The Experts below are selected from a list of 9411 Experts worldwide ranked by ideXlab platform
Ichiro Katayama - One of the best experts on this subject based on the ideXlab platform.
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anti mda5 antibody positive Dermatomyositis with lethal progressive interstitial lung disease and advanced gastric cancer
European Journal of Dermatology, 2014Co-Authors: Toshifumi Yamaoka, Yasuhito Hamaguchi, Manabu Fujimoto, Hiroyuki Murota, Chie Doi, Akinori Yokomi, Atsushi Tanemura, Mamori Tani, Hiroko Saruban, Ichiro KatayamaAbstract:Dermatomyositis (DM) is an autoimmune inflammatory disease often associated with internal malignancy and interstitial lung disease. The latter is found in about 50% of patients with DM and is a life-threatening complication of the disease [1]. Rapidly progressive interstitial lung disease (RP-ILD) associated with clinically amyopathic Dermatomyositis (CADM) is often lethal, despite treatment by corticosteroid therapy combined with immunosuppressive drugs [2].A number of autoantibodies can be detected [...]
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a case of juvenile Dermatomyositis manifesting inflammatory epidermal nevus like skin lesions unrecognized cutaneous manifestation of blaschkitis
Allergology International, 2010Co-Authors: Yuji Takahashi, Hiroyuki Murota, Shigetoshi Sano, Takeshi Okinaga, Koji Tominaga, Toshie Yano, Ichiro KatayamaAbstract:ABSTRACT Background Juvenile Dermatomyositis is potentially life threatening rare autoimmune illness that mainly affects muscle and skin. Cutaneous features are useful in establishing the diagnosis of this disease. Case Summary We report an 8-year-old male juvenile Dermatomyositis who presented epidermal nevus like- lesions on the back of the right thigh. Characteristic cutaneous changes such as Gottron's papules of the hand, heliotrope rash of the eyelids, and poikiloderma-like lesions on the back were observed. Diagnosis of juvenile Dermatomyositis was made by positive muscle biopsy and magnetic resonance imaging findings and typical cutaneous manifestations. However, epidermal nevus-like skin lesions, an acquired inflammatory dermatosis that follows Blaschko lines, seen in this case have been rarely reported in the literatures. Discussion We would like to report this case and discuss about the significance and pathogenesis of this rare cutaneous manifestation like Blaschkitis in juvenile Dermatomyositis.
Shinji Sato - One of the best experts on this subject based on the ideXlab platform.
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successful polymyxin b hemoperfusion treatment associated with serial reduction of serum anti cadm 140 mda5 antibody levels in rapidly progressive interstitial lung disease with amyopathic Dermatomyositis
Chest, 2013Co-Authors: Aoi Teruya, Kodai Kawamura, Yuko Yasuda, Kazuya Ichikado, Shinji Sato, Masakazu YoshiokaAbstract:Clinically amyopathic Dermatomyositis (CADM), a subtype of Dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with Dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic Dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.
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amyopathic Dermatomyositis developing rapidly progressive interstitial lung disease with elevation of anti cadm 140 mda5 autoantibodies
Modern Rheumatology, 2012Co-Authors: Shinji Sato, Masataka Kuwana, Yasuo SuzukiAbstract:Anti-clinically amyopathic Dermatomyositis (CADM)-140/MDA5 autoantibodies are specifically detected in patients with Dermatomyositis and are known to have a strong association with rapidly progressive interstitial lung disease (RP-ILD). Here we report an amyopathic Dermatomyositis (ADM) patient who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 titer. Respiratory symptoms gradually improved, and anti-CADM-140/MDA5 titer decreased in parallel to below the cutoff level. It may be useful to quantify CADM-140-specific autoantibodies for monitoring disease activity in patients with ADM and RP-ILD.
Yasuo Suzuki - One of the best experts on this subject based on the ideXlab platform.
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amyopathic Dermatomyositis developing rapidly progressive interstitial lung disease with elevation of anti cadm 140 mda5 autoantibodies
Modern Rheumatology, 2012Co-Authors: Shinji Sato, Masataka Kuwana, Yasuo SuzukiAbstract:Anti-clinically amyopathic Dermatomyositis (CADM)-140/MDA5 autoantibodies are specifically detected in patients with Dermatomyositis and are known to have a strong association with rapidly progressive interstitial lung disease (RP-ILD). Here we report an amyopathic Dermatomyositis (ADM) patient who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 titer. Respiratory symptoms gradually improved, and anti-CADM-140/MDA5 titer decreased in parallel to below the cutoff level. It may be useful to quantify CADM-140-specific autoantibodies for monitoring disease activity in patients with ADM and RP-ILD.
Molly A Kantor - One of the best experts on this subject based on the ideXlab platform.
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anti mda5 positive Dermatomyositis presenting as fever of unknown origin
Journal of General Internal Medicine, 2016Co-Authors: Neera S Narang, Anna Postolova, Nicole M Seminara, Molly A KantorAbstract:Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of Dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations. The diagnosis of Dermatomyositis was not evident on several clinical encounters due to the absence of these hallmark symptoms. We review the variable clinical manifestations of a subtype of Dermatomyositis associated with an autoantibody against melanoma differentiation-associated protein 5 (anti-MDA5) and suggest consideration of Dermatomyositis as a diagnosis in patients presenting with systemic illness and markedly elevated ferritin, even in the absence of elevated muscle enzymes and classic autoantibodies.
Masakazu Yoshioka - One of the best experts on this subject based on the ideXlab platform.
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successful polymyxin b hemoperfusion treatment associated with serial reduction of serum anti cadm 140 mda5 antibody levels in rapidly progressive interstitial lung disease with amyopathic Dermatomyositis
Chest, 2013Co-Authors: Aoi Teruya, Kodai Kawamura, Yuko Yasuda, Kazuya Ichikado, Shinji Sato, Masakazu YoshiokaAbstract:Clinically amyopathic Dermatomyositis (CADM), a subtype of Dermatomyositis with subtle or no muscle involvement, is occasionally accompanied by fatal, rapidly progressive interstitial lung disease (RP-ILD) that is resistant to aggressive immunosuppressive therapy. The presence of anti-CADM-140/MDA5 antibodies is diagnostic for patients with Dermatomyositis (particularly CADM) and is known to be strongly associated with the pathogenesis, disease activity, and mortality of RP-ILD. Polymyxin-B direct hemoperfusion (PMX-DHP), originally developed for the removal of endotoxin, has been demonstrated to be effective for treating various types of acute respiratory failure. We describe a patient with amyopathic Dermatomyositis who developed RP-ILD characterized by elevated anti-CADM-140/MDA5 autoantibodies, was resistant to combined steroid and immunosuppressant therapy, and was treated successfully with PMX-DHP. To our knowledge, this is the first case to indicate a serial reduction of anti-CADM-140/MDA5 autoantibodies, associated with clinical improvement, following PMX-DHP. Early intervention using PMX-DHP may improve the prognosis of RP-ILD accompanied by CADM.