OBJECTIVE: This study was conducted to elucidate the associations of HLA with systemic sclerosis (SSc) in Koreans. METHODS: HLA associations with SSc according to SSc-specific autoantibody status and clinical subsets (diffuse and limited) were investigated. HLA-A, B, and C antigens were typed by the serological method using microlymphocytotoxicity test, and HLA-DR by DNA typing method using PCR-reverse hybridization and PCR-SSCP in 56 Korean patients with SSc and 226 healthy controls. For SSc patients, anti-Scl-70 and anicentromere antibodies were tested by double immunodiffusion and indirect immunofluorescence, respectively. RESULTS: The results of HLA class I antigen typing showed that the frequencies of HLA-A24, B52 and B62 were increased, whereas those of A33, B44 and B58 were decreased in SSc patients compared to healthy controls. The frequency of HLA-DR2 was significantly increased, whereas that of HLA-DR13 was decreased in patients with SSc compared to controls. Among HLA-DR2 alleles, both HLA-DRB1*1501 and *1502 were increased in SSc patients compared to controls. According to clinical status, HLA-DRB1*1501 was increased in limited SSc patients and that of DRB1*1502 was increased both in diffuse and limited SSc patients compared to controls. According to autoantibody status, HLA- DRB1 1502 was significantly increased in anti-Scl-70-positive SSc patients and that of DRB1 1501 was increased in anti-Scl-70-negative SSc patients compared to controls. The association of HLA-DR2 alleles with SSc according to clinical subsets and anti-Scl-70 antibody status revealed that the frequency of HLA- DRB1 *1501 was significantly increased in anti-Scl-70-negative limited SSc patients compared to controls. CONCLUSIONS: These results suggest that different HLA-DR2 alleles are associated with different types of SSc in Koreans. HLA-DRB1 1502 shows strong association with anti-Scl-70-positive SSc, and DRB1 1501 with anti-Scl-70-negative limited SSc. It is concluded that the pathogenesis of SSc in Koreans is in part, based on the same genetic background.
Alleles ; Antibodies ; Asian Continental Ancestry Group ; DNA Fingerprinting ; Fluorescent Antibody Technique, Indirect ; HLA-A Antigens ; HLA-A24 Antigen ; HLA-DR Antigens ; HLA-DR2 Antigen ; HLA-DRB1 Chains ; Humans ; Immunodiffusion ; Scleroderma, Systemic*

OBJECTIVE: To investigate the specific cytokine pattern and its profiles in rheumatoid arthritis (RA), we measured the mRNA expression of the IL-4, IL- 2, IFN-r7 and IL-10 in the PBMC s and synovial tissue samples. METHOD: We analyzed the cytokine mRNA copy number semiquantitatively in the fresh PBMC s from 13 rheumatoid patients who were not treated with corticosteroid or DMARDs(disease modifying antirheumatic drugs) which may affect the expression of cytokine and 16 healthy normal controls. Mononuclear cells were separated into three compartment(total PBMC, CD4+ cells, and CD4 cells) by the magnetic bead method. Synovial tissues were obtained from surgical procedure freshly. RNA was extracted from 1x10 cells of each PBMC compartment and synovial tissue. To determine the copy number of cytokine mRNA expression, RT-PCR and dot blot hybridization was performed with the RNA extracted from the samples. RESULT: In CD4+ compartment IFN(interferon)-r mRNA was marginally lower in RA patients(143+114) than in normal control(742+1052, p=0.0517) but IL(interleukin)-4 mRNA expression was higher in RA group(73+50 vs. 32+23 in normal control, p=0.0066). Also in CD4- compartment IFN-r mRNA expression was lower in RA(479+850 vs. 6154+15,059 in normal control, p=0.1875) although it was not significant statistically and IL-4 expression was higher in RA group(308+277 vs. 150+100 in normal group, p=0.0428). In PBMC compartment IFN-1 mRNA expression was also decreased in RA group (148+145 vs. 712 +768, p=0.0148), but IL-4 mRNA expression was marginally increased(186+145 vs. 109+62, p=0.0652). In synovium, interestingly, there was virtually no de novo synthesis of IL-4. CONCLUSION: There is significant difference in cytokine pattern of peripheral PBMC between RA and control group. The main cellular IL-4 source in periph- eral blood is not the CD4+ cells. Also there is significant difference of cytokine pattern between the peripheral blood and eynovium in rheumatoid arthritis.
Arthritis, Rheumatoid* ; Humans ; Interleukin-10 ; Interleukin-4 ; RNA ; RNA, Messenger ; Synovial Membrane*

