No abstract available.
Herpes Zoster* ; Skin*

Primary antineutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV) constitute a group of small vessel vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome. Recently, many in vitro and in vivo studies have highlighted the role of ANCA as the main pathophysiological factor in the development of AAV. Two remarkable studies on ANCA pathogenesis were recently reported. One study examined anti-lysosomal membrane protein-2, which supports the 'shared epitope' theory. The other examined the neutrophil extracellular trap that is released by neutrophils primed by ANCA. Each disease of AAV shows a broad spectrum of the clinical features and severities, which makes it difficult to diagnose and treat them. Considerable effort has been made in the past decades to improve the treatment outcomes, reduce the incidence of relapse and avoid drug toxicity. This review describes the current understanding of AAV along with a few Korean reports.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Churg-Strauss Syndrome ; Drug Toxicity ; Glycosaminoglycans ; Incidence ; Membranes ; Microscopic Polyangiitis ; Neutrophils ; Recurrence ; Vasculitis ; Wegener Granulomatosis

Sarcoidosis can affect two or more members of the same family, and the reported occurrence of such familial sarcoidosis is variable from 0.5 to 14%. Recently we have experienced familial sarcoidosis affected mother and daughter, for the first time in Korea. Mother was diagnosed as Stage II sarcoidosis 4 years ago by transbronchial lung biopsy and cervical lymph node biopsy with compatible BAL finding in our hospital. This time, the daughter was admitted with bilateral hilar enlargement and anterior uveitis. Even though she had positive tuberculin skin test and atypical BAL finding(lymphocyte: 61%, CD4/CD8: 1.22). Transbronchial lung biopsy and mediastinal lymph node biopsy revealed noncaseating epithelioid granulorna without AFB. Slit lamp examination of the eyes showed severe anterior uveitis. Systemic steroid therapy was started due to progressive uveitis with antituberculous medication.
Biopsy ; Humans ; Korea* ; Lung ; Lymph Nodes ; Mothers ; Nuclear Family ; Sarcoidosis* ; Skin Tests ; Tuberculin ; Uveitis ; Uveitis, Anterior

In Korea, Resource-Based Relative Value Scale (RBRVS) was developed in 1997 and introduced in 2001 for the alternative of Korean Medical Fee Schedule. The RBRVS measures physician resource inputs to construct relative values for services and procedures. The RBRVS, as an administered price system, would need to be updated periodically. Changes in practice costs and practice patterns, and the rapid evolution of technology will require that relative values be adjusted over time. The Relative Value Scale Update Committee (RUC) in Korean Medical Association (KMA) is under updating annual review and 5-year review of Korean Relative Value Scale (RVS). The Korean RVS is estimated less balanced and rational in general, and furthermore there was no update after base-line study. So, it is the reason why this update of RVS is important. We, rheumatologists, are specialists for rheumatic diseases and are unfamiliar and unconcerned with health care system and medical insurance fee schedule, but this attitude is not appropriate to improve our specialized medical situation. This article reviewed the history of Korean medical insurance, development and update procedure of Korean RVS, and the current insurance problems in rheumatic diseases briefly. In Korea, RVS of medical services including rheumatology is relatively less compensated than the other invasive and imaging services. Therefore, Korean RVS update should be changed to more balanced and reasonable one for the medical service including rheumatology.
Appointments and Schedules ; Delivery of Health Care ; Fee Schedules ; Fees, Medical ; Insurance ; Korea ; Relative Value Scales* ; Rheumatic Diseases* ; Rheumatology ; Specialization

