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amyopathic dermatomyositis

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https://www.readbyqxmd.com/read/28443580/amyopathic-dermatomyositis-associated-with-histopathological-findings-of-organizing-pneumonia-and-pulmonary-vasculitis
#1
Jeong Uk Lim, Hye Seon Kang, Yong Hyun Kim, Tae Jung Kim
BACKGROUND: Clinically amyopathic dermatomyositis (CADM) is a distinct subgroup of dermatomyositis characterized by unique dermatologic manifestations without muscle involvement. CADM is frequently associated with interstitial lung disease (ILD), which usually has a rapidly progressive, fatal clinical course. Although CADM-related ILD (CADM-ILD) is well-described, data on the histopathology of CADM-ILD are limited. Organizing pneumonia and pulmonary vasculitis have rarely been reported...
April 13, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28422003/efficacy-and-safety-of-leflunomide-as-an-adjuvant-drug-in-refractory-dermatomyositis-with-primarily-cutaneous-activity
#2
Renata C de Souza, Fernando H C de Souza, Renata Miossi, Samuel K Shinjo
OBJECTIVES: To evaluate leflunomide as an adjuvant drug in refractory dermatomyositis (DM) with primarily cutaneous activity. METHODS: A retrospective, single-centre, cohort study including 18 adult patients with DM (classical or clinically amyopathic DM) and cutaneous activity from 2001 to 2016 was conducted. Patients were dependent on glucocorticoid and refractory to at least two full-dose immunosuppressants/immunomodulators or presented previous adverse events with immunobiological drugs...
April 18, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28413686/cytokine-profiles-of-amyopathic-dermatomyositis-with-interstitial-lung-diseases-treated-with-mycophenolate
#3
Masachika Hayashi, Ami Aoki, Katsuaki Asakawa, Takuro Sakagami, Toshiaki Kikuchi, Toshinori Takada
A 59-year-old Japanese man diagnosed with interstitial lung disease associated with amyopathic dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies was treated with intravenous methyl prednisolone (PSL) 1000 mg, oral PSL 1 mg/kg, and oral cyclosporin 200 mg daily. His respiratory condition worsened after treatment with two times of intravenous cyclophosphamide and another steroid pulse therapy as well as PSL and cyclosporin. Addition of mycophenolate mofetil (MMF), 1...
July 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28393009/a-case-of-anti-aminoacyl-trna-synthetase-ars-antibody-positive-polymyositis-pm-dermatomyositis-dm-associated-interstitial-pneumonia-ip-successfully-controlled-with-bosentan-therapy
#4
Tomoyuki Naito, Yosuke Tanaka, Mitsunori Hino, Akihiko Gemma
A 72-year-old woman was admitted to our hospital and was diagnosed with interstitial pneumonia (IP) associated with amyopathic dermatomyositis (ADM). The patient experienced three acute IP exacerbations in the 7 years that followed, which were each treated and resolved with steroid pulse therapy. The patient was closely examined for respiratory failure with right heart catheterization (RHC), which demonstrated that she had a mean pulmonary artery pressure (mPAP) of 34 mmHg. The patient was thus diagnosed as having pulmonary hypertension (PH) associated with anti-synthetase syndrome (ASS) and was started on bosentan therapy, which led to improvements in mPAP as well as in subjective symptoms over time...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28384112/inflammatory-myopathies-with-cutaneous-involvement-from-diagnosis-to-therapy
#5
Lyubomir A Dourmishev
The group of idiopathic inflammatory myopathies (IIM) include various disorders of skeletal muscles with or without skin involvement. The most common types are dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Dermatomyositis subdivides into various clinical forms such as juvenile, amyopathic or paraneoplastic dermatomyositis, scleromyositis, overlap or anti-synthetase syndromes, etc. Recently, numerous new antibodies defining the characteristic clinical phenotype have been described as anti-MDA5 antibodies associated with interstitial lung disease and amyopathic dermatomyositis or anti-TIF1γ antibodies as markers for paraneoplastic dermatomyositis...
March 1, 2017: Folia Medica
https://www.readbyqxmd.com/read/28374003/rapidly-progressive-melanoma-differentiation-associated-protein-5-positive-amyopathic-dermatomyositis-in-an-hiv-positive-patient
#6
Ashutosh Sharma, Marie Hudson, Kevin Watters, Robin Billick, Sabrina Fallavolita, Elena Netchiporouk
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28274528/predictive-factors-for-long-term-outcome-in-polymyositis-dermatomyositis-associated-interstitial-lung-diseases
#7
Tomoyuki Fujisawa, Hironao Hozumi, Masato Kono, Noriyuki Enomoto, Yutaro Nakamura, Naoki Inui, Ran Nakashima, Yoshitaka Imura, Tsuneyo Mimori, Takafumi Suda
BACKGROUND: Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28210382/paraneoplastic-dermatomyositis-with-cutaneous-and-myopathic-disease-responsive-to-adrenocorticotropic-hormone-therapy
#8
Marisa Wolff, Christopher Mancuso, Karan Lal, Damian Dicostanzo, Charles Gropper
Dermatomyositis is a myopathic or amyopathic autoimmune connective tissue disease that presents with classic dermatologic findings ranging from: poikilodermatous photosensitivity (shawl sign), eyelid edema and violaceous-pigmentation (heliotrope sign), lichenoid eruptions on the knuckles and elbows (Gottron's sign), periungual telangiectasias, and ragged cuticles (Samitz sign). Up to 30 percent of adult-onset cases of dermatomyositis may represent a paraneoplastic syndrome warranting a thorough work-up for malignancy...