Henoch-Schonlein purpura (HSP) is a systemic vasculitis with IgA dominant immune complex deposits affecting small vessels in the skin, joint, gastrointestinal tract, and kidneys. Gastrointestinal symptoms are common and may precede the appearance of characteristic skin rash. These manifestations include abdominal pain, bleeding, bowel infarction, intussusception, or even, perforation. However, hemorrhagic ascites has been rarely described in patients with HSP. The pathophysiologic mechanism is presumably a vasculitis of the small vessels within the serosa. We report a 37-year-old man with HSP complicated by hemorrhagic ascites. Contrast CT of the abdomen showed extensive bowel wall thickening and ascites. A paracentesis yielded hemorrhagic fluid. These abdominal manifestations were improved after methylprednisolone pulse therapy.
Abdomen ; Abdominal Pain ; Adult ; Antigen-Antibody Complex ; Ascites* ; Exanthema ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Immunoglobulin A ; Infarction ; Intussusception ; Joints ; Kidney ; Methylprednisolone ; Paracentesis ; Purpura, Schoenlein-Henoch* ; Serous Membrane ; Skin ; Systemic Vasculitis ; Vasculitis

Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells damaged by pathogenic autoantibodies and immune complexes. Nervous system involvement in patients with SLE encompasses a wide spectrum of neurologic and psychiatric features and the frequency of neuropsychiatric manifestations has been estimated at around 25% to 70%. American College of Rheumatology Ad Hoc Committee on neuropsychiatric lupus nomenclature developed case definitions for 19 different neuropsychiatric manifestations observed in SLE in 1999. Among them, Guillain-Barre syndrome and cerebral infarction are very rare neuropsychiatric manifestation. We experienced a 28-year-old woman with neuropsychiatric lupus which presented as Guillain-Barre syndrome and cerebral infarction. She was recovered after treatment with intravenous immunoglobulin, high dose methylprednisolone, cyclophosphamide and anticoagulants.
Adult ; Anticoagulants ; Antigen-Antibody Complex ; Autoantibodies ; Cerebral Infarction* ; Cyclophosphamide ; Female ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulins ; Lupus Erythematosus, Systemic ; Methylprednisolone ; Nervous System ; Rheumatology

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by disturbance in immune surveillance and has an increased risk of cancers. However, no case of tonsillar cancer in SLE has been reported. We report a case of tonsillar cancer in a patient with SLE treated with immunosuppressive agents. A 43-year-old woman with SLE who had been treated with hydroxychloroquine, prednisolone, and azathioprine was admitted because of a palpable mass on her right neck. The patient was diagnosed to have a tonsillar cancer with cervical lymph node metastasis. She received a wide tonsillectomy, right radical neck dissection and radiation therapy.
Adult ; Autoimmune Diseases ; Azathioprine ; Female ; Humans ; Hydroxychloroquine ; Immunosuppression ; Immunosuppressive Agents* ; Lupus Erythematosus, Systemic* ; Lymph Nodes ; Neck ; Neck Dissection ; Neoplasm Metastasis ; Prednisolone ; Tonsillar Neoplasms* ; Tonsillectomy

