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Jianyang Wang, Shulian Wang, Yongwen Song, Xinfan Liu, Jing Jin, Weihu Wang, Zihao Yu, Yueping Liu, Yexiong Li Department of Radiation Oncology, Cancer Hospital and Institute, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Purpose: To investigate the efficacy and toxicity of postoperative intensity-modulated radiation therapy (IMRT) for patients with soft tissue sarcomas (STSs) in the extremities and trunk wall. Patients and methods: Eighty patients with localized STSs in the extremities and trunk wall treated with function-conserving surgery and postoperative IMRT were analyzed. The primary locations were in the extremities in 51 patients and in the trunk wall in 29 patients. The margins were positive in nine patients and negative in 71 patients. The median dose of IMRT was 64 Gy. Results: At a median follow-up time of 38 months, eight patients developed local recurrences. The 5-year local control (LC) rate was 88.1%. The patients with negative margins exhibited much better LC than did those with positive margins (90% vs 64.8%, P=0.023). Multivariate analysis revealed that positive margin was an independent risk factor for LC. The 5-year distant metastasis-free survival, disease-free survival, and overall survival rates were 75.2%, 72.6%, and 83.6%, respectively. Large tumor size (>5 cm) was significantly associated with poor overall survival. Edema and joint stiffness were observed in 17.6% and 9.8% of patients with extremity STSs, respectively. Conclusion: IMRT provides excellent LC and low toxicity for patients with STSs in the extremities and trunk wall. Keywords: soft tissue sarcoma, extremities and trunk wall, intensity-modulated radiation therapy, local control, toxicitiess

Minji Jennifer Kim1, Yong Suk Jo2, Jee Hyun Kim2, Yong-Jin Kim3, Kyung Hwan Kim4, Eun Bong Lee2, Yeong Wook Song2, Eun Young Lee21School of Medicine, University of Manchester, Manchester, UK; 2Division of Rheumatology, 3Division of Cardiology, Department of Internal Medicine, 4Department of Thoracic Surgery, Seoul National University College of Medicine, Seoul, Republic of KoreaAbstract: Budd–Chiari syndrome has been described as a late complication of Behçet’s disease. Although the mortality rate associated with Behçet’s disease is low, it can escalate in the presence of Budd–Chiari syndrome and may be further complicated by intracardial thrombus formation. It is therefore important to detect and initiate management early in the disease course. The imaging modalities of choice should be minimally invasive as certain procedures may aggravate Behçet’s disease by initiating a thrombosis or aggravating an existing one. In Behçet’s disease-induced Budd–Chiari syndrome, cardiac investigation is crucial in the work-up in order to identify any cardiac involvement and determine the etiology of intracardial thrombus. Furthermore, the treatment should ultimately focus on controlling the activity of Behçet’s disease. We report an unusual case of Behçet’s disease presenting with Budd–Chiari syndrome complicated by intracardial thrombus in a young Korean man.Keywords: Behçet’s disease, Budd–Chiari syndrome, intracardial thrombus

Objectives: To investigate the clinical implications of serum levels of R-spondin 1 (RSPO1), a natural antagonist for Dickkopf-1 (DKK1), and of DKK1/RSPO1 ratios in rheumatoid arthritis (RA) patients. Method: Serum DKK1 and RSPO1 levels were measured in 102 RA patients and 39 age- and gender-matched healthy controls. In addition, DKK1 and RSPO1 levels were determined prior to and 3 months after anti-tumour necrosis factor alpha (anti-TNF-α) therapy in 15 RA patients. Clinical and laboratory data and baseline radiographs of the hands and feet were obtained. Serial radiographs were evaluated in 83 RA patients. Radiographic joint damage was assessed by the modified Sharp/van der Heijde score (SHS). Results: Serum RSPO1 levels were significantly reduced whereas serum DKK1 levels and DKK1/RSPO1 ratios were significantly increased in RA patients compared with controls (all p < 0.0001). Anti-TNF-α treatment significantly suppressed DKK1/RSPO1 ratios (p < 0.01). In contrast to DKK1 or RSPO1 levels, the ratios were significantly associated with erosive disease, elevated acute phase reactants, Disease Activity Score in 28 joints (DAS28) > 3.2, and radiographic progression rate (all p < 0.05). Although the RA patients with radiographic progression exhibited significantly increased DKK1 and reduced RSPO1 levels (p < 0.05), only the DKK1/RSPO1 ratio (log-transformed) was found to be a significant predictor of subsequent radiographic progression [odds ratio (OR) 2.07, p < 0.01]. Conclusions: In this study, the presence of RSPO1 in the circulation was shown for the first time. Our results suggest that the serum DKK1/RSPO1 ratio represents a better predictor of structural progression than either DKK1 or RSPO1 levels alone in RA patients. [ABSTRACT FROM AUTHOR]

