The major presenting manifestations of systemic lupus erythematosus (SLE) in Korea are known to be cutaneous manifestations, arthritis, nephritis, and fever. The ascites due to peritoneal involvement in SLE is quite common. However, it is unusual for massive ascites to be major presenting manifestations of SLE. In this report, we describe a case of SLE patient whose disease manifested as intractable ascites. This illustrates an unusual presentation and natural history of a complex autoimmune disease.
Arthritis ; Ascites* ; Autoimmune Diseases ; Fever ; Humans ; Korea ; Lupus Erythematosus, Systemic* ; Natural History ; Nephritis

Systemic lupus erythematosus (SLE) is a multisystemic disease that can involve the gastrointestinal tract, liver, and biliary system. Symptomatic pancreatic involvement, however, has rarely been reported. It may be part of the primary disease process, such as vasculitic or autoimmune etiology, or associated with drug therapy, in particular corticosteroid. We report here a lupus patient who developed severe pancreatitis within 30 hours of initiation of corticosteroid therapy; we also discuss the relation between pancreatitis and systemic lupus erythematosus.
Biliary Tract ; Drug Therapy ; Gastrointestinal Tract ; Humans ; Liver ; Lupus Erythematosus, Systemic* ; Pancreatitis*

The coronary arteries are also subject to congenital anomalies of both minor and major consequence. Hemodynamically significant primary anomalies of the coronary arteries are those which alter myocardial perfusion. There are four major types : coronary artery fistula, origin of the left voronary artery from the pulmonary artery, congenital coronary stenosis or atresia and origin of the left or right coronary artery from the opposite sinus of Valsalva with subsequent passage of the vessel between the aorta and right ventricular infundibulum. Minor anomlies are the variation of the origin of the coronary arteries from the aorta with normal distal circulation. We can see more coronary anomalies associated with congenital cardiac anomalies, probably representing a circulatory response to the primary intracardiac defect. We present a total of 76 cases of congenital coronary anomalies, reviewing 3946 cases of angiocardiography or selective coronary arteriography, performed during last 8 years at Sejong General Hospital.
Angiocardiography ; Angiography ; Aorta ; Arteries ; Coronary Stenosis ; Coronary Vessels* ; Fistula ; Hospitals, General ; Perfusion ; Pulmonary Artery ; Sinus of Valsalva

No abstract available.
Polychondritis, Relapsing*

We investigate the clinical characteristics of Sjogren's syndrome (SS). METHODS: We. retrospectively reviewed 59 patients with SS in the period from January 19: To 86 to January 1997. European Epidemiology Center criteria was used to diagnose SS. OBJECTIVES AND RESULTS: Thirty-one primary SS and 28 secondary SS patients were included in this study. There were 57 females and 2 males, the mean age was 45. 2+12.3 years (24 to 70 years). Patients with secondary SS were associated with rheumatoid arthritis(n=10), systemic lupus erythematosus(n=7), scleroderma(n=5), mixed connective tissue disease(n=3), and myositis(n=3). The common presenting symptoms were dry mouth in 92% and dry eyes in 90% of patients. Other symptom and signs included fatigue(27%), lymphadenopathy(22%) and parotid gland swelling (10%). The commonly presented autoantibodies were antinuclear antibody(83%) and rheumatoid factor(57%). Anti-Ro was positive in 49%(25/51) and anti-La in 37%(19/51). Anti-La positivity was significantly higher in primary SS(52%) compared to secondary SS(21%) (p<0.05). Schirmer test was positive in 98% (44/45), salivary radioscintigraphy in 74%(25/34), and lower lip biopsy in 47%(16/34). Extraglandular manifestations, such as Raynaud's phenomenon, vasculitis, glomerulonephritis, vaginal dryness, renal tubular acidosis, and primary biliary cirrhosis, were more frequently presented in primary SS compared to secondary SS (p<0. 05). CONCLUSION: The sex, age, and clinical manifestation of primary SS were similar with secondary SS, but extraglandular manifestations and anti-La antibody positivity were more frequent in primary SS.
Acidosis, Renal Tubular ; Autoantibodies ; Biopsy ; Connective Tissue ; Diagnostic Tests, Routine ; Epidemiology ; Female ; Glomerulonephritis ; Humans ; Lip ; Liver Cirrhosis, Biliary ; Male ; Mouth ; Parotid Gland ; Retrospective Studies ; Sjogren's Syndrome* ; Vasculitis

