• The Korean Journal of Nuclear Medicine
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• v.16 no.1
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• pp.15-22
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• 1982

To evaluate the diagnostic usefulness of radioisotope bone scan and clinical activity of myositis, bone scan using $^{99m}Tc-Methylene$ diphosphonate was serially done before and after treatment with prednisolone in 10 patients with well-documented dermatomyositis-polymyositis. The observed results were as follows. 1. In all 10 patients before treatment with prednisolone, the bone scans showed evidence of increased muscle uptake. Muscle uptake was markedly increased in 4 patients, moderately increased in 3 patients and minimally increased in 3 patients. 2. The site of increased muscle uptake was consistent with the site of clinically involved muscle which was weak and tender. 3. The degree of muscle uptake correlated with the severity of the muscle weakness and tenderness at the scan was done. In all 10 patients treated with high dose prednisolone, muscle uptake was decreased following therapy. Above results suggest the radioisotope bone scanning may be useful in the diagnosis and treatment of patient with dermatomyositis-polymyositis.

• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.1-6
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• 2002

The inflammatory myopathies are divided into three major and distinct subsets as polymyositis(PM), dermatomyositis(DM), and inclusion body myositis(IBM). This distinction is based on unique clinical, demographic, laboratory, histologic, therapeutic, prognostic, and immunopathologic criteria. Although the causes of PM, DM, and IBM are unknown, autoimmune mechanisms are implicated, as supported by their association with other putative or definite autoimmune diseases or viruses, the evidence for a T cell-mediated myocytotoxicity or complement-mediated microangiopathy, the presence of various autoantibodies and their response to immunotherapies. But in IBM the immune-mediated process is weaker and IBM patients do not readily respond to immunotherapies, there are convincing immunopathological signs to suggest that a definite autoimmune component, similar to that seen in PM, also plays a role in the cause of IBM.

• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.7-11
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• 2002

Among drug-induced myopathy, steroids are probably the most common cause. The risk of steroid myopathy(SM) increases with the dose and duration of use. It is typically a proximal myopathy, preferentially affecting the hip girdle muscles. Motor and sensory nerve conduction studies are normal. The needle EMG is usually within the normal range or may be minimally abnormal. Occasionally, low-amplitude, short-duration MUAPs may be seen in the proximal muscles. Of note, abnormal spontaneous activity is not seen. This point is often very useful in differentiating polymyositis(PM) from SM. It is common for patients with PM to be treated with steroids, respond well, and then have the steroids tapered. If muscle weakness then returns, it may be very difficult to differentiate recurrent PM from SM on clinical grounds. The presence of abundant abnormal spontaneous activity strongly suggests PM rather than SM.

• Annals of Clinical Neurophysiology
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• v.9 no.1
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• pp.11-15
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• 2007

Background: The term "overlap syndromes" designates a group of diseases in which polymyositis (PM) or dermatomyositis (DM) is associated with some other disorders of connective tissues. The aim of this study was to delineate the clinical features, laboratory findings, and outcome of treatment of "overlap syndromes" Methods: We analyzed the medical records of 16 patients (PM in 10, DM in 6) with well documented "overlap syndromes" between 1997 and 2004. The diagnosis was made when the criteria for two different disorders were fulfilled. Results: All patients were female. Age of onset ranged from 14 to 52 years (mean 29.8 years) with peak incidence in the third and fourth decades. Systemic lupus erythematosus (SLE) was associated in 10, systemic sclerosis in 7, and rheumatoid arthritis in 3 patients. Four of the patients had two different connective tissue diseases simultaneously. The characteristic clinical features were muscle weakness, arthralgia, Raynaud's phenomenon, and myalgia. In laboratory tests, creatine kinase (CK), lactic dehydrogenase (LDH), and transaminases were usually abnormal. Positive antinuclear antibody (ANA), rheumatoid factor (RF), and cryoglobulin were found in 100%, 69%, and 67% of the patients, respectively. Needle electromyography (EMG) showed abnormal findings compatible with myopathy in 15 patients. The pathology of muscle biopsy from 14 patients revealed findings compatible with inflammatory myopathy. Glucocorticoids were administered to 15 patients. The muscle strength improved in all the treated patients, which was well correlated with repeat CK level and EMG findings. Conclusions: The presence of autoantibodies such as ANA, RF, and cryoglobulin in patients with PM or DM highly suggests the possibility of an overlap syndromes. These syndromes reveal a strong female predominance. The myositis associated with them usually shows a good response to glucocorticoids treatment.

