[Show abstract][Hide abstract] ABSTRACT: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH.
[Show abstract][Hide abstract] ABSTRACT: The diagnosis of spondyloarthritis (SpA) has a lengthy delay; we investigated the outcomes and factors associated with the delayed diagnosis of SpA. This was a cross-sectional study on patients with SpA who visited a rheumatology clinic at a single tertiary centre. The data were collected from face-to-face interviews, physician assessments of disease status and reviews of medical records. In total, 105 patients with SpA were consecutively enrolled. Of the included patients, 94 had axial SpA and 11 had peripheral SpA. The median diagnostic delay was 8 years (interquartile range, 3-14) for axial SpA. Comparisons between the early and late diagnosis groups were performed to identify the factors related to delayed diagnosis in axial SpA. A definite diagnosis of SpA led to proper management and clinical improvements. The patients with delayed diagnosis showed worse outcomes in disease activity, function, spinal mobility and/or radiographic damage. These patients also demonstrated a less favourable treatment response according to the Bath Ankylosing Spondylitis Disease Activity Index and the rate of radiographic progression. Multivariate analysis indicated that a prior diagnosis of mechanical back pain was an independent factor associated with diagnostic delay. The diagnosis of SpA is often delayed. Delayed diagnosis is associated with worse outcomes and poor treatment responses in SpA patients. Physician and patient awareness of inflammatory back pain are essential for the early diagnosis of SpA, and a referral guideline for patients with suspected SpA is needed.
[Show abstract][Hide abstract] ABSTRACT: Recurrent oral ulcer (ROU) is a common condition that significantly impacts quality of life. It is often related to systemic diseases, such as Behçet’s disease (BD), Crohn’s disease, and ulcerative colitis. Treatment of ROU depends on its severity: from topical agents for mild cases to systemic agents, such as corticosteroids, azathioprine, or other immunosuppressants for severe cases. Recently, good results have been reported with infliximab in refractory ROU. However, the optimal dosage and treatment duration have not been determined and the cost and potential side effects should be considered.
We report on four patients who received a single-dose infliximab for refractory ROU. Two patients had refractory ROU with no underlying disease; one of them had soft palate perforation accompanied by severe oral ulcers. The two other patients had ROU of BD without major organ involvement. All patients received a single infusion of infliximab and an additional infusion was given on demand in one patient. Infliximab showed a rapid, good response in three patients and was also effective in improving the acute inflammation in the perforation of the soft palate, which had been resistant to conventional therapies. These effects diminished over a few weeks, but the ROU were tolerable and it was not necessary to increase steroids or add another medicine for about 1 year. We suggest that a single infusion of infliximab can be considered for refractory ROU.
American Journal of Otolaryngology 09/2014; · 1.23 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: AimTo evaluate treatment patterns and clinical factors affecting gout flare in South Korea.Methods
We retrospectively examined data from 401 patients seen at nine rheumatology multicenter clinics, under urate lowering therapy (ULT) more than 6 months after stopping prophylactic medication. Demographic data, clinical and laboratory features were collected at the initiation of ULT, upon stopping prophylaxis, and 6 months after.ResultsThe mean age was 52.2 years and mean disease duration was 25.0 months. The male-to-female count was 387 : 14. The most common ULT starting agent was allopurinol 83.8%. Colchicine (62.3%) was the most commonly prescribed prophylactic agent. During ULT, 134 of the 401 patients (33.4%) experienced at least one gouty attack in the period from stopping prophylaxis to 6 months later. The duration of prophylaxis was different between those with serum uric acid levels below 6 mg/dL and those over 6 mg/dL (P = 0.001). Of the 179 patients (44.6%) who attained target serum uric acid (SUA) levels (6 mg/dL) at the end of prophylaxis, those taking < 6 months of prophylaxis suffered more frequent flares than those taking it ≥ 6 months (42.9% vs. 26.3%, P = 0.041). The time interval to the first attack after stopping prophylaxis was shorter in the < 6 months group than the ≥ 6 months group (13.5 weeks vs. 22.5 weeks, P = 0.007).Conclusions
Prophylaxis more than 6 months from initiation of ULT, and achieving target SUA (< 6 mg/dL) at the time of stopping prophylaxis is associated with fewer gout flares during ULT.
