• Title/Summary/Keyword: Dermatomyositis

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Subcutaneous tissue calcification in a patient with rheumatoid arthritis (류마티스 관절염 환자에서 발생한 피하조직 석회화)

  • Kim, Dong Hyun;Kim, Kyung Jin;Kwon, Sung Min;Cha, Sung Ouk;Lee, Jung Ouk
    • Journal of Yeungnam Medical Science
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    • v.33 no.2
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    • pp.120-124
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    • 2016
  • Subcutaneous tissue calcification in rheumatic diseases usually occurs in connective tissue diseases, such as systemic lupus erythematosus, scleroderma, and dermatomyositis. Domestic cases of calcification in rheumatoid arthritis have not been reported. The mechanism of subcutaneous tissue calcification may differ depending on the cause and it can develop on all parts of the body. Calcification occurring in rheumatic diseases is a major mechanism of tissue damage caused by chronic inflammation. No standard therapy for calcification has been established; however, many studies have reported on medical and surgical treatment. We report on subcutaneous tissue calcification in a rheumatoid arthritis patient tissue calcification on both sides of the buttocks, the upper limbs, and the lower limbs.

A Case of Nonspecific Interstitial Pneumonia Complicated with Spontaneous Pneumomediastinum, Subcutaneous Emphysema and Pneumatosis Interstinalis (비특이성 간질성 폐렴에서 발생한 자발성 종격동기종, 피하기종과 대장기종)

  • Park, Myung Jae
    • Tuberculosis and Respiratory Diseases
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    • v.64 no.2
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    • pp.138-143
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    • 2008
  • Pneumatosis intestinalis or spontaneous pneumomediastinum are rarely associated with nonspecific interstitial pneumonia (NSIP). However, the development of both conditions in the same patient simultaneously has not been reported previously. A 56-year-old man with NSIP developed spontaneous pneumomediastinum accompanied by subcutaneous emphysema and pneumatosis intestinalis after the treatment with intravenous high dose steroid. The development of spontaneous pneumomediastinum, subcutaneous emphysema and pneumatosis intestinalis in this patient was possibly due to the factors such as NSIP, high dose steroid therapy and subclinical dermatomyositis. Treatment with corticosteroid and cyclosporin gradually improved his exacerbated NSIP and pneumomediastinum, subcutaneous emphysema, pneumatosis intestinalis.

Sphingosine 1-Phosphate Receptor Modulators and Drug Discovery

  • Park, Soo-Jin;Im, Dong-Soon
    • Biomolecules & Therapeutics
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    • v.25 no.1
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    • pp.80-90
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    • 2017
  • Initial discovery on sphingosine 1-phosphate (S1P) as an intracellular second messenger was faced unexpectedly with roles of S1P as a first messenger, which subsequently resulted in cloning of its G protein-coupled receptors, $S1P_{1-5}$. The molecular identification of S1P receptors opened up a new avenue for pathophysiological research on this lipid mediator. Cellular and molecular in vitro studies and in vivo studies on gene deficient mice have elucidated cellular signaling pathways and the pathophysiological meanings of S1P receptors. Another unexpected finding that fingolimod (FTY720) modulates S1P receptors accelerated drug discovery in this field. Fingolimod was approved as a first-in-class, orally active drug for relapsing multiple sclerosis in 2010, and its applications in other disease conditions are currently under clinical trials. In addition, more selective S1P receptor modulators with better pharmacokinetic profiles and fewer side effects are under development. Some of them are being clinically tested in the contexts of multiple sclerosis and other autoimmune and inflammatory disorders, such as, psoriasis, Crohn's disease, ulcerative colitis, polymyositis, dermatomyositis, liver failure, renal failure, acute stroke, and transplant rejection. In this review, the authors discuss the state of the art regarding the status of drug discovery efforts targeting S1P receptors and place emphasis on potential clinical applications.

Extraskeletal Osteosarcoma Around the Knee Joint - A Case Report - (슬관절 주위에 발생한 연부조직 골육종 - 증례 보고 -)

  • Lee, Bong-Jin;Kim, Tae-Ho;Ha, Chang-Won;Kim, Sung-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.15 no.1
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    • pp.81-86
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    • 2009
  • An extraskeletal osteosarcoma is a rare malignancy. A small number of cases and studies have been reported in the world and only two cases have been reported in Korea. We experienced an extraskeletal osteosarcoma around the knee joint of 91-year-old male who was the oldest case in the literatures. It was developed without history of trauma, irradiation, myositis ossificans, and heterotopic ossification of dermatomyositis. This patient was treated with excision alone, however he was alive and there were no sign of local recurrence or distant metastasis and functional loss during 1-year follow-up.