No abstract available.
Tongue Neoplasms* ; Tongue*

Isolated and severe left main coronary ostial stenosis is a rare case. In the majority of these patients ostial stenosis was associated with any of the conditions known to involve the coronary ostia. These conditions include syphilitic aortitis, Takayasu's aortitis, familial hypercholesterolemia, and aortic valve disease. A 34-year young female patient was presented with exertional and stabbing anterior chest pain. There was no history of syphilis, diabetes mellitus, hypertension, hyperlipidemia and smoking. Coronary angiogram showed isolated left main coronary ostial stenosis. Transesophageal echocardiography(TEE) showed acute angle takeoff of the left main coronary artery. She underwent surgical angioplasty of coronary ostia with a patch of autologous pericardium. After angioplasty, TEE showed dilatation of left main coronary ostium and her clinical symptom improved.
Angioplasty ; Aortic Valve ; Aortitis ; Chest Pain ; Constriction, Pathologic* ; Coronary Vessels ; Diabetes Mellitus ; Dilatation ; Female ; Humans ; Hyperlipidemias ; Hyperlipoproteinemia Type II ; Hypertension ; Pericardium ; Smoke ; Smoking ; Syphilis ; Syphilis, Cardiovascular

The purpose of this study was to determine the factors which were responsible for delaying early diagnosis and optimal management of rheumatoid arthritis (RA) in Korea. We interviewed 109 outpatients diagnosed as RA being treated by rheumatologists, and we eventually analyzed 98 patients' data. The median length of time from symptom onset to the first visit to a medical doctor, to diagnosis, and visiting a rheumatologist were 8 weeks, 23 weeks, and 42 months respectively. The subspecialist with whom the patients consulted with for the longest time before visiting a rheumatologist were an orthopaedic surgeon for 51 patients, a Chinese herbal doctor for 19 patients, and a pharmacist for 16 patients. For early diagnosis and optimal management of RA in Korea, we believe that it is necessary to reduce the use of unconventional medical services such as Chinese herbal medicine and nonprescribed medication, and to emphasize rheumatologic and rehabilitative care in the early stage.
Adult ; Alternative Medicine/utilization ; Arthritis, Rheumatoid/therapy* ; Arthritis, Rheumatoid/rehabilitation ; Female ; Health Services/utilization* ; Human ; Korea ; Male ; Middle Age ; Rheumatology/methods

BACKGROUND: The Fas/Fas ligand (FasL) system plays an important role in apoptosis by involvement in various immunologic functions, especially the removal of autoreactive and activated T-cells. sFas is a variant of the Fas receptor molecule, which lacks the transmembrane domain by alternative splicing of Fas mRNA and has an inhibitory effect in apoptosis by inhibition of the Fas/FasL pathway. sFasL is a coverted form of FasL by metalloproteinase and is increased in various malignant and autoimmune diseases. In this study, we investigated the expression of sFas and sFasL in systemic lupus erythematosus (SLE) and evaluated their usefulness as markers of disease activity. MATERIALS AND METHODS: The concentration of sFas and sFasL in sera from 43 patients with SLE, 17 with rheumatoid arthritis (RA) and 15 normal healthy persons were measured using sFas (S) ELISA Kit and sFas Ligand ELISA Kit (MBL Co., LTD., Nagoya, Japan), respectively. Twenty of 43 SLE sera were paired samples of 10 patients obtained on admission and discharge. RESULTS: The concentration of sFas in SLE (3.12 +/- 2.28 ng/mL) was significantly higher than in RA (2.23 +/- 0.37 ng/mL) and in the normal control (2.12 +/- 0.33 ng/mL). In particular, the concentration of sFas in sera on admission (4.35 +/- 3.68 ng/mL) was significantly higher than in the sera on discharge (2.89 +/- 0.66 ng/mL), but, the concentration of sFasL among the 3 groups was not statistically different. CONCLUSIONS: These results suggest that apoptosis is involved in the pathogenesis of SLE and sFas might be a useful marker as a predictor of disease activity. Further study on the correlation between sFas and other disease activity markers, such as CRP, CH50, CD4 cell count and autoantibody titer is needed. Also, the evalution of sFas as a predictor of disease progression on follow-up studies of these patients is needed.
Alternative Splicing ; Antigens, CD95 ; Apoptosis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; CD4 Lymphocyte Count ; Disease Progression ; Enzyme-Linked Immunosorbent Assay ; Fas Ligand Protein* ; Follow-Up Studies ; Humans ; Lupus Erythematosus, Systemic* ; RNA, Messenger ; T-Lymphocytes

No abstract available.
Animals ; Congenital Abnormalities* ; Rats*