OBJECTIVE: This study is to determine the cross-cultural adaptation and validation of the Korean version of the EQ-5D (KEQ-5D) in various rheumatic patients. METHODS: We performed translation, backward translation and cognitive debriefing by the guidelines of the EuroQol group. The validity was evaluated by using the KEQ-5D, Korean Short- Form 36 (KSF-36) and disease-specific measures on 508 out-patients with ankylosing spondylitis, fibromyalgia syndrome, rheumatoid arthritis, osteoarthritis or systemic lupus erythematosus. Construct validity were evaluated by testing a-priori hypotheses of the association between the KEQ-5D and other measures. For the verification of reliability, we gave the same questionnaires twice to another 57 patients 1-week apart, and intra-class correlations and Kappa statistics were estimated. To test responsiveness, 60 patients with RA repeated the KEQ-5D at 12-week intervals within the context of 12-week clinical trials of a TNF blocker, and we calculated the t-test, effect size and standardized response mean RESULTS: There were no major modifications on the cross-cultural adaptation. The relationships between the KEQ-5D and the generic or disease-specific measures were observed to be as we expected previously in a-priori hypotheses:higher KEQ-5Dindex and KEQ-5DVAS scores were correlated with generic or disease-specific measures on the better health status, and groups with a better functional class showed higher KEQ-5D scores. The intra-class correlations of the KEQ-5Dindex and KEQ-5DVAS were 0.751 and 0.767, respectively, and Kappa statistics for agreement ranged from 0.455 to 0.772 across five dimensions. The standardized response means of KEQ-5Dindex and KEQ-5DVAS were 0.649 and 0.410. CONCLUSION: The KEQ-5D had good validity and sensitivity in several rheumatic conditions. Also, its reliability and responsiveness were moderate to good.
Arthritis, Rheumatoid ; Fibromyalgia ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Osteoarthritis ; Outpatients ; Quality of Life ; Surveys and Questionnaires ; Rheumatic Diseases* ; Spondylitis, Ankylosing

To determine whether angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism is associated with the development and clinical features of systemic sclerosis (SSc) in Korean, we studied seventy two Korean patients with SSc fulfilling the ACR preliminary classification criteria. The controls were 114 healthy, disease free Koreans. ACE I/D genotypes were determined by PCR method using oligonucleotides. Sixty eight patients (94.4%) were women and age at diagnosis was 43.5+/-12.6 yr old (mean+/-SD). Thirty nine patients (54.2%) had a diffuse type of SSc. There were no statistical differences in the frequencies of all ACE I/D genotypes and D allele between patients and controls, and neither between diffuse and limited types of SSc. ACE I/D gene polymorphism was not associated with the development of SSc in Korea. The investigation for the pathogenesis of SSc requires more studies about the role of other candidate genes such as endothelin, TGF-beta, nitric oxide, or angiotensin II receptor in addition to the ACE genes.
Scleroderma, Systemic/*enzymology/*genetics ; *Polymorphism, Genetic ; Peptidyl-Dipeptidase A/*genetics ; Middle Aged ; Male ; Korea ; Humans ; Genotype ; Gene Frequency ; Female ; DNA/genetics ; Case-Control Studies ; Base Sequence ; Alleles ; Adult

Retropharyngeal calcific tendinitis is defined as inflammation of the longus colli muscle and is caused by the deposition of calcium hydroxyapatite crystals, which usually involves the superior oblique fibers of the longus colli muscle from C1-3. Diagnosis is usually made by detecting amorphous calcification and prevertebral soft tissue swelling on radiograph, CT or MRI. In this report, we introduce a case of this disease which was misdiagnosed as a retropharyngeal tuberculous abscess, or a muscle strain of the ongus colli muscle. No calcifications were visible along the vertical fibers of the longus colli muscle. The lesion was located anterior to the C4-5 disc, in a rheumatoid arthritis patient with atlantoaxial subluxation. Calcific tendinitis of the longus colli muscle at this location in a rheumatoid arthritis patient has not been reported in the English literature.
Adult ; Arthritis, Rheumatoid/*complications ; Atlanto-Axial Joint/*physiopathology ; Calcinosis/*complications/*diagnosis ; Diagnosis, Differential ; Dislocations/*complications ; Female ; Humans ; *Magnetic Resonance Imaging ; Pharyngeal Diseases/*complications/*diagnosis ; Tendinopathy/*complications/*diagnosis

