, Mónica Larrea, Ignacio Yanguas
Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash)...
January 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29417209/rapidly-progressive-interstitial-lung-disease-due-to-anti-mda5-antibodies-without-skin-involvement-a-case-report-and-literature-review
#14
REVIEW
Juan González-Moreno, Manuel Raya-Cruz, Ines Losada-Lopez, Ana Paula Cacheda, Cristina Oliver, Bartomeu Colom
Anti-MDA5 antibodies have been strongly associated with rapidly progressive interstitial lung disease (RP-ILD) in dermatomyositis (DM) patients, especially in the clinically amyopathic subset (CADM). We present a case of anti-MDA5 antibody-associated RP-ILD in a patient with arthritis but with no other clinical signs suggestive of DM or CADM successfully treated with a combination of cyclophosphamide, cyclosporine and corticoids. A review of the literature was also done. Despite its rarity, anti-MDA5 antibody-associated ILD should be suspected in cases of RP-ILD even without other signs of DM or CADM as prompt and aggressive treatment could improve prognosis...
February 7, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29411180/adam-17-is-expressed-in-the-inflammatory-myopathy-and-is-involved-with-interstitial-lung-disease
#15
Airi Nishimi, Takeo Isozaki, Shinichiro Nishimi, Sho Ishii, Takahiro Tokunaga, Hidekazu Furuya, Kuninobu Wakabayashi, Tsuyoshi Kasama
The "A disintegrin and metalloprotease" (ADAM) family is thought to play an important role in tissue destruction and inflammatory reactions. ADAM-17 was first described as the protease responsible for tumor necrosis factor (TNF)-α shedding. Here, we have shown the expression of ADAM-17 in inflammatory myopathy and demonstrated the role of inflammation in interstitial lung diseases (ILD). ADAM-17 in inflammatory myopathy serum [polymyositis (n = 26), dermatomyositis (n = 34), and clinically amyopathic dermatomyositis (n = 10)] and healthy control (n = 19) was measured using enzyme-linked immunosorbent assay...
April 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29380533/analysis-of-anti-melanoma-differentiation-associated-gene-5-antibody-in-hong-kong-chinese-patients-with-idiopathic-inflammatory-myopathies-diagnostic-utility-and-clinical-correlations
#16
Ho So, Ricky W-K Ip, Victor T-L Wong, Ronald M-L Yip
AIM: To compare the prevalence of the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) in Hong Kong Chinese patients with dermatomyositis (DM) and polymyositis (PM); in addition, to examine the association of anti-MDA5 Ab and the clinical characteristics of these patients. METHODS: Twenty consecutive existing patients with DM being followed up at the Rheumatology Clinic of Kwong Wah Hospital, Hong Kong were recruited. Twenty patients with PM were recruited from the same clinic as the controls...
January 30, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29346626/anti-aminoacyl-trna-synthetase-antibody-positive-clinically-amyopathic-dermatomyositis
#17
Toshiki Ebisudani, Takanori Asakura, Soichiro Ueda, Makoto Ishii, Tomoko Betsuyaku
No abstract text is available yet for this article.
January 15, 2018: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/29338781/correlation-between-disease-activity-and-serum-ferritin-in-clinically-amyopathic-dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-a-case-report
#18
Kazuhiro Yamada, Kazuhisa Asai, Atsuko Okamoto, Tetsuya Watanabe, Hiroshi Kanazawa, Mai Ohata, Masahiko Ohsawa, Kazuto Hirata
BACKGROUND: Clinically amyopathic dermatomyositis with anti-Melanoma Differentiation-Associated gene 5 (MDA5) antibody often presents with severe interstitial lung disease. Although serum ferritin level is known to reflect interstitial lung disease activity, there are few case reports describing this association. CASE PRESENTATION: A 58-year-old man was referred to our outpatient clinic with a 3-week history of cough and respiratory distress. He had erythema over the V area of the neck and a Gottron's sign...
January 16, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29331962/splicing-variant-of-wdfy4-augments-mda5-signalling-and-the-risk-of-clinically-amyopathic-dermatomyositis
#19
Yuta Kochi, Yoichiro Kamatani, Yuya Kondo, Akari Suzuki, Eiryo Kawakami, Ryosuke Hiwa, Yukihide Momozawa, Manabu Fujimoto, Masatoshi Jinnin, Yoshiya Tanaka, Takashi Kanda, Robert G Cooper, Hector Chinoy, Simon Rothwell, Janine A Lamb, Jiří Vencovský, Heřman Mann, Koichiro Ohmura, Keiko Myouzen, Kazuyoshi Ishigaki, Ran Nakashima, Yuji Hosono, Hiroto Tsuboi, Hidenaga Kawasumi, Yukiko Iwasaki, Hiroshi Kajiyama, Tetsuya Horita, Mariko Ogawa-Momohara, Akito Takamura, Shinichiro Tsunoda, Jun Shimizu, Keishi Fujio, Hirofumi Amano, Akio Mimori, Atsushi Kawakami, Hisanori Umehara, Tsutomu Takeuchi, Hajime Sano, Yoshinao Muro, Tatsuya Atsumi, Toshihide Mimura, Yasushi Kawaguchi, Tsuneyo Mimori, Atsushi Takahashi, Michiaki Kubo, Hitoshi Kohsaka, Takayuki Sumida, Kazuhiko Yamamoto
OBJECTIVES: Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare autoimmune diseases in which both genetic and environmental factors play important roles. To identify genetic factors of IIM including polymyositis, dermatomyositis (DM) and clinically amyopathic DM (CADM), we performed the first genome-wide association study for IIM in an Asian population. METHODS: We genotyped and tested 496 819 single nucleotide polymorphism for association using 576 patients with IIM and 6270 control subjects...
April 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29301801/clinically-amyopathic-dermatomyositis-associated-with-anti-mda5-antibody
#20
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
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