International Journal of Rheumatic Diseases 08/2014; · 1.65 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Chronic obstructive pulmonary disease (COPD) is one of the causes of cor pulmonale. Cor pulmonale patients with pulmonary hypertension have a significant lower survival rate than patients without. However, there is no conclusive treatment options in cor pulmonale and pulmonary hypertension associated with COPD until now. We report a patient with cor pulmonale and pulmonary hypertension associated with severe form of COPD and tuberculous destroyed lung who achieved marked clinical, functional and echocardiographic hemodynamic improvements with inhaled iloprost for six months.
Journal of cardiovascular ultrasound 06/2014; 22(2):95-7.
[Show abstract][Hide abstract] ABSTRACT: OBJECTIVES: To identify non-major histocompatibility complex susceptible genes that might contribute to Behçet's disease (BD). METHODS: We performed a genome-wide association study using DNA samples from a Korean population consisting of 379 BD patients and 800 controls. A replication study was performed in a Japanese population (363 BD patients and 272 controls). To evaluate the functional implication of the target single nucleotide polymorphisms (SNP), gene expression levels in peripheral T cells, allele-specific modulation of promoter activity and biological effect of mRNA knockdown were investigated. RESULTS: We found a novel association of BD to the GIMAP locus, mapped to chromosome 7q36.1 (rs1608157, p=6.01×10(-8) in a minor allele dominant model; rs11769828, allele based p=1.60×10(-6)). A fine mapping study identified an association with four additional SNP: rs1522596 (OR=1.45, p=7.70×10(-6)) in GIMAP4; rs10266069 (OR=1.32, p=2.67×10(-4)) and rs10256482 (OR=1.27, p=5.27×10(-4)) in GIMAP2; and rs2286900 (OR=1.61, p=3.53×10(-5)) in GIMAP1 areas. Replication study using DNA samples from the Japanese population validated the significant association between BD and the GIMAP locus. The GIMAP4 promoter construct plasmid with the minor allele of rs1608157 displayed significantly lower activity than one with the major allele. Moreover, CD4 T cells from BD patients showed a lower level of GIMAP4 mRNA, and GIMAP4 knockdown was protective against Fas-mediated apoptosis. CONCLUSIONS: These results suggest that a GIMAP cluster is a novel susceptibility locus for BD, which is involved in T-cell survival, and T-cell aberration can contribute to the development of BD.
Annals of the rheumatic diseases 10/2012; · 8.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Matrix metalloproteinase (MMP)-9 expression is reported to be upregulated in several primary vasculitides. The -1562C>T and -91 [CA](n) repeat polymorphisms can affect MMP-9 promoter activity. We investigated the distributions of these functional polymorphisms in 122 patients with Behçet's disease (BD) and in 122 gender- and age-matched healthy controls. Plasma levels of MMP-9 were analyzed. The frequency of "L" alleles with [CA](n) <21 was significantly lower in all BD patients (vs controls, odds ratio (OR) = 0.371 [95% confidence interval 0.152-0.905]) and male patients (vs male controls, OR = 0.117 [0.019-0.737]). Furthermore, the frequency of "H/H" homozygote with [CA](n) > or = 21 was significantly higher in BD patients than controls (OR = 2.677 [1.065-6.729]). Moreover, the frequency of CL haplotype with lower promoter activity was significantly lower in BD patients (vs controls, OR = 0.374 [0.149-0.939]) and in BD patients with visceral involvement (OR = 0.202 [0.044-0.916]). Although plasma MMP-9 levels were not different between controls and BD patients, concentrations of this substance were significantly higher in male patients (vs male controls, p = 0.044) or patients with visceral involvements (vs patients without visceral involvement, p = 0.027). These results suggest that MMP-9 is a novel susceptibility gene and its promoter polymorphisms can affect the development of visceral involvement in BD.
