• Title/Summary/Keyword: Systemic Sclerosis

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Tc-99m-MIBI Uptake in Active Pulmonary Tuberculosis : A Case Report (활동성 폐 결핵에서의 Tc-99m-MIBI 섭취 : 1예 보고)

  • Lee, Chang-Ho;Park, Chan-Hee;Hwang, Hee-Sung;Bae, Moon-Sun
    • The Korean Journal of Nuclear Medicine
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    • v.30 no.3
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    • pp.379-381
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    • 1996
  • Technetium-99m MIBI was developed as a myocardiac perfusion imagine agent and has been used effectively in the detection and post-therapeutic evaluation of various neoplasm such as thyroid, lung, bone and breast tumors. As an infrequent findings, Tc-99m MIBI agent has shown in non-neoplastic pulmonary conditions Including fibrosing alveolitis, pulmonary actinomycosis, active pulmonary sarcoidosis, pulmonary interstitial fibrosis in progressive systemic sclerosis and active osteomyelitis. In a recent report conducted by Cetin Oncel, Tc-99m MIBI imaging is an effective method in the detection and follow-up of pulmonary tuberculosis We have also experienced Tc-99m MIBI uptake in active pulmonary tuberculosis incidentally found in a patient with suspected proliferative villonodular synovitis of the left ankle.

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A Case Report on Scars from Autoimmune Disorder Treated with Incisional Needle Therapy (절개침법을 적용한 자가면역질환 흉터 치료 1례)

  • Joo-Yeon Cho;Tae-Kyung Lim;Min-Hee Kim
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.37 no.1
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    • pp.107-112
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    • 2024
  • Objectives : This case report aims to demonstrate the effectiveness of incisional needle therapy in treating facial scars resulting from autoimmune disorder. Methods : A single case of a 29-year-old female patient with facial scars due to systemic sclerosis was treated with incisional needle therapy. The treatment was applied over 14 sessions from February to September 2023. The efficacy of the treatment was evaluated using clinical photographs and the Physician's Global Assessment(PGA). Results : The treatment significantly improved scar appearance and reduced blood vessel visibility. The PGA scores, with an average of 2.66, indicating moderate improvement. Conclusions : Incisional needle therapy demonstrated potential as an effective treatment for scars caused by autoimmune disorders, due to minimal side effects and non-interference with ongoing treatments. It offers a promising alternative to conventional treatments, suggesting a need for further research for wider applications.

Rational Diagnosis and Management of Sjogren's Syndrome (쉐그렌 증후군의 합리적 진단 및 관리)

  • Tae, Il-Ho;Kwon, Jeong-Seung;Jeon, Young-Mi;Choi, Jong-Hoon;Shim, Woo-Hyun;Ahn, Hyung-Joon
    • Journal of Oral Medicine and Pain
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    • v.32 no.4
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    • pp.397-402
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    • 2007
  • Sjogren's syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Primary SS presents alone as xerostomia, keratoconjunctivitis sicca and patotid gland enlargement, secondary SS occurs in connection with other autoimmune disorder such as rheumatitoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. Among many other systemic complication, lymphoma is considered seriously. Patient with SS had a 44 times higher relative risk of lymphoma, and clinically identifiable lymphoma occurs in approximately 5% of patients with SS. So, patients with SS should be closely monitored. In dental office, diagnosis of SS is important in view of high risk of lymphoma. When a dentist diagnose and manage dry mouth, he or she should consider possibility of SS all the times and have knowledge of diagnostic criteria of SS.

Prognostic Value and Histologic Correlation of Sonography in Childhood Nephrotic Syndrome (신증후군 환아에서 신장초음파의 예후적 가치 및 조직검사와의 상관관계에 대한 연구)

  • Cheon Hae-Won;Yoo Kee-Hwan;Hong Young-Sook
    • Childhood Kidney Diseases
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    • v.2 no.1
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    • pp.26-33
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    • 1998
  • This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)

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A Case of Hodgkin's Disease as Endobronchial Presentation (기관지내 종양으로 발견된 호지킨병 1예)

  • Lee, Hyo-Jin;Cheon, Du-Su;Yun, Sang-Myung;Park, Sam-Seok;Ryu, Ki-Chan;Lee, Min-Ki;Lee, Chang-Hun;Park, Soon-Kew
    • Tuberculosis and Respiratory Diseases
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    • v.45 no.3
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    • pp.614-618
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    • 1998
  • Although mediastinal involvement by Hodgkin's disease is frequent, the initial presentation of the lymphoma by an endobronchial lesion is rare. A 23-year-old man was admitted with progressive dyspnea. Initial chest roentgenogram showed atelectasis of right middle and lower lobe. Fiberoptic bronchoscopy showed a polypoid mass obstructing the right mainstem bronchus. The mass lesion was histologically confirmed as Hodgkin's disease, nodular sclerosis type. He received systemic chemotherapy and improved rapidly.

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Overlap Syndromes in Polymyositis and Dermatomyositis (다발근육염과 피부근육염에서 관찰된 중첩증후군)

  • Park, Kyung Seok;Kim, Nam-Hee;Hong, Yoon-Ho;Sung, Jung-Joon;Nam, Hyunwoo;Park, Seong-Ho;Lee, Kwang-Woo
    • 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.

