Induction of Fas Antigen Expression, and Apotopsis of Human B Cell by CD40 and Tumor Necrosis Factor Receptor Ligation / 대한류마티스학회지

OBJECTIVE: CD40 and the TNFR belong to the NGF/TNFR supergene family. Ligation of CD40 on B cells induces activation ignals leading to proliferation, Ig isotype class switching, germinal center formation but also induces Fas antigen expression.In addition,CD40 ligation induces pro-inflammatory cytokines including TNF-alpha and LT-alpha gene transcription by human B cell.TNF-alpha is a pleiotropic cytokine and also induces Fas antigen expression on various cells. Lately it has been known that TNF-alpha plays an important role in the pathogenesis of chronic inflammatory diseases,including rheumatoid arthritis,or chronic inflammatory bowel diseases.However there have been occurrence of autoantibodies,or autoimmune disease such as lupus after use of anti TNF-alpha blocking agents. In this report,we tested the relationship and biological significance of CD40 ligation and TNFR signaling with respect to Fas antigen expression on human B cells. METHODS: Ramos Burkitt's lymphoma B cell was used as a prototype of ger-minal center B lymphocyte,and R2G6 cell was utilized as a model of activated germinal center B cell.CD40 lgation was performed by the coculture with CD40 ligand bearing L-293 cells,or anti-CD40 monoclonal antibody,whereas control was obtained with CD-8-L-293 cells or control antibody.Expression of Fas antigen was determined with flow cytometer.Apoptosis assay was conducted by two ways.Alamar blue reduction assay after sIgM cross linking or anti-Fas anti-body,in the presence or absence of CD40 ligation or TNF-alpha .In addition,DNA content assay was utilized to make sure the proportion of apoptotic Ramos B cells by various treatments. RESULTS: 1)CD40 and TNF-alpha induced Fas antigen expression on Ramos B cell line cells and rendered them susceptible to Fas-mediated apoptosis.2)CD40 and TNFR signaling upregulate Fas antigen independently.3)Both TNFR and CD40 signaling rescue sIgM crosslink induced apoptosis of Ramos B cell line cells,only CD40,but not TNFR,signaling rescues Ramos cells from Fas-mediated apoptosis. CONCLUSION: Taken together,these results demonstrate that B cell signaling via two distinct members of the NGF/TNFR superfamily,CD40 and TNFR, independently engage the Fas pathway and provide mechanisms for eliminating B cells.Acting alone,both signals will ready B cells for Fas-mediated apoptosis. In concert with sIg signaling,the rescue effect provided uniquely by CD40 ligation assures the selective survival of only those B cells which have bound antigen and presented it to antigen-specific T(h) cells .

Clinical Time Course in SLE Diagnosis,Risk Factors for Delayed Diagnosis,and Association with Prognosis / 대한류마티스학회지

OBJECTIVE: This study is aimed to investigate clinical time course in the diagnosis of SLE,clinical features at disease onset,and their effects on organ damage and disease activity METHODS: We evaluated 244 patients in the Korean Hanyang Lupus Cohort by medical record review and direct interview:the time at disease onset,clinical diagnosis,and diagnosis meeting ACR criteria for SLE (ACR diagnosis),clinical features at onset,SLICC/ACR damage index,SLAM-R,education,and income. The risk factors of delayed diagnosis and their effect on damage and activity were examined with uni and multivariate analyses. RESULTS: Mean age and disease duration were 33.9 (+/- 11.9)and 6.2 (+/- 4 .4 )years. The mean time from onset to clinical diagnosis and ACR diagnosis were 2.4 (+/- 3.6)and 2.7 (+/- 3.3)years.The clinical features at disease onset were as follows;arthritis (46.3%),skin rash (13.7%),fever (7.4%),hematologic disorder (7.4%),nephritis (5.7%),Raynaud phenomenon (3.7%),neuropsychiatric syndrome (2.4%).Diagnosis was delayed when oral ulcer,photosensitivity,and arthritis were presented as 1st clinical features compared to when nephritis, malar rash,and fever as 1st clinical features.Delayed diagnosis (based on median time in clinical diagnosis)was not associated with damage,disease activity,education,and income. CONCLUSIONS: SLE was diagnosed late over 2 years after first symptoms onset and the time interval from clinical symptoms to ACR diagnosis is about 4 months;the delayed diagnosis and the prognosis in terms of damage and activity might depend on 1st clinical features.

A Case of Abdominal Aortic Aneurysm Treated with Immunosuppressants in Behcet's Disease / 대한류마티스학회지

Behcet's disease can elicit serious vascular complications such as thrombophlebitis, arterial occlusion, and arterial aneurysm. The most common cause of death in Behcet's disease is secondary to vascular complication. The treatment for abdominal aortic aneurysm which was frequently complicated by fatal rupture (60%) is mainly a surgical management. If surgical treatment is not possible due to various causes, alternative medical therapy such as immunosuppressives and steroid would be recommended. We report here the case of medically controlled abdominal aortic aneurysm in a 47-year-old patient with Behcet's disease who refused to undergo surgical treatment. Immunosuppressive therapy and steroid could be an alternative treatment of aneurysmal manifestations in Behcet's disease.

