OBJECTIVE: Strong genetic evidence has shown an association between cytotoxic T lymphocyte associated antigen-4 (CTLA-4) and autoimmune diseases. This study was set out to determine whether the polymorphisms of the CTLA-4 exon 1 and promoter are associated with susceptibility to systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) and their clinical features. METHODS: Polymerase chain reaction of genomic DNA-restriction fragment length polymorphism using Bst E II and Tru9 I was used to determine genotypes of the CTLA-4 exon 1 and promoter in 80 SLE, 86 RA patients and 86 healthy control subjects. Clinical manifestations were analyzed in each patient and correlated with the genotypes. RESULTS: The genotype frequency of the CTLA-4 exon 1 differed between SLE patients and controls (chi-squared=.74, 2 degrees of freedom (df), p=.03). The CTLA-4 AG genotype occurred more frequently in patients with SLE (46.3% vs. 33.7% controls). On the other hand, the CTLA-4 AA genotype as well as the CTLA-4 GG genotype was less frequent among SLE patients than among control subjects (1.3% vs. 9.3% and 52.5% vs. 57.0% respectively). The genotype distribution of the CTLA-4 promoter differed between SLE patients and control subjects (CT, TT, CC genotypes 27.5%, 0%, 72.5% vs. 16.3%, 4.7%, 79.1% controls respectively, chi-squared=.36, 2 df, p=0.04). When the association was analyzed with respect to sex, the distribution of the CTLA-4 exon 1- promotor genotypes was significantly different between female SLE patients and females in the control group (chi-squared=8.16, 3 df, p=0.04). The frequencies of the CTLA-4 exon 1 and promoter genotypes, allele and phenotypes and exon 1-promotor genotypes were not significantly different between RA patients and control subjects. Clinically, there were no significant differences in patients with SLE and RA according to the CTLA-4 polymorphisms. CONCLUSION: The polymorphisms within the CTLA-4 exon 1 and promoter appear to play a role in susceptibility to SLE, but not to be associated with clinical features of SLE, susceptibility to RA and its clinical features.
Alleles ; Arthritis, Rheumatoid* ; Autoimmune Diseases ; Exons* ; Female ; Freedom ; Genotype ; Hand ; Humans ; Lupus Erythematosus, Systemic* ; Lymphocytes ; Phenotype ; Polymerase Chain Reaction

OBJECTIVE: To investigate whether the polymorphism of Fas promoter gene is associated with susceptibility to systemic lupus erythematosus (SLE) and its clinical features. METHODS: Polymerase chain reaction of genomic DNA-restriction fragment length polymorphism using MvaI was used to determine genotypes of the Fas promoter in 87 SLE patients and 87 healthy control subjects. Clinical manifestations were analyzed in each patient and correlated with the genotypes. RESULTS: The genotype distribution of the Fas promoter did not differ between SLE patients and control subjects (AA, GA, GG genotypes 31.0%, 54.0%, 14.9% in SLE patients vs. 29.9%, 55.2%, 14.9% in controls respectively, chi2=0.03, 2 degrees of freedom, p=0.99). No significant differences were also found in allele frequencies between the groups. Clinically in the lupus patients according to the Fas promoter polymorphism, there were no significant differences in age at onset, anti-ds DNA titer, C3, C4 level, renal involvement, number of ACR (American College of Rheumatology) criteria presented, SLE Disease Activity Index, SLICC/ACR (The Systemic Lupus international Collaborating Clinics/American College of Rheumatology) damage index, and autoantibody profiles except for anti-RNP antibody. The frequency of anti-RNP antibody in the lupus patients was increased in AA group (71.4%) compared to GA and GG groups (26.2% and 30.0%, p=0.007). CONCLUSION: The Fas promoter polymorphism does not seem to confer susceptibility to SLE, but seems to have some influence on the development of certain autoantibody like anti-RNP antibody, suggesting that the Fas promoter polymorphism is functional.
DNA ; Freedom ; Gene Frequency ; Genotype ; Humans ; Lupus Erythematosus, Systemic* ; Polymerase Chain Reaction

OBJECTIVE: To investigate whether the polymorphism of Fas promoter gene is associated with susceptibility to systemic lupus erythematosus (SLE) and its clinical features. METHODS: Polymerase chain reaction of genomic DNA-restriction fragment length polymorphism using MvaI was used to determine genotypes of the Fas promoter in 87 SLE patients and 87 healthy control subjects. Clinical manifestations were analyzed in each patient and correlated with the genotypes. RESULTS: The genotype distribution of the Fas promoter did not differ between SLE patients and control subjects (AA, GA, GG genotypes 31.0%, 54.0%, 14.9% in SLE patients vs. 29.9%, 55.2%, 14.9% in controls respectively, chi2=0.03, 2 degrees of freedom, p=0.99). No significant differences were also found in allele frequencies between the groups. Clinically in the lupus patients according to the Fas promoter polymorphism, there were no significant differences in age at onset, anti-ds DNA titer, C3, C4 level, renal involvement, number of ACR (American College of Rheumatology) criteria presented, SLE Disease Activity Index, SLICC/ACR (The Systemic Lupus international Collaborating Clinics/American College of Rheumatology) damage index, and autoantibody profiles except for anti-RNP antibody. The frequency of anti-RNP antibody in the lupus patients was increased in AA group (71.4%) compared to GA and GG groups (26.2% and 30.0%, p=0.007). CONCLUSION: The Fas promoter polymorphism does not seem to confer susceptibility to SLE, but seems to have some influence on the development of certain autoantibody like anti-RNP antibody, suggesting that the Fas promoter polymorphism is functional.
DNA ; Freedom ; Gene Frequency ; Genotype ; Humans ; Lupus Erythematosus, Systemic* ; Polymerase Chain Reaction