Human immunology 04/2010; 71(7):717-22. · 2.55 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The objective of this study was to investigate clinical and radiographic features and gender differences in Korean patients with adult-onset ankylosing spondylitis. Multicenter cross-sectional studies were conducted in the rheumatology clinics of 13 Korean tertiary referral hospitals. All patients had a confirmed diagnosis of ankylosing spondylitis according to the modified New York criteria. Clinical, laboratory, and radiographic features were evaluated and disease activities were assessed using the Bath ankylosing spondylitis disease activity index. Five hundred and five patients were recruited. The male to female ratio was 6.1:1. Average age at symptom onset was 25.4+/-8.9 yr and average disease duration was 9.6+/-6.8 yr. Males manifested symptoms at a significantly earlier age. HLA-B27 was more frequently positive in males. Hips were more commonly affected in males, and knees in females. When spinal mobility was measured using tragus-to-wall distance and the modified Schober's test, females had significantly better results. Radiographic spinal changes, including bamboo spine and syndesmophytes, were more common in males after adjustment of confounding factors. In conclusion, we observed significant gender differences in radiographic spinal involvement as well as other clinical manifestations among Korea patients with adult-onset ankylosing spondylitis. These findings may influence the timing of the diagnosis and the choice of treatment.
Journal of Korean medical science 04/2010; 25(4):532-5. · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: To identify immunoglobulin variable heavy chain (VH) gene usages in Korean ankylosing spondylitis (AS) patients, expression level of VH2 genes from peripheral blood mononuclear cells (PBMCs) of 8 AS patients and 9 healthy donors was analysed by quantitative real-time PCR (Q-PCR). Q-PCR results demonstrated VH2 genes were overexpressed in AS patients (Relative amount of mRNA of VH2 genes to a house-keeping gene, 7.13+/-7.77 vs, 0.68+/-0.55; P<0.0001). The sequence analysis revealed the majority of them contained CDC42 binding protein kinase Beta (CDC42 BPB) genes. The insertion of CDC42 BPB gene was confirmed by PCR with primers corresponding CDC42 BPB and CH genes. Our study revealed VH2 overexpression and unique rearrangement in Ig VH genes from peripheral blood of AS patients. This may imply aberrant immunoglobulin gene rearrangement in B cell occurs in Korean AS patients, which requires further investigation.
Experimental and Molecular Medicine 02/2010; 42(5):319-26. · 2.57 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: POEMS syndrome--characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes--is a rare multi-systemic disease. Its skin change is often like that of connective tissue diseases such as scleroderma. Although clinical manifestations of POEMS syndrome are known to be diverse, arterial or venous thrombosis is a less-recognised feature. We report a 41-year-old man with scleroderma-like skin change who was initially negative for monoclonal protein, but finally confirmed as having POEMS syndrome. During the disease course, he was complicated by extensive arterial thromboses including stroke. This case evokes the need to include POEMS syndrome in the differential diagnoses in patients with scleroderma-like skin change and to repeat measurements of monoclonal protein to confirm its diagnosis. A POEMS patient with extensive arterial thromboses may have a catastrophic disease course, requiring earlier diagnosis and more aggressive treatment.