January 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/28154280/an-atypical-clinical-course-of-anti-mda5-antibody-positive-interstitial-lung-disease-in-a-patient-with-three-deteriorations-in-9-years
#9
Yuki Sato, Kojiro Otsuka, Koji Tamai, Yuichiro Ono, Yasuhito Hamaguchi, Keisuke Tomii
Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28129490/the-cutaneous-and-systemic-findings-associated-with-nuclear-matrix-protein-2-antibodies-in-adult-dermatomyositis-patients
#10
Anna Rogers, Lorinda Chung, Shufeng Li, Livia Casciola-Rosen, David F Fiorentino
OBJECTIVE: To characterize the cutaneous and systemic clinical phenotype of dermatomyositis patients with anti-NXP-2 antibodies. METHODS: We conducted a retrospective cohort analysis of 178 dermatomyositis patients seen at the Stanford University Clinic. Electronic chart review employing a keyword search strategy was performed to collect clinical and laboratory data. Anti-NXP-2 antibodies were assayed by immunoprecipitation using NXP-2 produced by in vitro transcription/translation...
January 27, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28127433/fibrocellular-non-specific-interstitial-pneumonia-with-favourable-prognosis-in-clinically-amyopathic-dermatomyositis
#11
Takahiro Yoshizawa, Keishi Sugino, Kazutoshi Shibuya, Toshimasa Uekusa, Sakae Homma
We report the case of a 49-year-old man who experienced dry cough and itching eruption for 2 months. The patient had Gottoron's sign, mechanic's hand, and high levels of serum Krebs von den Lungen-6 and surfactant protein D, but was negative for anti-aminoacyl transfer RNA synthetase antibodies. High-resolution chest computed tomography revealed subpleural band-like or patchy consolidation with predominance in the bilateral lower lobe. Bronchoalveolar lavage fluid showed increased total cell counts with lymphocytosis...
September 2016: Respirology Case Reports
https://www.readbyqxmd.com/read/28099216/a-rare-case-of-amyopathic-juvenile-dermatomyositis-associated-to-psoriasis-successfully-treated-with-ustekinumab
#12
Claudia L Montoya, Martha L Gonzalez, Fabio E Ospina, Gabriel J Tobón
Coexistence between two or more immunological-mediated diseases implies a clinical and therapeutic challenge that is uncommon in the clinical field. We report a rare case of a young patient with amyopathic juvenile dermatomyositis and erythrodermic psoriasis, with an excellent response to ustekinumab.
January 18, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28089977/antimelanoma-differentiation-associated-gene-5-antibody-expanding-the-clinical-spectrum-in-north-american-patients-with-dermatomyositis
#13
Siamak Moghadam-Kia, Chester V Oddis, Shinji Sato, Masataka Kuwana, Rohit Aggarwal
OBJECTIVE: To determine the clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM. METHODS: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/27995057/rapidly-progressive-interstitial-lung-disease-due-to-anti-mda-5-antibody-positive-clinically-amyopathic-dermatomyositis-complicated-with-cervical-cancer-successful-treatment-with-direct-hemoperfusion-using-polymyxin-b-immobilized-fiber-column-therapy
#14
Hidenori Ichiyasu, Yasumiko Sakamoto, Chieko Yoshida, Kazuhiko Sakamoto, Ryosuke Fujita, Go Nakayama, Hiroko Okabayashi, Sho Saeki, Shinichiro Okamoto, Hirotsugu Kohrogi
The anti-melanoma differentiation-associated gene 5 (MDA-5) antibody is a marker of clinically amyopathic dermatomyositis (CADM) and rapidly progressive interstitial lung disease (ILD) with acute respiratory failure. A 35-year-old woman with cervical cancer showed Gottron's papules, severe hypoxemia, and diffuse ground-glass opacities on chest computed tomography. She was diagnosed with rapidly progressive ILD associated with CADM. Her serum was positive for the anti-MDA-5 antibody. Combination therapy with corticosteroids, immunosuppressants, and direct hemoperfusion using polymyxin B-immobilized fiber column (PMX-DHP) improved her respiratory dysfunction...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27928683/specific-autoantibodies-in-dermatomyositis-a-helpful-tool-to-classify-different-clinical-subsets
#15
Giulia Merlo, Andrea Clapasson, Emanuele Cozzani, Luigi Sanna, Giampaola Pesce, Marcello Bagnasco, Martina Burlando, Aurora Parodi
Autoantibodies are important in the diagnosis of dermatomyositis. They can be divided in two different groups: myositis-associated autoantibodies (MAA) prevailing in overlap syndromes, and myositis-specific autoantibodies (MSA), with diagnostic specificity exceeding 90%. Our purpose was to detect retrospectively the prevalence of the most common MSAs in a group of 19 adult DM patients (13 women, 6 men). A severe DM (SDM), with extensive cutaneous and muscular manifestations, dysphagia, and sometimes pneumopathy, was detected in ten cases...
March 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/27919567/splicing-factor-proline-glutamine-rich-is-a-novel-autoantigen-of-dermatomyositis-and-associated-with-anti-melanoma-differentiation-associated-gene-5-antibody
#16
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...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27856478/prognostic-factors-of-idiopathic-inflammatory-myopathies-complicated-with-interstitial-lung-disease-protocol-for-a-systematic-review-and-meta-analysis
#17
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
https://www.readbyqxmd.com/read/27813167/how-often-are-pediatric-patients-with-clinically-amyopathic-dermatomyositis-truly-amyopathic
#18
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...
January 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/27784516/dermatomyositis-part-1-definition-epidemiology-etiology-and-pathogenesis-and-clinics
#19
REVIEW
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
https://www.readbyqxmd.com/read/27693019/enzyme-linked-immunosorbent-assays-for-detection-of-anti-transcriptional-intermediary-factor-1-gamma-and-anti-mi-2-autoantibodies-in-dermatomyositis
#20
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...
December 2016: Journal of Dermatological Science
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