• Tuberculosis and Respiratory Diseases
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• v.51 no.4
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• pp.354-363
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• 2001

Background : Although lung involvement has been reported in 5 to 46% of dermatomyositis/polymyositis(DM/PM) patients, reports of the condition in Korea are rare. This study evaluated the clinical features of lung involvement in DM/PM patients. Methods : The medical records, laboratory results and radiologic findings of 79 DM/pM patients, who attended the Seoul National University Hospital (SNUH) between 1989 and 1999, were reviewed retrospectively. Results : A total 79 patients of whom 24 patients(33%) showed lung involvement, were enrolled in this study. More patients with lung involvement were female(F:M=11:1), and older compared with those without lung involvement. Patients with lung involvement presented with dyspnea(79%), coughing(67%), an elevated ESR, and CK/LD. Anti-Jo 1 antibody test was positive in 30%, which is significantly higher in patients with lung involvement. A simple chest X-ray of the patients with lung involvement exhibited reticular opacity(50%), reticulonodular opacity(30%), patchy opacity(29%), nodular opacity(13%) and linear opacity(4%). HRCT(n=24) showed ground glass opacity(75%), linear or septal thickening(50%), patchy consolidation(42%), honey-combing(33%) and nodular opacity(17%). The pulmonary function test showed a restrictive ventilatory pattern(77%) and a lower diffusing capacity(62%). The patients were followed up during a mean duration of $30{\pm}28$ months. They were treated with steroid only(50%) or a combination of steroids and cytotoxic agents(46%). Muscle symptoms were improved in 89% with treatment, but an improvement in the respiratory symptoms or in the pulmonary function test was rare. Patients with lung involvement had a higher mortality rate(21%) than those without lung involvement(10%) during the follow-up periods. Conclusion : DM/PM patients with lung involvement were mostly female, older and had a higher positive rate Anti-Jo 1 antibodies, but there was no significant difference in prognosis.

• 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.

• Journal of Veterinary Clinics
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• v.13 no.1
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• pp.81-86
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• 1996

Systemic lupus erythematosus in a dog, suspected systemic lupus erythematosus in a dog, and autoimmune thrombocytopenic purpura hemmorrhagica in a dog are reported. A fice-year old, female Chihuahua (Case 1) showed initially hemorrhagic diathesis and purpura hemorrhagica. Afterward, it showed polymyositis and polyarthritis. LE-cell was demonstrated on LE-cell preparation trom blood. Systemic lupus erythematosus was diagnosed. This reponded well to the immunosuppressants, but developed iatrogenic Cushing syndrome and steroid hepatopathy. A two-and-half-year old, male toy poodle (Case 2) had chief complaint of red urine. Occult blood test for the urine sediment. This did not respond at all to antibiotics and carbazochrome, which is one of systemic coagulants. LE-cell was demonstrated on LE-cell preparation from blood. This responded relatively well to immunosupressants such as prenisolone, azathioprine and cyclophosphamide. systemic lupus erythematosus is suspected. A nine-year-and-three-month old, female Maltese (Case 3), which had history of congestive heart failure and ovariohysterectomy showed purpura hemorrhagica in the skin of chest. This had severe thrombocytopenia and leukocytosis. As prednisolone was administered before immunological examination or demonstration of LE-cell, it was impossible to diagnose whether purpura hemorrhagica developed as a member of systemic lupus erythematosus or independent of systemic lupus erythematosus. This responded well to prednisolone, and so autoimmune thrombocytopenic purpura hemorrhagica was diagnosed.

• Journal of Yeungnam Medical Science
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• v.25 no.2
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• pp.117-123
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• 2008

Dermatomyositis is characterized by progressive, symmetric, proximal muscle weakness and a nonsuppurative inflammatory myopathy of unknown etiology involving predominantly skeletal muscles. It is also characterized by typical skin lesions. Interstitial lung disease has a poor prognosis when it is associated with dermatomyositis. Organizing pneumonia is a disease in which granulation tissue fills the lumina of terminal and respiratory bronchioles and extends into the distal airspaces. The cryptogenic nature of the process is appreciated in that organizing pneumonia patterns of injury can be seen in secondary forms of the disease (secondary organizing pneumonia). Organizing pneumonia has been reported to occur in 5~10% in dermatomyositis-polymyositis patients. Anti-histidyl tRNA synthetase antibody (anti-Jo-1) is a predictive disease marker that is reported to occur in up to 70% of patients. We describe a 49-year-old male dermatomyositis patient who presented with organizing pneumonia and was found to have negative anti-Jo-1 antibody.