Scleroderma is rare disease of unknown etiology characterized by fibrosis of skin and internal organs such as lung, gastrointestinal tract, kidney, heart and so on. The association between scleroderma and malignancy has been a controversy during recent years. We report a 77-year old female who had scleroderma and squamous cell carcinoma of esophagus. She was diagnosed as esophageal carcinoma and then sclerotic skin change developed in both hands and feet 3 months later. We present this case with a review of literatures.
Aged ; Carcinoma, Squamous Cell ; Esophageal Neoplasms ; Esophagus ; Female ; Fibrosis ; Foot ; Gastrointestinal Tract ; Hand ; Heart ; Humans ; Kidney ; Lung ; Rare Diseases ; Skin

Mononeuritis multiplex is a peripheral neuropathy characterized by degeneration of one or more named nerve trunks in association with the systemic vasculitides. Mononeuritis multiplex occurs rarely in systemic lupus erythematosus (SLE) patients and usually develops as a late complication of the disease. When mononeuritis multiplex occurs as a presenting feature of SLE, the diagnosis is often delayed causing considerable morbidity. We describe a patient who presented with mononeuritis multiplex as an initial manifestation of SLE.
Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Mononeuropathies* ; Peripheral Nervous System Diseases ; Systemic Vasculitis

OBJECTIVE: Connective tissue growth factor (CTGF) has been proposed to play a role in fibrotic process of systemic sclerosis. Since hypoxia was known to be associated with fibrosis in several profibrogenic conditions, we investigated whether CTGF expression in dermal fibroblast is regulated by hypoxia caused by microvascular loss. METHODS: Dermal fribroblasts from patient with systemic sclerosis and normal controls were cultured in the presence of cobalt chloride (CoCl2), a chemical inducer of HIF-1alpha or hypoxic culture conditions. Expression of HIF-1alpha, VEGF and CTGF was evaluated by semiquantitative reverse transcription-polymerase chain reaction and Western blotting. RESULTS: Scleroderma fibroblasts expressed increased levels of HIF-1alpha, VEGF and CTGF compared to normal dermal fibroblasts. Dermal fibroblasts exposed to various concentration of CoCl2 (1~100microM) enhanced the expression of CTGF mRNA in dose-dependent fashion. Actinomycin D significantly blocked the hypoxia-mediated up-regulation of CTGF mRNA expression, whereas cycloheximide did not block the up-regulation. Up-regulation of CTGF by hypoxia was not mediated by endogenous production of transforming growth factor (TGF)-beta. In time-kinetics study, dermal fibroblasts from scleroderma patients exhibited earlier peak expression of CTGF mRNA than those from normal dermal fibroblasts. In addition, simultaneous treatment of suboptimal concentration of CoCl2 and TGF-beta exhibited the up-regulation of CTGF mRNA in additive fashion. Interferon-gamma did not modulate the expression of CTGF mRNA induced by CoCl2, while the up-regulation of CTGF by TGF-beta was downregulated by Interferon-gamma in a dose-dependent fashion. CONCLUSION: These data indicate that hypoxia up-regulates the expression of CTGF in dermal fibroblasts and provide the evidence that hypoxia caused by microvascular alterations contributes the progression of fibrosis in systemic sclerosis by up-regulation of CTGF.
Anoxia* ; Blotting, Western ; Cobalt ; Connective Tissue Growth Factor* ; Connective Tissue* ; Cycloheximide ; Dactinomycin ; Fibroblasts* ; Fibrosis ; Humans ; Interferon-gamma ; RNA, Messenger ; Scleroderma, Systemic ; Transforming Growth Factor beta ; Transforming Growth Factors ; Up-Regulation ; Vascular Endothelial Growth Factor A