[Show abstract][Hide abstract] ABSTRACT: Interferon regulatory factor-1 (IRF-1) is a transcription factor that regulates the functions of type I and II interferons and plays a role in host protection. Behçet's disease (BD) is an idiopathic systemic vasculitis that is often complicated with thrombotic features, and infectious agents have long been postulated to be a disease-triggering factor in its pathogenesis. The authors investigated the distributions of IRF-1 promoter -415 C/A, -410 A/G, and -300 A/G, and 3'-untranslated region (UTR) A/G polymorphisms in 105 BD patients (mean age 41.7 +/- SEM 1.1 years, 44 male and 61 female) and in 105 gender- and age-matched healthy controls. The frequencies of individual alleles and genotypes were not different between the control and BD groups. However, the frequency of AGGG haplotype was significantly higher (73.5% vs 60.2%, odds ratio [OR] = 1.842, 95% confidence interval [95% CI] = 1.219-2.783, p(c) = 0.036) and that of the CAAG haplotype was significantly lower (2.2% vs 9.5%, OR = 0.195, 95% CI = 0.068-0.559, p(c) = 0.02) in BD patients than in healthy controls. In addition, the frequency of the AGGG haplotype was significantly higher (80.3% vs 57.4%, OR = 3.033, 95% CI = 1.716-5.360, p(c) = 0.001) and that of the CAAG haplotype was significantly lower (0.8% vs 12.3%; OR = 0.059, 95% CI = 0.010-0.357, p(c) = 0.005) in female BD patients than female controls. By subgroup analyses, the CAAA haplotype tended to be more common in BD patients with moderate or severe disease than in those with mild disease (25.4% vs 13.6%, OR = 2.158, 95% CI = 1.046-4.440, p = 0.037 before Bonferroni correction). When BD patients were subclassified by a history of deep vein thrombosis (DVT), the CAAA haplotype was found to be significantly increased the risk of DVT (42.1% vs 15.7%, OR = 3.906, 95% CI = 1.836-8.324, p(c) = 0.0015) and the AGGG haplotype tended to reduce this risk (57.9% vs 77.3%, OR = 0.403, 95% CI = 0.195-0.834, p(c) = 0.0685). Furthermore, the frequency of the CAAA haplotype was significantly higher in BD patients that had experienced a thrombotic event than in those that had not (40.5% vs 15.5%, OR = 3.7147, 95% CI = 1.778-7.770, p(c) = 0.0015). These results suggest that IRF-1 is a novel susceptibility gene in BD, especially in women, and furthermore, that IRF-1 polymorphisms may be related to thrombosis in BD patients.
Human Immunology 10/2007; 68(9):770-8. · 2.30 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: SKI306X, which consists of biologically active ingredients from Clematis mandsburica, Tricbosantbes kirilowii, and Prunella vulgaris, was developed and tested in preclinical trials in Korea. Those studies found that SKI306X was associated with an anti-inflammatory and analgesic effect, and that it can delay the destruction of cartilage in rheumatoid arthritis (RA).
The aim of this study was to compare the pain relief and tolerability of SKI306X and celecoxib in patients with RA.
This study was a 6-week, multicenter, randomized, double-blind, double-dummy, Phase III, noninferiority clinical trial. Eligible patients were aged 18 to 80 years, had a history of RA with a disease duration of > or =3 months, and were functional American College of Rheumatology (ACR) class I, II, or III before entry. After a washout period of 2 weeks, patients were randomized to SKI306X 200 mg TID or celecoxib 200 mg BID for 6 weeks. The primary end point was a change in patient assessment of pain intensity using a visual analog scale (VAS). The secondary end points were a 20% improvement in response rate as defined by the ACR (ACR20) and the frequency of rescue medication use. Results after 3 and 6 weeks of treatment were compared with baseline and between treatment groups, and all patients were assessed for adverse events (AEs), clinical laboratory data, and vital signs. AEs were identified based on spontaneous reports by patients during interviews conducted by the investigators and the study coordinator.
Two hundred twenty-two Korean patients from 7 medical centers were assessed and 183 were enrolled and randomized to 1 of 2 treatment groups. Ninety-one patients (10 male, 81 female; mean [SD] age, 52.13 [12.64] years; mean [SD] duration of RA, 9.08 [10.23] years; no. [%] of ACR class I, II, and III, 13 [14.29], 44 [48.35] and 34 [37.36] patients, respectively) received SKI306X 200 mg TID and 92 patients (10 male, 82 female; mean [SD] age, 51.78 [10.94] years; mean [SD] duration of RA, 8.78 [7.78] years; no. [%] of ACR class I, II, and III, 14 [15.22], 44 [47.83], and 34 [36.96] patients, respectively) received celecoxib 200 mg BID. An analysis of the change in reported pain intensity as determined by VAS (mm) score between baseline and week 3 (mean [SD], 13.64 [16.62] vs 14.45 [15.89]), and between baseline and week 6 (18.4 [20.8] vs 17.9 [19.1], respectively) suggested that SKI306X was not inferior to celecoxib. The number of patients who achieved ACR20 response rate was not significantly different between the SKI306X group and the celecoxib group at week 3 (16/87 [18.4%] vs 24/87 [27.6%], respectively) and at week 6 (29/87 [33.3%] vs 29/87 [33.3%]). The frequency of rescue medication use was not significantly different between the SKI306X group and celecoxib group at week 3 (54/87 [62.1%] vs 47/87 [54.0%], respectively) or week 6 (57/87 [65.5%] vs 49/87 [56.3%]). Drug-related AEs were reported by 27 (29.7%) patients in the SKI306X group and 22 (23.9%) patients in the celecoxib group. The most frequent drug-related AEs were epigastric pain (9/91 [9.9%]) in the SKI306X group and glutamyltranferase elevation (4/92 [4.3%]) in the celecoxib group. No significant between-group differences were observed in the prevalence of drug-related clinical- or laboratory-determined AEs.