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Diffuse Alveolar Damage Associated with Polymyositis (다발성 근염에 동반된 Diffuse Alveolar Damage 1예)

  • Park, Tae-Eung;Lee, Se-Young;Lee, Kwang-Hi;Jung, Sung-Hwan;Uh, Soo-Taek;Lim, Kun-Il;Park, Choon-Sik;Jin, Byung-Won;Park, Jai-Sung;Choi, Deuk-Lin;Jin, So-Young
    • Tuberculosis and Respiratory Diseases
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    • v.42 no.3
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    • pp.400-406
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    • 1995
  • Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis (PM-DM), or may occur later in the evolution of disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. We experienced a case of polymyositis associated with diffuse alveolar damage(DAD) that was proven in open lung biopsy. The patient was a 52 year-old woman who was presented with 6 months' duration of generalized ache, edema on ankle and wrist, non-productive cough and mild dyspnea. She had typical symptoms and physical findings of interstitial pneuminitis, and elevated muscle enzyme levels in serum with characteristic histologic findings of myositis on muscle biopsy. She also had typical interstitial lung disease pattern on high resolution CT and restrictive pattern on pulmonary function tests. The findings of open lung biopsy was compatible with diffuse alveolar damage(DAD). She failed to respond to the therapeutic trials with corticosteroid and cyclophosphamide, and finally expired due to acute respiratory distress syndrome.

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A case of mixed connective tissue disease presenting initially with Raynaud's phenomenon (레이노드 증후군으로 초기 발현된 복합 교원성 질환 1예)

  • Kim, Soo Young;Choi, Young Seok;Kim, Young Ok;Woo, Young Jong
    • Clinical and Experimental Pediatrics
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    • v.51 no.8
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    • pp.886-891
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    • 2008
  • Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report a rare childhood case of MCTD in a 7-year-old girl presenting initially with Raynaud's phenomenon, swollen hands, and ulceration of the right index finger tip followed by alopecia and arthritis during follow-up.

A Case of Bronchiolitis Obliterans Organizing Pneumonia anteceded by Rheumatoid Arthritis (류마티스 관절염에서 동반된 폐쇄성 세기관지염.간질성 폐염 1예)

  • Kim, Sin Gon;Kim, Jin Yong;Lee, Young Ho;Cho, Jae Youn;Kim, Han Kyeom;Song, Gwan Gyu
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.4
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    • pp.630-636
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    • 1996
  • Bronchiolitis obliterans organizing pneumonia(BOOP) is a pathologic entity characterized by the formation of plugs of fibrous tissue in bronchioles and alveolar ducts. It clad been described in association with several connective tissue diseases, i.e. systemic lupus erythematosus, Behcet's disease, polymyositis-dermatomyositis, polymyalgia rheumatica and Sjogren's syndrome. Recently, there were few additional reports on BOOP, anteceded by rheumatoid arthritis. We clad experienced a case of 60 years old female patient who clad been admitted for acute respiratory difficulty and abnormal chest roentgenographic findings. She was diagnosed as having rhematoid arthritis 20 years ago. On the course of our treatment, she did not respond to antibiotics. Moreover, no microorganism grew in the sputum and blood. Thus, the bronchoscopic biopsy was done. The patient showed a dramatic response to steroid therapy, and the pathologic bindings of the bronchoscopic biopsy confirmed as BOOP. To our knowledge, this is the first reported case of BOOP anteceded by rheumatoid arthritis in Korea.

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A Case of Disseminated Mycobacterium intracellulare Infection in an Immunocompromised Host (면역 저하 환자에서 발생한 파종성 Mycobacterium intracellulare 감염 1예)

  • Kim, Sun Young;Oh, Dong Wook;Yu, Ji Hee;Kim, Donghoi;Noh, Sehui;Roh, JaeHyung;Jung, Sang-Su;Yoo, Dong-Jun;Shim, Tae Sun
    • Tuberculosis and Respiratory Diseases
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    • v.67 no.1
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    • pp.32-36
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    • 2009
  • We report a case of disseminated Mycobacterium intracellulare infection in a 31-year-old man who had been diagnosed as having dermatomyositis and systemic lupus erythematosus 3-years prior. The patient developed a left pleural effusion M. intracellulare was repeatedly isolated from the pleural fluid. After antimycobacterial treatment, the patient's pleural effusion resolved, but a left knee joint effusion developed newly and M. intracellulare was cultured from the joint fluid. At present, the patient has been taking antimycobacterial medication for 15 months but his left knee joint fluid remains positive for M. intracellulare. To our knowledge, this is the second reported case of disseminated NTM infection in a non-HIV infected patient in Korea.