Telomerase Activity in Rheumatoid Arthritis / 대한류마티스학회지

No abstract available.

The Characteristics of Elderly Onset Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVES: Patients who develop rheumatoid arthritis over 60 years old(elderly-onset rheumatoid arthritis, EORA) are different from those of younger-onset disease(YORA). Several aspects are emphasized; more equal sex distribution, more often systemic complaints, more larger joints involvement, high ESR rates, and less rheumatoid factor positivity. We compared the clinical characteristics of EORA with those of YORA. METHODS: Five hundred and fifty-six patients who had met the ACR criteria for rheumatoid arthritis were studied. We compared the difference with clinical manifestation, disease activity markers, serologic markers including rheumatoid factor and anti-perinuclear factor, and radiologic changes between EORA and YORA. RESULTS: EORA : YORA ratio is 49 : 507, no difference between male and female ratio. The age at onset of EORA is 67.3 +/- .89 years old, YORA is 4.9 +/-10.5 years old, disease duration is not different in both groups. The ESR of EORA (47.65 +/-13.8) is higher than that of YORA ( 39.49+/-.1 ), the positivity of antiperinuclear factor is significantly increased in the EORA group as compared with YORA group. The comparisons of disease activity including Ritchie index; early RA in both groups showed significant difference only in rheumatoid factor titer. CONCLUSION: The EORA is similar to YORA except high ESR, high rheumatoid factor titer, and high positivity of antiperinuclear factor. However, we failed to demonstrate that the EORA runs different course with heterogeneous pathogenesis to the YORA.

A Case of Hypothyroid Myopathy / 대한류마티스학회지

Hypothyroidism may be accompanied by different rheumatic syndromes such as neuropathy. Arthropathy and myopathy. Sebere myopathy with high elevations of creatine kinase (CK) is rarely seen in hypothyroidism. Authors experinced a case of myopathy with hypothyroidism. Similar to rheumatoid arthritis or polymyositis like syndrome. We have treated with thyroid hormone replacement (Levothyroxine sedium 100ug/day) for 10 months. The initial serology resulted in : free T4 0.51ng/dl, TSH 110ulU/ml, LDH 465 units, and CK 4385 units. After tratment, we gained free T4 1.60ng/dl, TSH 0.14uIU/ml, LDH 110 units, and CK 80 utits and musculoskeletal symptoms are much improved We reprort this patient with a review of literatures.

Effect of Raynaud's Phenomenon on Espohageal Motility / 대한류마티스학회지

OBJECTIVES: In the connective tissue disease patients, esophageal dysfunction is often closely associated with the presence of Raynaud's phenomenon. But there are no previous reports concerning the values of esophageal manometry in the connective tissue disease with Raynaud s phenomenon in Korea. Therefore, we performed this study to evaluate esophageal function in connective tissue disease with Raynaud' s phenomenon. METHODS: Total 86 subjects were employed in this study including 30 normal control group, 14 mixed connective tissue disease(MCTD), 21 systemic sclerosis, 16 systemic lupus erythematosus(SLE), and 5 Raynaud s phenomenon only. In each subject, esophageal manometric study was performed with lower compliance capillary infusion system. RESULTS: The mean age(+SD) of patients and controls were as follows : MCTD 34.1(+8.9), systemic sclerosis 44.9(+9. 3), SLE 32. 1(+7.9), and normal controls 31. 9 (+ 5. 3). All patients with MCTD and systemic sclerosis had Raynaud s phenomenon. Twelve out of 14 patients with MCTD, 17 out of 21 patients with systemic sclerosis, were abnormal in esophageal manometry. Nine out of 16 SLE had Raynaud s phenomenon. Among 9 SLE with Raynaud s phenomenon, 5 patients(55.5%) were abnormal in esophageal manometry, and among 7 SLE without Raynaud's phenomenon, 2 patients (28. 6%) were abnormal in esophageal manometry. Among 5 patients with Raynaud's phenomenon, 3 patients were abnormal in esophageal manometry. Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynaud's phenomenon compared with the patients without Raynaud's phenomenon (P=O. 0219). CONCLUSIONS: Abnormal esophageal manometry finding is more common in the connective tissue disease patients with Raynauds phenomenon compared with the patients without Raynaud s phenomenon.