• The Journal of Korean Physical Therapy
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• v.16 no.4
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• pp.23-37
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• 2004

Fibromyalgia syndrome(FMS) is a chronic pain disorder of unknown etiology characterized by widespread musculoskeletal aches and pains, stiffness, and general fatigue, disturbed sleep and sleepiness. Frequently misdiagnosed, FMS is often confused with myofascial pain syndrome, polymyalgia rheumatica, polymyositis, hypothyroidism, metastatic carcinoma, rheumatoid arthritis (RA), juvenile rheumatoid arthritis, chronic fatigue syndrome, or systemic lupus erythematosus, any of which may occur concomitantly with FMS. The management of FMS often begins with a thorough examination and a diagnosis from a physician who is formally trained in tender-point/trigger-point recognition. An initial diagnosis provides reassurance to the patient and often reduces the anxiety and depression patterns associated with FMS. The most common goals in the management of FMS are (1) to break the pain cycle, (2) to restore sleep patterns, and (3) to increase functional activity levels. Because FMS is a multifactorial syndrome, it is likely that the best treatment will encompass multiple strategies. Medication with analgesics and antidepressants and also physiotherapy, are often prescribed and give some relief. The other most effective intervention for long-term management of FS to date is physical exercise. Physical therapists can instruct patients in the use of heat at home (moist hot packs, heating pads, whirlpools, warm showers or baths, and hot pads) to increase local blood flow and to decrease muscle spasm and tension. Also instruct patients in the proper use of cold modalities (ice packs, ice massage, and cool baths) to anesthetize localized areas of pain (tender points) and break the pain cycle. Massage and tender-point massage also may promote muscle relaxation. To date, the two most important interventions for the long-term management of FS are patient education and physical exercise. Lately, is handling FMS and Chronic Fatigue syndrome(CFS) together, becuase FMS and CFS are poorly understood disorders that share similar demographic and clinical characteristics. Because of the clinical similarities between both disorders it was suggested that they share a common pathophysiological mechanism, namely, central nervous system dysfunction.

• 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.

• The Journal of Korean Physical Therapy
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• v.12 no.2
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• pp.255-265
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• 2000

The term 'myositis ossificans' encompasses four categories of clinicopathological disorders. The first, myositis ossificans progressive(fibrodysplasia ossificans progressive), is a rare genetic disease characterized by progressive heterotopic ossification involving skeletal muscle, tendon, ligaments, and fascia, with congenital malformation of the great toes, and usually microdactyly, monophalangism, and mal formed proximal phalanges. with valgus deformity of metatarsophalangeal joint. The ossification begins shortly after birth and may contribute to the patient's death. The second, heterotopic ossificans, can occur in patients with neuromuscular and chronic diseases such as paraplegia, poliomyelitis, polymyositis, bum, tetanus, and infection. But the lesions in these cases often lack the typical histologic features of myositis ossificans. The third, myositis ossificans traumatica, is the most common; it develops in response to soft tissue trauma such as a single severe injury, minor repetitive injures, fracture, joint dislocation, stab wound, or surgical incision. The forth, nontraumatic myositis ossificans, also designated :pseudomalignant osseous tumors of extraskeletal soft tissues' and 'psedomalignant myositis ossificans', occurs in persons repeated small mechanical injures or nonmechanical soft tissue injuries due to local ischemia, inflammation. or other factors cannot be ruled out in such cases.

• Tuberculosis and Respiratory Diseases
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• v.48 no.2
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• pp.236-245
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• 2000

Background: Transbronchial lung biopsy(TBLB) has known to yield useful information for pulmonary infiltrates of uncertain etiology, However, its safety and usefulness have not been conclusive in the critically ill patients with respiratory failure. Moreover, TBLB has not been recommended for patients with mechanical ventilation. This study was conducted to investigate the diagnostic values and risks of Will performed on critically ill patients at bedside to obtain information on the pulmonary infiltrate of unknown etiology. Methods: Twenty patients(21 admissions with 23 cases) with diffuse pulmonary infiltrates who were treated in a medical intensive care unit of a tertiary referral hospital from January 1994 to May 1998, were enrolled for the study. Their medical records were retrospectively reviewed. TBLB was opted when a noninvasive diagnostic work-up failed to reveal the cause for the pulmonary infiltrate. The procedure was performed at patients' bedside without assistance of fluoroscopy. Bronchial washing or bronchoalveolar lavage was performed on the same pulmonary segment before performing TBLB. Results: Adequate specimens were obtained in 18 cases(78%). TBLB provided a specific diagnosis in two cases. The results of TBLB suggested the underlying etiology in 9 cases; bacterial pneumonitis(4), hypersensitivity pneumonitis(1), polymyositis(1), radiation fibrosis(1), idiopathic pulmonary fibrosis(1), and BOOP(1). Therapeutic decisions were altered in 11 cases(47.8%) based on the TBLB results. Pneumocystis carinii was found in the BAL fluid of another case. Ten patients with a therapeutic change and ten patients without a management change had mortality rates of 40% and 80%, respectively. The APACHE III scores were significantly higher in patients with complications($72.8{\pm}21.8$) compared with those without complications ($48.3{\pm}18.9$)(p<0.05). The complication rates were higher in those with mechanical ventilation(50%) than in those without Mechanical ventilation(33%), but the difference was not statistically significant(p=0.3). Conclusions: TBLB may be a useful diagnostic option for critically ill patients with unknown cause of pulmonary infiltrates. However, it should be cautious be used with care for patients with mechanical ventilation or for severely ill patients.