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A Case of Erdheim-Chester Disease Who Has Policythemia Vera (진성 적혈구증다증 환자에서 발현한 Erdheim-Chester Disease 1예)

  • Kim, Ji Eun;Lee, Hyun Jeong;Rhee, Chin Kook;Yoon, Hyung Kyu;Song, Jeong Sup
    • Tuberculosis and Respiratory Diseases
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    • v.64 no.3
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    • pp.224-229
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    • 2008
  • Erdheim-Chester disease (ECD) is a rare disease that is characterized by multi-organ involvement of foamy histiocytes. It causes systemic inflammation, and also demonstrates various clinical manifestations and has a poor prognosis. We encountered a case of ECD in a patient that had been treated for underlying polycythemia vera. As far as we know, this is the first reported case worldwide where ECD developed in association with polycythemia vera. A 59-year-old man visited our hospital due to pleuric pain at the right side of the chest. Pleural tissue that was obtained following a thoracoscopic biopsy showed non-Langerhan's cell histiocytosis, suggesting the presence of ECD. The histiocytes stained positively for CD68, but were negative for S-100 and CD1a. The patient also complained of pain at both hips and the right shoulder area. An X-ray and magnetic resonance image demonstrated that the lesion showed sclerosis and osteolysis in both the proximal femur and right humerus. Treatment was started with predinisolone, and subsequently cyclophosphamide was added. ECD is a very rare multi-systemic disease, and its cause and therapeutic options have not yet been defined. ECD has a poor prognosis. Therefore, we believe that additional case studies are needed prior to the determination of a novel therapy for ECD.

The Etiology of the Diffuse Infiltrative Disease of the Lung in Korea (한국에서의 미만성 침윤성 폐질환의 원인별 분포)

  • Kim, J.H.;Choi, S.J.;Kim, D.S.;You, J.H.;Kang, H.M.;You, S.H.;Jo, D.I.;Kim, J.W.;Han, S.K.;Kim, K.Y.;Kim, Y.S.;Park, C.S.;Kim, W.S.;Kim, W.D.;Jang, J.;Lee, W.Y.;Choi, B.H.;Hue, S.H.;Shin, D.H.;Lee, J.H.
    • Tuberculosis and Respiratory Diseases
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    • v.38 no.1
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    • pp.1-7
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    • 1991
  • To study the frequency of the underlying disease of the diffuse pulmonary infiltrates in korea, we ansalyzed retrospectively 982 patients who were seen at nine university hospitals and one general hospital in Seoul area. The results are folloing: 1) Among the total 982 patients, 490 patients were male and 492 patients were female. The mean age was 44.3 years. 2) The most common etiology was milliary tuberculosis (38%), which was followed by, idopathic pulmonary fibrosis (27%), pulmonary fibrosis associated with collagen-vascular disease (15%), and diffuse pulmonary infiltrates by malignancy (10%). 3) Amon the connective tissue disease which was accompanied by the interstitial lung disease, rheumatoid arthritis was the most common disease (43%), systemic lupus erythematosus was the 2nd (28%), and progressive systemic sclerosis was the 3re (16%). 4) Among 101 cases of malignant disease, lung was the most frequent primary site (31%), which was followed by stomach (28%), thyroid (16%), and breast (6%). 5) For the diagnosis of the underlying disease of pulmonary infiltrates, the transbronchial lung biopsy was performed in 21% of the patients and open lung biopsy was done in 7%.

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Study on the Clonality of Endothelial Cell Proliferation in Plexiform Lesions in Patients with Pulmonary Hypertension Associated with CREST Syndrome (CREST 증후군에 동반된 폐고혈압 환자에서 총상병변내 내피세포 증식의 클론성에 관한 연구)

  • Lee, Sang-Do;Jeon, Yong-Gam;Lee, Ji-Hyun;Shim, Tae-Sun;Lim, Chae-Man;Koh, Yun-Suck;Kim, Woo-Sung;Kim, Dong-Soon;Kim, Won-Dong;Tuder, Rubin M.
    • Tuberculosis and Respiratory Diseases
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    • v.47 no.2
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    • pp.150-160
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    • 1999
  • Background: The CREST syndrome is an indolent form of progressive systemic sclerosis. Although its clinical progress is indolent, pulmonary hypertension(PH) associated with CREST syndrome have grave prognosis with over 40 percent mortality rate at 2 year follow-up. But the pathogenesis of pulmonary hypertension in this disease is not known, and classified as either primary or secondary PH. Clonality of endothelial cell proliferation in plexiform lesion is a molecular marker which allows distinction between primary and secondary PH. We performed this study to know whether the PH associated with CREST syndrome is a variant of primary PH or is a secondary PH. Methods: We assessed the X-chromosome inactivation based on the methylation pattern of the human androgen-receptor gene by PCR(HUMARA). Endothelial cells in plexiform lesions from female patients(n=3) with PH associated with CREST syndrome were microdissected from paraffin blocks. Vascular smooth muscle cells and lung parenchyma were also microdissected for clonality studies. Results: The proliferating endothelial cells in 14 plexiform lesions were all polyclonal. Similarly proliferated smooth muscle cells from 5 vessels with medial hypertrophy were also polyclonal. Conclusion: These results suggest that the pulmonary hypertension associated with CREST syndrome has different pathogenesis from primary PH and to be classified as secondary PH.

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