Adult Onset Still's Disease: Clinical Features and Prognostic Factors in Korea / 대한류마티스학회지

OBJECTIVE: This study was undertaken to review the disease course, clinical and laboratory manifestations, prognosis and treatment of adult onset Still s disease (AOSD) in Korea. METHODS: Thirty-two patients with AOSD were enrolled from 1986 to 1997 in Hanyang University Hospital. Diagnosis of AOSD was based on the criteria proposed by Yamaguchi. We classified the disease course into self-limited, inter mittent, or chronic disease course. RESULTS: Twenty-four (75%) patients were female. Skin rash occurred in 28 (88%) patients, lymphadenopathy in 8 (25%), hepatomegaly in 4 (13%), and pericarditis in 2 (6%) out of 32 patients. The most commonly affected joints were knee joints (88%). Elevated LDH was seen in 18 (60%) patients and decreased CK in 17 (61%) patients. Rheumatoid factor was detected in 4 (13%) patients and ANA in 12 (38%) patients. Anemia (Hb < 10 g/dL) was seen in 13 (41%) patients and hypoalbuminemia (<3. 5 g/dL) in 14 (52%) patients. Elevated ferritin (300 ng/mL) level was seen in 23 (79%) patients. Twenty-five (78%) patients had elevated serum transaminase. Bone marrow studies were performed in 16 patients. Nine out of 16 patients showed hyperplasia of the myeloid series and 2 patients displayed the features of a hemophagocytic syndrome. The mean duration of follow up of 32 patients was 32 months (range 3- 108). Eight (27%) patients had a self-limited, 9 (30%) an intermittent, and 13 (43%) a chronic disease course. The hypoalbuminemia was significantly associated with an "intermittent or chronic disease group" (p<0. 05). Thirty-two patients received systemic corticosteroids and 21 patients received single or combination of disease modifying antirheumatic drugs. CONCLUSION: We found that hypoalbuminemia at presentation was significantly associated with an unfavorable outcome, intermittent or chronic disease group. The clinical manifestations and disease course of AOSD in Korea were similar to those previously reported in other countries except significantly lower incidence of lymphadenopathy, hepatomegaly, and pericarditis.

Massive Subacromial Bursitis with Rice Bodies in Rheumatoid Arthritis / 대한류마티스학회지

Rice bodies are numerous small fibrinous, cartilaginous-like materials which were first described in tuberculous joints. Rice bodies are common findings in joints afflicted with rheumatoid arthritis or other seronegative arthropathy. But less commonly, those can be seen in periarticular bursae or at the sites of tendon or ligament insertion. Rice bodies, thought to be a nonspecific response to synovial inflammation, probably evolve from ischemia in a proliferative synovium. Some of these bodies contain a core of collagen with a mantle of fibrin, others contain only fibrin. Subacromial bursa are sometimes involved in the patients with rheumatoid arthritis and can reach impressive dimensions before becoming clinically detectable because significant constrain is lacking. Subacromial arthrography or magnetic resonance imaging accurately delineated the existence of cartilaginous loose bodies before surgical exision. We experienced a man with rheumatoid arthritis who had massive subacromial bursitis with rice bodies. His shoulder had been swollen since 5 years ago. He felt no pain and had only mild limitation of motion. Subacromial arthrography or magnetic resonance imaging of right shoulder showed multiple rice bodies in enlarged subacromial bursa. Operation finding showed a large encapsulated mass in the subacromial bursa, and hundreds of fibrinous rice bodies were revealed, which were resected. We report this patient with a review of literatures.

Radiological Feature and Significance of Hand X-Rayin Early Rheumatoid Arthritis / 대한내과학회지

OBJECTIVES: To evaluate the disease status in relation to the radiological findings of hands using some short term indices of disease activity and laboratory tests in early rheumatoid arthritis. METHOD: 136 patients with symptoms of rheumatoid arthritis for less than 2 years were studied by means of measuring erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, and anti-nuclear antibody. Of these, 71patients were performed the radiographies of hands at mean 1.3months after diagnosis. Also, 30 radiological films of hands were studied for measurement of intra- & inter-observer variations by well-trained rheumatologist and radiologist. All films were scored by the modified Sharps method. RESULTS: The radiological features of hands showed that the carpal bone was involved more common in the bony erosion (5.7%) and the joint space narrowing (8.8%), and the radiological progression in the interval of 24 months was positively correlated with the disease duration (p<0.05). Also, the radiological lesions and progressions appeared more severe in high disease activity, C-reactive protein (r : 0.334, p : 0.004) and Ritchie index (r : 0.249, p : 0.01) at diagnosis. On the other hand, they were correlated negatively with the age of disease onset (r : -0.357, p : 0.002). The Spearman correlations of inter- & intra-observer variations were significant by 0.716 and 0.775. CONCLUSION: The development of radiological lesions in early rheumatoid arthritis is closely correlated with the duration of disease. The patients with early rheumatoid arthritis who had active arthritis at the time of diagnosis showed more severe radiological progressions.

In The Detection of Early Sacroiliitis / 대한류마티스학회지

No abstract available.

A Case of Mixed connective tissue disease associated with antiphospholipid antibody syndrome / 대한류마티스학회지

No abstract available.

Insufficiency Fractures of Sacrum and Pubic Rami in Rheumatoid Arthritis: Report of 3 Cases and Review of the Literature / 대한류마티스학회지

No abstract available.