Behcet's disease is characterized by recurrent orogenital ulcers and ocular and cutaneous inflammatory lesions. It is a multisystem disorder affecting the skin, mucous membrane, eyes, joints, CNS and blood vessels. The vascular involvements consist of thrombophlebitis, arterial occlusion and arterial aneurysm. Rupture of large artery aneurysm is the leading cause of death in patients with Behcet's disease and surgical treatment is necessary. But, surgical treatment is often difficult and may lead to formation of further false aneurysms at the site of vascular anastomosis. Endovascular stent-graft placement emerged as an alternative treatment that is less invasive with a lower risk. We report a case of the aneurysm of right common iliac artery associated with Behcet's disease. Percutaneous stent-graft placement was attempted and successfully controlled aneurysmal manifestations.
Aneurysm* ; Aneurysm, False ; Arteries ; Behcet Syndrome* ; Blood Vessels ; Cause of Death ; Humans ; Iliac Artery* ; Joints ; Mucous Membrane ; Rupture ; Skin ; Thrombophlebitis ; Ulcer

Arterial dissection is an important cause of stroke. We report two cases of isolated posterior inferior cerebellar artery (PICA) dissection diagnosed by high-resolution vessel-wall MRI (HRVW-MRI). One subject complained of abrupt-onset vertigo and headache, and the other subject had headache, vertigo, and Horner syndrome. Conventional MRA showed only focal dilatation of the PICA, but HRVW-MRI revealed intramural hematoma and double-lumen contour in the PICA, suggesting arterial dissection. We suggest that the use of HRVW-MRI should be considered when diagnosing isolated PICA dissection in a PICA infarct with an unknown cause.
Arteries* ; Dilatation ; Headache ; Hematoma ; Horner Syndrome ; Magnetic Resonance Imaging* ; Pica ; Stroke ; Vertigo

OBJECTIVE: The use of Complementary and Alternative Medicine (CAM) is common especially among patients with chronic conditions. The aim of this study was to investigate the percentage and characteristics of patients who make use of CAM and analyse the effect of CAM in RA. METHODS: Two hundreds and twelve patients with RA were interviewed by a trained nurse according to structured questionnaire about sociodemographic features and type, effect and cost of CAM which was used. RESULTS: Among 212 patients with RA, 73 patients had used CAM (34.4%). There was no difference in sociodemographic features between the user and non-user of CAM. The chinese medicine containing herbal medicine and acupuncture (43.8% and 24.7%) was most commonly used. There was no difference in the score of Korean Health Assessment Questionnaire (KHAQ) between the user and non-user of CAM. In the user group, the scores of the subjective effect and satisfaction for CAM were higher than those for the hospital services. The average yearly cost for CAM was 790,000 Won/person and it was about 67.5% of the one for hospital services. CONCLUSION: In patients with RA, the percentage of using CAM was 34.4%. Herbal medicine was most commonly used. CAM did not affect the outcome of the patients with RA. However, the scores of the subjective effect and satisfaction for CAM were higher than those for the hospital services. For the proper management of patients with RA, it is necessary to know further data about the effectiveness and side effects of CAM through experimental and clinical research.
Acupuncture ; Arthritis, Rheumatoid ; Asian Continental Ancestry Group ; Complementary Therapies* ; Herbal Medicine ; Humans ; Surveys and Questionnaires

We describe a patient with rheumatoid arthritis(RA) who developed bronchiolitis obliterans organizing pneumonia(BOOP) during the treatment of bucillamine. A 51 year-old man was admitted to the hospital for an abnormal shadow on his chest radiogragh. He had been diagnosed as having RA 3 years previously and had been receiving 200 mg of bucillamine for 21 months. Two months prior to admission, he presented with a cough and his chest X-ray showed opacities in both lower lungs. He was treated with antibiotics for 2 months after the development of cough and lesions on the chest X-ray, but the symptoms and lung lesions became more aggravated. On admission, an HRCT revealed airspace consolidations in the subpleural space of both basal lungs and a CT-guided fine needle aspiration biopsy showed Masson's body filling air space, interstitial infiltration of acute and chronic inflammatory cells and type II cell hyperplasia, consistent with BOOP. Bucillamine was stopped and 50 mg of prednisolone was administered. His symptoms and infiltrations on the chest X-ray resolved. We suggest that bucillamine should be considered as a drug possibly associated with BOOP.
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Anti-Inflammatory Agents, Non-Steroidal/adverse effects* ; Arthritis, Rheumatoid/drug therapy* ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis* ; Bronchiolitis Obliterans Organizing Pneumonia/chemically induced* ; Case Report ; Cysteine/therapeutic use ; Cysteine/analogs & derivatives ; Cysteine/adverse effects* ; Follow-Up Studies ; Human ; Male ; Middle Age ; Radiography, Thoracic ; Risk Assessment ; Tomography, X-Ray Computed