• Tuberculosis and Respiratory Diseases
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• v.40 no.2
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• pp.197-202
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• 1993

Polymyositis (PM) is a inflammatory connective tissue disease involving predominantly skeletal muscles, characterized by symmetrical, proximal muscle weakness, inflammation, and frequently, degeneration. Interstitial lung disease in association with PM occurs in 5~10% of cases and carries an especially grave prognosis. Although the cause of lung involvement in PM is not known, the underlying pathologic process in the lung is an immune mediated inflammation of alveolar structures, alveolitis. It is of interest, therefore, that cyclophosphamide, an immune modulating agent, has been reported to be effective in the treatment of PM. We report a case of corticosteroid resistant PM associated with interstitial lung disease, successfully treated with cyclophosphamide. A 37-year-old female was presented with 8 months duration of cough, exertional dyspnea, and muscle weakness. She had typical symptoms, physical findings, and elevated muscle enzyme levels in serum with characteristic findings of muscle biopsy. She also had typical interstitial lung disease pattern on chest X-ray and high resolution CT with restrictive pattern on pulmonary function test. The findings of transbronchial lung biopsy was compatible with interstitial lung disease. She failed to respond to corticosteroid initially. Subsequently steroids and cyclophosphamide were given with excellent clinical improvement.

• Korean Journal of Pharmacognosy
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• v.39 no.4
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• pp.330-334
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• 2008

Shin Kwae Hwan(神快丸) is a Chinese patent medicine which has been used for polymyositis, trigeminal neuralgiaand ischiodynia in Korea. It consists of 15 kinds of powdered crude drugs. In powered crude drugs, it is hard to identify each component by chemical analysis or morphological examination. However, the method of identification of powdered crude drugs has not been clearly established. For the identification of individual crude drugs in such powdery mixtures, microscopic method may advantageously be used, as it requires only small amount of specimens. In this study, it is demonstrated that the microscopic method is very effective for the identification of 15 crude drug ingredients in Shin Kwae Hwan.

• Tuberculosis and Respiratory Diseases
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• v.79 no.3
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• pp.188-192
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• 2016

Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids.

• Annals of Clinical Neurophysiology
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• v.8 no.2
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• pp.196-198
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• 2006

It has been reported that antisynthetase syndrome belongs to the idiopathic myositis group which includes pulmonary interstitial disease, arthritis, Raynaud's phenomenon, and mechanic's hand, associated with the anti-Jo1 antibody. A 60- year-old man presented with one month history of lower limbs weakness, rapidly progressive exertional dyspnea, and arthralgia. A markedly increased titers of anti-Jo1 antibodies were found. Chest CT showed idiopathic pulmonary fibrosis. Muscle biopsies were consistent with polymyositis. A high dose corticosteroids and cyclosporine were not effective. We report a case of antisynthetase syndrome, in which immunosuppressive agents could not rescue the deteriorating disease course.

• Journal of the Korean Magnetic Resonance Society
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• v.20 no.2
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• pp.50-55
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• 2016

Backbone $^1H$, $^{13}C$ and $^{15}N$ resonance assignments are presented for the anticodon binding domain (residues 557-674) of human glycyl-tRNA synthetase (GRS). Role of the anticodon binding domain (ABD) of GRS as an anticancer ligand has recently been reported and its role in other diseases like Charcot-Marie-Tooth (CMT) and polymyositis have increased its interest. NMR assignments were completed using the isotope [$^{13}C/^{15}N$]-enriched protein and chemical shifts based secondary structure analysis with TALOS+ demonstrate similar secondary structure as reported in X-ray structure PDB 2ZT8, except some C-terminal residues. NMR signals from the N-terminal residues 557 to 571 and 590 to 614 showed very weak or no signals exhibiting dynamics or conformational exchange in NMR timescale.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.48-52
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• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.