The Experts below are selected from a list of 204 Experts worldwide ranked by ideXlab platform
Jun Shimizu - One of the best experts on this subject based on the ideXlab platform.
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Inflammatory myopathies associated with Anti-Mitochondrial antibodies
Brain, 2012Co-Authors: Meiko Hashimoto Maeda, Shoji Tsuji, Jun ShimizuAbstract:Anti-Mitochondrial antibodies, the characteristic markers of primary biliary cirrhosis, have been detected in most patients with this disease. However, the prevalence of these antibodies in inflammatory myopathies and their clinical and histopathological significance has not been determined. Sera from 212 consecutive patients with inflammatory myopathies were screened for Anti-Mitochondrial antibodies by enzyme-linked immunosorbent assay. The clinical and histopathological features of Anti-Mitochondrial Antibody-positive patients were analysed and statistically compared with those of Anti-Mitochondrial Antibody-negative patients. Twenty-four patients positive for Anti-Mitochondrial antibodies (seven patients with and 17 patients without primary biliary cirrhosis) were identified (11.3%). Thirteen patients had a clinically chronic disease course of >12 months before their diagnosis at hospitals. Six of these 13 patients (four asymptomatic patients with increased creatine kinase levels and two patients with arrhythmia) had not been aware of muscle weakness, but all 13 patients had muscle atrophy at initial presentation. As complications, eight patients had cardiac involvement including arrhythmias (five patients with supraventricular tachycardia; two with ventricular tachycardia; and one patient with atrioventricular block), six patients had moderately decreased ejection fraction and six patients had decreased vital capacity, two of whom required respiratory support. Regarding muscle histopathological findings, in addition to inflammation, 13 patients had chronic myopathic changes and six had granulomatous lesions. Statistical analysis showed that the clinical features of a chronic disease course, cardiac involvement and muscle atrophy, and the histopathological features of chronic myopathic changes and granulomatous inflammation, were significantly more frequently observed in patients with Anti-Mitochondrial Antibody-positive inflammatory myopathy than in patients who were negative for Anti-Mitochondrial antibodies. Except for cardiac involvement, which is more frequently observed in patients with primary biliary cirrhosis, no significant differences in clinical or histopathological features were found between patients with or without primary biliary cirrhosis. Our study revealed that inflammatory myopathies associated with Anti-Mitochondrial antibodies were frequently found in patients with the clinical features of a chronic disease course, muscle atrophy and cardiopulmonary involvement, and the characteristic histopathological feature of granulomatous inflammation. Our study suggests that inflammatory myopathies associated with Anti-Mitochondrial antibodies form a characteristic subgroup.
Akatsuki Kubota - One of the best experts on this subject based on the ideXlab platform.
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Variable Cardiac Responses to Immunosuppressive Therapy in Anti-Mitochondrial Antibody-Positive Myositis.
Canadian Journal of Cardiology, 2019Co-Authors: Satoshi Bujo, Eisuke Amiya, Toshiya Kojima, Shintaro Yamada, Hisataka Maki, Masato Ishizuka, Masae Uehara, Yumiko Hosoya, Masaru Hatano, Akatsuki KubotaAbstract:Abstract We describe a case of Anti-Mitochondrial Antibody-positive myositis associated with cardiovascular involvement. An electrophysiological study (EPS) showed binodal dysfunction, and cardiac magnetic resonance (CMR) imaging revealed left ventricular dysfunction with diffuse, patchy T2 high-intensity areas and late gadolinium enhancement indicative of inflammation and fibrosis. The left ventricular dysfunction was successfully treated with immunosuppressive therapy as documented by CMR. Persistence of conduction system dysfunction was confirmed by EPS, and a pacemaker was implanted. CMR and EPS concisely documented the variable cardiac response to treatment in Anti-Mitochondrial Antibody-positive myositis. We demonstrate the utility of cardiac investigations in this rare disorder.
Marvin J. Fritzler - One of the best experts on this subject based on the ideXlab platform.
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Primary biliary cirrhosis and autoantibodies.
Japanese Journal of Clinical Immunology, 2008Co-Authors: K. Miyachi, Hiroshi Miyakawa, Oda M, Tsuneyoshi Horigome, Marvin J. FritzlerAbstract:Abstract Fifty years have passed since Anti-Mitochondrial antibodies were found in patients with primary biliary cirrhosis (PBC). PBC is an autoimmune hepatic disease in which 85-90% of patient antibodies bind to mitochondrial antigens that include pyruvate dehydrogenase complex (PDC)-E2 and other members of the oxaloacid dehydrogenase family. In addition, indirect immunofluorescence (IIF) assays utilizing HEp-2 cell substrates have been used to identify anti-centromere antibodies in 20-30% of PBC sera. These antibodies are generally easily recognized, however, anti-nuclear envelope and anti-multiple nuclear dot antibodies are occasionally more difficult to recognize with certainty by IIF. The use of enzyme linked immunosorbent assays that utilize recombinant gp210 (an autoantigen of the nuclear envelope) and/or sp100 (a protein target represented by multiple nuclear dots) should be particularly considered in Anti-Mitochondrial Antibody negative PBC sera. Although the clinical significance of these antibodies still remains to be determined, there is evidence that the existence of anti-gp210 antibodies are related to poorer prognosis and more aggressive disease progression.