Infectious diseases, particularly pneumonia, remain a major cause of morbidity and mortality in patients with systemic lupus erythematous (SLE), accounting for 30~50% of all death. Because infection appears in various forms in lupus patients, early detection and appropriate treatment is very important. In the case of diffuse infiltration in the whole lung field, we should make a differential diagnosis with drug-induced pneumonitis, interstitial lung disease and pneumonia caused by atypical microorganism infection. In particular, in case of atypical microorganism pneumonia, we usually suspect cytomegalovirus, pneumocystis jiroveci and ebstein-barr virus, but influenza pneumonia is relatively neglected. Here, we report on the case of a 26-year-old female patient with lupus nephritis who presented with dyspnea and diffuse pulmonary infiltration immediately after intravenous cyclophosphamide. She was diagnosed with primary influenza B pneumonia and successfully treated with oseltamivir.
Accounting ; Adult ; Communicable Diseases ; Cyclophosphamide ; Cytomegalovirus ; Diagnosis, Differential ; Dyspnea ; Female ; Humans ; Influenza, Human ; Lung ; Lung Diseases ; Lung Diseases, Interstitial ; Lupus Nephritis ; Oseltamivir ; Pneumocystis jirovecii ; Pneumonia ; Viruses

Henoch-Sch nlein purpura is an acute, self-limited vasculitis syndrome which shows characteristic skin, joint, renal and gastrointestinal manifestations. It is common in childhood and may also occur in adults with fatal complications such as nephritis and gastrointestinal bleeding. We experienced a case of a 20-year-old woman who presented with palpable purpura and severe arthritis. The histopathologic examination of the skin revealed leukocytoclastic vasculitis with perivascular deposition of IgA and she was diagnosed with Henoch-Sch nlein purpura. Despite treatment with prednisolone for one month, she had more aggravated purpura and fatal gastrointestinal bleeding. The symptoms were improved shortly by cyclophosphamide pulse therapy with plasmapheresis but symptoms were aggravated and symmetric mononeuropathy of the ulnar nerve developed. She was treated with 400 mg/day of thalidomide and symptoms were improved. We herein report a case of Henoch-Sch nlein purpura successfully treated with thalidomide which was refractory to prednisolone, immunosuppressive drugs and plasmapheresis.
Adult ; Dermatologic Agents/*therapeutic use ; Female ; Human ; Purpura, Schoenlein-Henoch/*drug therapy ; Thalidomide/*therapeutic use

We report here a case of vascular Behcet's disease. 32-year-old young woman who suffered from chest pain was diagnosed with acute myocardial infarction four years before admission. At that time she manifested symtoms of Behcet's disease, such as oral ulcers, genital ulcers, multiple arthralgia, and a peculiar hyperirritability reaction to needle puncture. At this time she was admitted due to acute chest pain and intermittent abdominal pain. The diagnosis of an acute anteroseptal myocardial infarction was made on the basis of the electrocardiography findings. Cardiac catheterization revealed segmental stenosis up to 95% at the mid-left anterior descending artery without aneurysm formation at other coronary trees and there were no atherosclerotic changes. Coronary stent was inserted in the lesion. Abdominal CT showed inferior vena cava and right hepatic vein obstruction, which supports the diagnosis of Budd-Chiari syndrome. Our case demonstrated the followings which are not common to vasculo-Behcet disease; 1) initial clinicopathologic manifestations at the coronary artery, 2) abrupt total occlusion with clear cut lesion at left anterior descending artery, 3) recurrent myocardial infarctions, 4) Budd-Chiari syndrome, 5) combined superior mesenteric artery, inferior mesenteric artery and celiac trunk obstruction. Among the systemic manifestation of Behect's disease, cardiac involvement is very rare but should be considered as one of the most important features that influences the prognosis.
Abdominal Pain ; Adult ; Aneurysm ; Anterior Wall Myocardial Infarction ; Arteries ; Arthralgia ; Budd-Chiari Syndrome* ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Constriction, Pathologic ; Coronary Vessels ; Diagnosis ; Electrocardiography ; Female ; Heart Diseases ; Hepatic Veins ; Humans ; Mesenteric Artery, Inferior ; Mesenteric Artery, Superior ; Myocardial Infarction* ; Needles ; Oral Ulcer ; Prognosis ; Punctures ; Stents ; Tomography, X-Ray Computed ; Ulcer ; Vena Cava, Inferior