We report a case of a 48-year-old man with a paradoxic upper airway obstruction and central sleep apnea that developed after an anterior cervical spinal fusion. Nine months before being admitted to this hospital, he was diagnosed with a herniated intervertebral disc between the 5th and 6th cervical spine, and the first operation was carried out. Two months later, a pseudoarthrosis has developed and a second operation, an anterior interbody fusion of the C5 and C6 using autogenous strut bone graft, was performed. After the second operation, he began to complain of snoring, excessive daytime sleepiness, insomnia, and a bizarre sound heard near the upper airway during breathing. Nasopharyngoscope and magnetic resonance imaging disclosed a paradoxical narrowing of the nasopharynx during expiration. On the overnight polysomnography, the apnea index was 8.7/h (central apnea, 7.0/h; obstructive apnea, 1.7/h). Nasal continuous positive airway pressure was applied, but he complained of pressure-intolerance, and laser-assisted uvulopalatoplasty was then performed. Two months after surgery, clinical symptoms as well as the apneas had improved markedly. We suggest that this paradoxic upper airway obstruction might be associated with the anterior cervical spinal surgery even though the mechanism is unclear. This case also emphasizes that an upper airway obstruction can contribute to the development of central sleep apnea.
Airway Obstruction* ; Apnea ; Continuous Positive Airway Pressure ; Humans ; Intervertebral Disc ; Magnetic Resonance Imaging ; Middle Aged ; Nasopharynx ; Polysomnography ; Pseudarthrosis ; Respiration ; Sleep Apnea, Central* ; Sleep Initiation and Maintenance Disorders ; Snoring ; Spinal Fusion ; Spine* ; Transplants

A 67-year old woman was admitted due to left femur neck fracture. Pre-operative laboratory data revealed azotemia, and kidney ultrasonogram and pelvis MRI showed bilateral hydronephrosis and huge uterine myoma. On past history, she had uterine myoma since her thirties, but she refused to undergo operation. We initially planned percutaneous nephrostomy (PCN). After stabilization of renal function, we performed subsequent total hysterectomy after insertion of catheters on both ureters, and PCN catheters were removed after confirming that both ureteral catheters worked well. During follow up period of two months after PCN, renal function was gradually improved, but it was not normalized. Size of removed myoma was about 25x15 cm, and histopathologic findings were consistent with leiomyoma. In conclusion, myoma uteri is one of the rare causes of bilateral hydronephrosis, and it may lead to irreversible damage to kidney if left untreated for a long time.
Aged ; Azotemia ; Catheters ; Female ; Femoral Neck Fractures ; Follow-Up Studies ; Humans ; Hydronephrosis* ; Hysterectomy ; Kidney ; Kidney Failure, Chronic ; Leiomyoma* ; Magnetic Resonance Imaging ; Myoma ; Nephrostomy, Percutaneous ; Pelvis ; Pregnenolone Carbonitrile ; Ultrasonography ; Ureter ; Urinary Catheters ; Uterus

A 51-year old man presented with generalized edema for 10 days. He was admitted to the hospital for a renal biopsy, and a pancreatic mass was incidentally detected by ultrasonography. The renal biopsy was consistent with membranous glomerulonephritis and the pancreas biopsy revealed chronic granulomatous inflammation with multinucleated giant cells, which was suggestive of tuberculosis of the pancreas. The patient was initially treated with a steroid and anti-tuberculosis drugs, but the proteinuria in the nephrotic range persisted throughout the 3-month follow-up. With the presumed diagnosis of tuberculosis-induced membranous nephropathy, immunosuppressant therapy was stopped and anti-tuberculosis drugs were administered for 31 months. With anti-tuberculous treatment, the proteinuria was reduced from 22 g/day to 0.57 g/day. A follow-up abdominal CT revealed a marked reduction in the size of the pancreatic mass. This finding suggests that the membranous nephropathy in our case was related to the pancreatic tuberculosis rather than it being related to primary nephrotic syndrome.
Biopsy ; Edema ; Follow-Up Studies ; Giant Cells ; Glomerulonephritis, Membranous ; Humans ; Inflammation ; Nephrotic Syndrome ; Pancreas ; Proteinuria ; Tuberculosis