The results of this study suggest that SKI306X was generally well tolerated and not inferior to celecoxib in regard to pain relief in these Korean patients with RA.
[Show abstract][Hide abstract] ABSTRACT: Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of systemic lupus erythematous (SLE). We report a 28-year-old man with NMO as neuropsy-chiatric manifestation of SLE. He was diagnosed as lupus nephritis at 16-year-old. He had optic neuritis at three years and seven months ago. Oral prednisolone was tapered off according to the improved eye symptoms. Two months later, he visited rheumatology clinics for urinary distur-bance and paresthesia on both feet. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from second to sixth cervical level and from eleventh to twelfth thoracic level. We diagnosed neuromyelitis optica and treated with intravenous cyclophos-phamide therapy monthly for three times. He was discharged without any neurological deficits and has been followed up. Fig. 1. (A) Fundus photography as the frist episode of optic neuritis. Left fundus photography shows the hyperemia, edematous change and blurring around optic disc and right fundus photography shows normal optic disc. (B) Fundus photography as the second episode of optic neuritis. Right fundus photography shows the hyperemia around optic disc and left fundus photography shows the pale optic disc.
The Journal of the Korean Rheumatism Association 01/2007; 14(3).
[Show abstract][Hide abstract] ABSTRACT: This study evaluated the efficacy and tolerability of tramadol 37.5-mg/acetaminophen 325-mg combination tablets (tramadoUAPAP) as add-on therapy in subjects with rheumatoid arthritis (RA) pain that was inadequately controlled by NSAIDs and disease-modifying antirheumatic drugs alone.
Subjects in this multicenter, double-blind trial were randomized in a 3:1 ratio to receive 1 tramadol/ APAP tablet TID or a matching placebo for 1 week. Stable doses of previous medications were continued during the study. The primary efficacy variable was the mean daily pain relief score over 1 week, measured on a 6-point scale (4 = complete; ' = a lot; 2 = some; 1 = a little; 0 = none; -1 = worse). Secondary outcomes included the mean daily pain intensity score, measured on a 100-mm visual analog scale (VAS) (from 0 mm = no pain to 100 mm = extreme pain); pain intensity and pain relief at day 7; subjects' and investigators' mean overall assessments of study drug, measured on a Likert scale (from 2 = very good to -2 = very poor); and subjects' assessments of 8 aspects of physical function (measured on the Health Assessment Questionnaire).
Of 277 subjects randomized to treatment, 267 (201 tramadol/APAP, 66 placebo) were included in the intent-to-treat population. Mean (SD) daily pain relief scores at the end of 1 week were significantly greater in the tramadol/APAP group compared with the placebo group (1.04 [0.89] vs 0.78 [0.80], respectively; P = 0.037), and mean daily pain intensity scores at the end of 1 week were significantly lower (47.23 [19.96] vs 53.81 [16.59]; P = 0.018). Physical function at the end of 1 week did not differ significantly between tramadol/APAP and placebo. Two hundred seventy-two subjects (205 tramadol/APAP, 67 placebo) were evaluable for tolerability. One hundred thirty-three of these subjects had at least 1 adverse event. The incidence of adverse events was significantly higher in the tramadol/APAP group than in the placebo group (57.6% vs 22.4%; P < 0.001). Discontinuations due to adverse events occurred in 19.0% of the tramadol/APAP group and 3.0% of the placebo group (P = 0.001). Of 213 treatment-related adverse events in tramadol/APAP subjects, nausea (34.1%) was the most frequent, followed by dizziness (20.0%) and vomiting (15.6%). One serious adverse event--chest discomfort, nausea, and vomiting after taking study medication-occurred in a subject receiving tramadol/APAP The symptoms resolved 1 day after discontinuing tramadol/APAP.