OBJECTIVE: Ultrasonography (USG) of joints has a unique position for the diagnosis of joint diseases. Bone surface, cartilage, periarticular soft tissue and their pathologic changes can be assessed by USG. This study was aimed to compare the radiographic and ultrasonographic findings in osteoarthritis (OA) of the knee joint and to evaluate the usefulness of each modality to evaluate the disease early and determine the severity of the arthritis. METHODS: Fifty osteoarthritis patients classified by the American College of Rheumatology (ACR) clinical criteria from December 2002 to April 2003 were included in the study. Routine radiography (standing anteroposterior, lateral, skyline view) and systemic USG examination of both knee were performed. We compared the incidence of the radiographic and ultrasonographic abnormality related to the pathologic change of the knee OA and suggesting the severity of the OA which would help to decide the therapeutic modality. RESULTS: In patient with knee OA, plain radiography showed abnormal findings in 37/50 (74%) patients, but USG showed at least five abnormal findings in all 50 patients. The abnormal findings detected only by plain radiography were subchondral sclerosis and subchondral cyst (14% and 4% each). But, the thinning of cartilage (94%), Baker's cyst (94%), cartilage degeneration (54%), meniscal protrusion (44%), meniscal tear (34%), meniscal cyst (32%), and the pannus (22%) were detected only by USG. Among the findings shared by both method, joint space narrowing was detected better by plain radiography than USG, but fluid accumulation, spur, meniscal calcification and osteochondroma were detected more frequently by USG. CONCLUSION: USG is more sensitive to find the pathologic changes related to the knee OA and to diagnose OA than the plain radiography. But each of the plain radiography and USG have their own unique value for the evaluation of OA in the knee. So the USG supplements the plain radiography in the examination of the knee OA.
Arthritis ; Bone Cysts ; Cartilage ; Diagnosis ; Humans ; Incidence ; Joint Diseases ; Joints ; Knee Joint ; Knee* ; Osteoarthritis ; Osteoarthritis, Knee* ; Osteochondroma ; Popliteal Cyst ; Radiography* ; Rheumatology ; Sclerosis ; Ultrasonography*

OBJECTIVE: To evaluate the clinical and radiological results and to compare the results of posterolateral lumbar fusion in rheumatic and non-rheumatic disease. METHODS: A retrospective review of results was carried out in 20 patients who had posterolateral lumbar fusion with rheumatic disease from Jul. 1996 to Aug. 2002. And same cases of non-rheumatic disease were compared. The diagnosis of rheumatic disease was confirmed by the ARA revised criteria. Bony union was evaluated by Lenke's criteria and the clinical results by Katz's satisfaction degree. Statistical analysis was performed by paired T-test and ANOVA test. RESULTS: In rheumatic disease group, there were 7 males and 13 females. Mean age was 56.6 (20~68) years and mean fused segments were 2.7 (1~7). Mean follow-up period was 41 months (12~80) after surgery. In non-rheumatic group, there was 6 males and 14 females. Mean age was 57.1 (35~71) years and mean fusion segments were 2.9 (1~4), Mean follow-up period was 40.2 (12~88) months. In age and fusion segments between two group, there was no statistical difference. In rheumatic disease group, the diagnosis were rheumatoid arthritis in 18 patients, ankylosing spondylitis in 1, and systemic lupus erythematosus in 1 patient. The other operations for combined disease were 8 total knee arthroplasty and 3 total hip arthroplasty. There were no statistical differences in operation time (p=0.527), perioperative bleeding (p=0.653) and postoperative (p=0.830) bleeding between two group. In radiological bony union, all patients of two groups showed A and B grade by Lenke's criteria. Bony union was complete at 5.5 (5~8), 5.1 (4~7) months after surgery. There was no significant difference in clinical satisfaction (p=0.756). CONCLUSION: There were no significant differences in clinical and radiological results between the rheumatic and non-rheumatic patients with disease of the lumbar spine.
Arthritis, Rheumatoid ; Arthroplasty ; Arthroplasty, Replacement, Hip ; Diagnosis ; Female ; Follow-Up Studies ; Hemorrhage ; Humans ; Knee ; Lupus Erythematosus, Systemic ; Male ; Retrospective Studies ; Rheumatic Diseases* ; Spinal Fusion* ; Spine* ; Spondylitis, Ankylosing