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A case of limited cutaneous systemic sclerosis developing anti-mitochondria Antibody positive primary biliary cirrhosis after acute myocardial infarction.
Clinical Rheumatology, 2006Co-Authors: Kiyomitsu Miyachi, Tetsuroh Okano, Raleigh W. Hankins, Minoru Ihara, Akira Miyamoto, Miwako Iwai, Katsuhiko Mikoshiba, Marvin J. FritzlerAbstract:In this report, we present a 63-year-old woman who had limited cutaneous systemic sclerosis and subsequently developed typical primary biliary cirrhosis after an acute myocardial infarction. The patient initially developed Raynaud's phenomenon, and 4 years later visited the clinic in 1994 complaining of abdominal distress, xerostomia, and xerophthalmia. A diagnosis of limited cutaneous systemic sclerosis was based on Raynaud's phenomenon, sclerodactyly and anti-centromere antibodies. She was also found to have anti-inositol 1,4,5-trisphosphate receptor 3 (IP(3)R3) antibodies, but Anti-Mitochondrial antibodies were only weakly positive. Seven years later, she developed vertigo and nausea, and was hospitalized due to complaints of an oppressive sensation of the anterior chest. Electrocardiogram results showed a reduction of R waves and ST segment elevation in II, III, and aVf leads. Coronary angiography showed 99% obstruction of the left anterior descending artery and 50% of stenosis of the right coronary artery. Three years later, the patient was noted to have Anti-Mitochondrial antibodies. Retrospective analysis of the patient's sera showed that IP(3)R3 antibodies were decreasing. Since myocardium is particularly rich in mitochondria, it is thought that myocardial infarction may have been the triggering event that initiated antigen-presenting cells to selectively induce an Anti-Mitochondrial Antibody response.
Chuanzhu Yan - One of the best experts on this subject based on the ideXlab platform.
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Idiopathic inflammatory myopathies with Anti-Mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort.
Neuromuscular Disorders, 2019Co-Authors: Ying Hou, Meirong Liu, Yue-bei Luo, Yuan Sun, Kai Shao, Tingjun Dai, Yuying Zhao, Chuanzhu YanAbstract:Abstract Anti-Mitochondrial antibodies, the hallmark of primary biliary cirrhosis, have been detected in many patients with idiopathic inflammatory myopathies and these Anti-Mitochondrial-Antibody-associated idiopathic inflammatory myopathies frequently show unique characteristics. We detected Anti-Mitochondrial antibodies in Chinese idiopathic inflammatory myopathy and summarized the clinical findings of these Anti-Mitochondrial-Antibody-positive patients. Of 136 patients, seven (5.15%) were found to be Anti-Mitochondrial-Antibody-positive. Primary biliary cirrhosis was present in 2 of these 7 patients, chronic disease duration in 2 patients and asymmetrical muscle weakness in 4 patients. The mean disease course was 8.58 months, and the mean creatine kinase level was 2256.53 U/L. Myositis-specific antibodies were found in 3 patients. According to clinical features and muscle histopathological findings, 3 patients were classified as dermatomyositis, 2 as possible polymyositis and 2 as necrotizing autoimmune myopathy. Of the 6 Anti-Mitochondrial-Antibody-positive patients receiving follow-ups of 12–83 months, they all showed marked clinical improvement. Our study indicates that Anti-Mitochondrial antibodies are relatively rare in Chinese idiopathic inflammatory myopathy patients. These patients generally show various clinical features and have favorable treatment outcomes. Anti-Mitochondrial Antibody testing may be helpful to confirm the diagnosis of idiopathic inflammatory myopathy, especially in patients with atypical manifestations.
Hiroshi Miyakawa - One of the best experts on this subject based on the ideXlab platform.
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Primary biliary cirrhosis and autoantibodies.
Japanese Journal of Clinical Immunology, 2008Co-Authors: K. Miyachi, Hiroshi Miyakawa, Oda M, Tsuneyoshi Horigome, Marvin J. FritzlerAbstract:Abstract Fifty years have passed since Anti-Mitochondrial antibodies were found in patients with primary biliary cirrhosis (PBC). PBC is an autoimmune hepatic disease in which 85-90% of patient antibodies bind to mitochondrial antigens that include pyruvate dehydrogenase complex (PDC)-E2 and other members of the oxaloacid dehydrogenase family. In addition, indirect immunofluorescence (IIF) assays utilizing HEp-2 cell substrates have been used to identify anti-centromere antibodies in 20-30% of PBC sera. These antibodies are generally easily recognized, however, anti-nuclear envelope and anti-multiple nuclear dot antibodies are occasionally more difficult to recognize with certainty by IIF. The use of enzyme linked immunosorbent assays that utilize recombinant gp210 (an autoantigen of the nuclear envelope) and/or sp100 (a protein target represented by multiple nuclear dots) should be particularly considered in Anti-Mitochondrial Antibody negative PBC sera. Although the clinical significance of these antibodies still remains to be determined, there is evidence that the existence of anti-gp210 antibodies are related to poorer prognosis and more aggressive disease progression.