In this study, tramadol/APAP used as add-on therapy in subjects with symptomatic RA was associated with a significant improvement in pain relief and a significant reduction in pain intensity compared with placebo, with no improvement in physical function. Use of tramadol/APAP may be considered when analgesics are needed in addition to conventional NSAIDs and disease-modifying antirheumatic drugs in subjects with RA.
[Show abstract][Hide abstract] ABSTRACT: Filaggrin is expressed in the cornified layer of epidermis and known to be one of the antigenic targets in rheumatoid arthritis. Although the citrulline residue in filaggrin is thought to be an antigenic determinant recognized by autoantibodies, the diagnostic sensitivity of synthetic citrullinated peptide is variable. To investigate the implication of anti-filaggrin antibodies recognizing uncitrullinated filaggrin in rheumatoid arthritis, we assayed antibody titers using unmodified recombinant filaggrin in the sera from 73 patients with rheumatoid arthritis, 150 patients with other connective tissue diseases and 70 normal controls. We also performed the correlation analysis between antibody titers and the clinical variables in patients with rheumatoid arthritis. Titers of IgG anti-filaggrin antibodies were significantly higher in rheumatoid arthritis patients compared to normal controls (P=0.02), but not in patients with osteoarthritis, ankylosing spondylitis or systemic lupus erythematosus. IgG anti-filaggrin antibodies were more frequently found in patients with rheumatoid arthritis compared to normal controls (12.3% vs 1.4% respectively, P=0.04). An anti-filaggrin antibody titer was correlated with visual analogue scale of pain, tender joint count, Ritchie articular index or C-reactive protein, but not with anti-nuclear antibody or rheumatoid factor. These results suggest that anti-filaggrin antibody recognizes the uncitrullinated filaggrin as an antigen and its titer correlates with clinical parameters, explaining the variable sensitivity of anti-filaggrin antibody test.
Experimental and Molecular Medicine 01/2006; 37(6):546-52. · 2.57 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We investigated the frequency and distribution of osteopenia according to the clinical severity in ankylosing spondylitis (AS). Bone mass was measured in men with mild (n = 45) and severe AS (n = 31) with dual-energy X-ray absorptiometry (DEXA). Definition of clinical severity was based on the Schober's test. Osteopenia was commonly detected (48% in mild AS and 39% severe AS) and, in mild disease, more frequently observed at the lumbar spine than any of the proximal femur sites. In severe AS, however, the frequency of osteopenia at the femoral neck and Ward's triangle was as high as at the lumbar spine. Both bone mineral density and T-scores in severe disease were lower than in mild disease at the femur neck, Ward's triangle, and total proximal femur, but not in the lumbar spine. The progression of osteopenia may be reflected more reliably at proximal femur sites than at the lumbar spine.
Rheumatology International 12/2005; 26(1):30-4. · 2.21 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We investigated the clinical features of Korean patients with adult-onset ankylosing spondylitis (AAS) and examined the differences between AAS patients with and without peripheral joint disease (PJD).
We studied 67 consecutive patients with primary AAS who visited the rheumatology clinic of a tertiary referral hospital. All patients experienced joint symptoms after the age of 15 and fulfilled the modified New York criteria for ankylosing spondylitis. Hips and shoulders were not considered as peripheral joints.
The male-to-female ratio was 8.6:1.0. Mean age at disease onset was 22.3 +/- 5.5 (mean +/- s.d.) yr and disease duration was 10.8 +/- 8.0 yr. Spinal symptoms were the first manifestations in 80.6% of patients. During the disease course, hip, shoulder and peripheral joint involvement were found in about 60% of patients. In patients with PJD, the most commonly affected joints were the knees and ankles. The pattern of PJD, in most cases, was asymmetrical and mono/oligoarticular. AAS patients with PJD had fewer spinal symptoms than those without PJD as a presenting feature (71.8 vs 92.9%, P = 0.035). The modified Schober test showed greater increments in patients with PJD (4.9 +/- 2.4 vs 3.0 +/- 2.4 cm, P = 0.002). Forced vital capacity was better in patients with PJD (79.0 +/- 11.4 vs 70.8 +/- 15.5% of predicted value, P = 0.016). Totally ankylosed sacroiliitis, spinal squaring and syndesmophytes on radiographs were less common in the patients with PJD than in those without PJD (33.3 vs 64.2%, P = 0.012; 20.5 vs 67.9%, P = 0.000; and 38.5 vs 71.4%, P = 0.008, respectively).
Peripheral joints as well as shoulder and hip joints were more frequently involved during the disease course in Korean AAS patients compared with earlier reports in Caucasians. The general joint involvement pattern of PJD was similar to patterns reported previously. Our data suggest that, clinically and radiographically, AAS patients with PJD have a less severe spinal disease course than those without PJD.
[Show abstract][Hide abstract] ABSTRACT: To assess the frequency of juvenile onset ankylosing spondylitis (JAS) in Korean patients with AS and to differentiate the clinical characteristics of JAS from adult onset ankylosing spondylitis (AAS).
We studied 98 consecutive patients with AS who visited the rheumatology clinic of a tertiary referral center and compared clinical and radiographic features of JAS (n = 41) with those of AAS (n = 57).
Median age at onset in JAS was 14 years (range 7-16) and in AAS 22 years (range 17-38) (p < 0.01). Patients with JAS at presentation showed fewer spinal symptoms and more frequent peripheral joint symptoms than those with AAS (41.5% vs 80.7% and 73.2% vs 36.8%, respectively; p < 0.01). Current cervical spine disease was more frequent in AAS (66.7% vs 43.9%; p = 0.02) and current knee disease in JAS (26.8% vs 8.8%; p = 0.02). Patients with JAS showed a shorter tragus-wall distance (mean +/- SD 10.6 +/- 1.7 vs 13.1 +/- 6.9 cm; p < 0.01), more mobility on the modified Schober test (5.7 +/- 2.0 vs 4.0 +/- 2.6 cm; p < 0.01) and chest expansion (4.4 +/- 1.7 vs 3.2 +/- 1.8 cm; p < 0.01), and a better forced vital capacity (75.1 +/- 14.1% vs 82.1 +/- 16.1% of predicted value; p = 0.03) than those with AAS. Totally ankylosed sacroiliitis and spinal syndesmophyte on radiographs were less frequent in JAS patients than in AAS (19.5% vs 47.4% and 17.1% vs 54.4%, respectively; p < 0.01).
The frequency of JAS (41.3%) among Koreans was higher than that reported for Caucasians. General joint involvement pattern at disease onset in JAS was similar to previous reports. Our data suggest that clinically and radiographically JAS has a less severe spinal disease course than AAS.
The Journal of Rheumatology 08/2002; 29(8):1780-5. · 3.26 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Systemic lupus erythematosus (SLE) is characterized by the presence of various autoantibodies and the deposition of immune complex, which is cleared by Fcgamma receptors. Genotype analysis was done to investigate whether the FcgammaRIIIA-176F/V polymorphism is a risk factor for SLE in Koreans. We genotyped 145 Korean SLE patients and 75 control subjects for FcgammaRIIIA-176F/ V. After amplifying a 1.7-kb fragment containing the Fcgamma/RIIIA-176F/V polymorphic site using two FcgammaRIIIA gene-specific primers, we performed a nested polymerase chain reaction (PCR) for allele-specific genotyping at position 559 in FcgammaRIIIA. FcgammaRIIIA genotype or allele distribution was not significantly different between lupus patients and controls, and also between lupus nephritis patients and healthy controls. Neither creatinine clearance, 24 h urine proteinuria, number of American College of Rheumatology (ACR) criteria, nor the Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage index was different according to the genotype. In conclusion, FcgammaRIIIA-176F/V polymorphism is not associated with SLE in Koreans.
Rheumatology International 05/2002; 21(6):222-6. · 2.21 Impact Factor