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Detection and significance of Anti-Mitochondrial Antibody in various collagen diseases
Kanzo, 2007Co-Authors: Eriko Iwasawa, Hiroshi Miyakawa, K Kikuchi, Akiko Nimi, Masako Hara, Naoyuki KamataniAbstract:原発性胆汁性肝硬変（PBC）は，自己免疫異常に基づく肝疾患で，各種膠原病を少なからず合併する．しかしながら，各種膠原病におけるPBCの合併については不明な点も少なくない．そこで今回，各種膠原病を対象として，PBCの血清診断に必須の抗ミトコンドリア抗体（AMA）を検索し，その臨床的意義を検討した．各種膠原病と診断された302例を対象として，間接蛍光抗体法とELISA法にてAMAをスクリーニングし，さらにWestern blot法にてAMAの解析を行った．AMAは302例中14例（4.6%）と比較的高率に検出され，疾患別には，強皮症で4例，全身性エリテマトーデスで3例，慢性関節リウマチと血管炎で各2例に検出された．PDC-E2抗体（74kDa）は6例に検出されたに過ぎなかったが，BCOADC-E2抗体（50kDa）は10例と多数に検出された．この14例のうち6例に，抗セントロメア抗体が検出された．さらに,14例中9例は経過中PBCの合併は疑われておらず，今回の検討で初めてAMA陽性と判明した症例であった．膠原病の診療においてもPBCの合併を念頭に入れていく必要があると結論された.
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Analysis of two major anti-M2 antibodies (anti-PDC-E2/anti-BCOADC-E2) in primary biliary cirrhosis: relationship to titers of immunofluorescent Anti-Mitochondrial Antibody.
Hepatology Research, 2000Co-Authors: Hiroshi Miyakawa, Naomi Kawaguchi, Kazuhiro Abe, K Kikuchi, Eriko Kitazawa, Hirotoshi Fujikawa, Makoto KakoAbstract:To analyze anti-M2 components in primary biliary cirrhosis (PBC) we measured two major anti-M2 antibodies (anti-PDC-E2 and anti-BCOADC-E2) by immunoblotting and ELISA, and compared the results between 38 immunofluorescent Anti-Mitochondrial Antibody (AMA)-negative PBC patients (group A) and 39 strongly AMA-positive PBC patients (group B) with titers of 1:640. Using bovine heart mitochondrial fraction as antigen, the immunoblot positivity rate of anti-PDC-E2 in group B was significantly higher than that in group A, whereas the positivity rate of anti-BCOADC-E2 was not significantly different between the two groups. This result was similar to that obtained by ELISA using recombinant fusion proteins. In group A there was a significant inverse correlation between ELISA optical density values of anti-PDC-E2 and of anti-BCOADC-E2, but in group B there was no correlation between the two values. Only three patients from group A and 21 from group B were positive for both antibodies. Taken together these results appear to indicate that the detection of anti-BCOADC-E2 is critical for the accurate serological diagnosis of AMA-negative PBC patients. The detection of anti-BCOADC-E2 may also help to distinguish between AMA-negative PBC and autoimmune cholangitis patients.
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Western blot analysis of anti-M2 antibodies in Anti-Mitochondrial Antibody-negative primary biliary cirrhosis
Rinsho byori. The Japanese journal of clinical pathology, 1997Co-Authors: Naomi Kawaguchi, Minoru Shibata, Hiroshi Miyakawa, Kazuhiro Abe, Kitazawa E, Fujikawa H, K Kikuchi, Makoto Kako, Akitaka Shibuya, Yasushi OnozukaAbstract:One variety of Anti-Mitochondrial Antibody(AMA) is characteristically found in sera from patients with primary biliary cirrhosis(PBC). The major target antigens of this type of AMA are M2s. It is well known, however, that AMA-negative PBC also exists. An alternative disease concept, called autoimmune cholangiopathy, recently has been advocated. This new concept is defined by the following criteria: 1)the failure to detect AMA and anti-M2, 2)the detection of a diffuse type of anti-nuclear Antibody and anti-smooth muscle Antibody, 3)pathological findings compatible with PBC, and 4)the effectiveness of prednisolone. However, the difference between AMA-negative PBC and autoimmune cholangiopathy is controversial. Therefore, we analyzed antibodies to four major M2 proteins with Western blotting in 34 cases of immunofluorescent AMA-negative PBC. In 31(91.2%) of these 34 AMA-negative sera, antibodies to at least one of these four major M2 proteins was detected. In serum samples from 34 control patients with AMA-positive PBC, antibodies to at least one of these four proteins were detected in all cases. In addition, we studied the frequency of cases which satisfied the serological criteria of autoimmune cholangiopathy. In only one(0.7%) of 141 cases was the serological criteria met. We conclude that to clarify the serological differences between autoimmune cholangiopathy and AMA-negative PBC, the analysis of M2 proteins by Western blotting is essential.