Objectives: We hypothesized that monocytes in patients with granulomatosis with polyangiitis (GPA) are polarized towards alternative activation with decreased tumour necrosis factor (TNF)-α production and that tissue-infiltrating monocytes/macrophages in granulomatous GPA lesions express CD163, a marker of alternative macrophage activation. Method: CD16+ monocytes in peripheral blood mononuclear cells (PBMCs) were quantified by flow cytometry. Monocytes were stimulated with increasing concentrations of lipopolysaccharide (LPS), and TNF-α production was measured at 4 and 24 h. CD163 expression in lung biopsies of patients with GPA was detected by immunohistochemistry. Results: Circulating CD16+ monocytes were more frequent in GPA patients compared to controls (4.7 ± 2.8% vs. 1.9 ± 1.2%, p < 0.001). Upon activation with LPS, TNF-α production did not differ between CD16+ and CD16- monocytes. Stimulated monocytes from GPA patients produced significantly less TNF-α compared with monocytes from healthy controls (2903 ± 1320 pg/mL vs. 8335 ± 4569 pg/mL, p < 0.001). Macrophages expressing CD163 were enriched in granulomatous lung lesions of GPA patients. Conclusions: Decreased TNF-α production by circulating monocytes and CD163 overexpression by tissue monocytes/macrophages in granulomatous pulmonary lesions may suggest that monocytes/macrophages are alternatively activated in GPA. [ABSTRACT FROM AUTHOR]

The article presents a study which aims to assess the clinical importance and examine the immune cell profile in the bone marrow of systemic lupus erythematosus (SLE) patients. It states that the study uses immunihistochemistry in examining the immune cells and apoptotic cells in the bone marrow of 16 bone marrow samples. It is inferred that there is increased apoptosis and distinct immune cell profile in the bone marrow of SLE patients.

The article presents a study which measures the costs of illness and the health-related quality of life (HRQOF) among patients with systemic lupus erythematosus (SLE) in South Korea. Topics discussed include the use of multiple and multivariate logistic regressions to assess the cost of illness and HRQOF for SLE patients, the treatment of renal disorders and depression, and the factors associated with higher medical costs for patients.

OBJECTIVE: To identify systemic sclerosis (SSc) susceptibility loci via a genome-wide association study. METHODS: A genome-wide association study was performed in 137 patients with SSc and 564 controls from Korea using the Affymetrix Human SNP Array 5.0. After fine-mapping studies, the results were replicated in 1,107 SSc patients and 2,747 controls from a US Caucasian population. RESULTS: The single-nucleotide polymorphisms (SNPs) (rs3128930, rs7763822, rs7764491, rs3117230, and rs3128965) of HLA-DPB1 and DPB2 on chromosome 6 formed a distinctive peak with log P values for association with SSc susceptibility (P = 8.16 x 10(-13)). Subtyping analysis of HLA-DPB1 showed that DPB1*1301 (P = 7.61 x 10(-8)) and DPB1*0901 (P = 2.55 x 10(-5)) were the subtypes most susceptible to SSc in Korean subjects. In US Caucasians, 2 pairs of SNPs, rs7763822/rs7764491 and rs3117230/rs3128965, showed strong association with SSc patients who had either circulating anti-DNA topoisomerase I (P = 7.58 x 10(-17)/4.84 x 10(-16)) or anticentromere autoantibodies (P = 1.12 x 10(-3)/3.2 x 10(-5)), respectively. CONCLUSION: The results of our genome-wide association study in Korean subjects indicate that the region of HLA-DPB1 and DPB2 contains the loci most susceptible to SSc in a Korean population. The confirmatory studies in US Caucasians indicate that specific SNPs of HLA-DPB1 and/or DPB2 are strongly associated with US Caucasian patients with SSc who are positive for anti-DNA topoisomerase I or anticentromere autoantibodies. [ABSTRACT FROM AUTHOR]

Background: To investigate the association between myositis autoantibodies and clinical subsets of inflammatory myositis in Korean patients.Methods: Immunoprecipitation was performed using the sera of classic polymyositis (PM) (n = 11) and dermatomyositis (DM) (n = 38) patients who met the Bohan and Peter criteria for definite inflammatory myositis. A panel of defined myositis autoantibodies was surveyed to investigate the association between each autoantibody and clinical subsets of inflammatory myositis.Results: Either MSAs, anti-p140, or anti-p155/140 antibodies were found in 63.3% (31/49) of the study subjects. Anti-140-kDa-polypeptide (anti-p140) (18.4%, 9/49) and anti-155/140-kDa polypeptide (anti-p155/140) (16.3%, 8/49) antibodies were the most common, followed by anti-Mi2 (14.3%, 7/49), anti-ARS (12.2%, 6/49) and anti-SRP (2.0%, 1/49) antibodies. All MSAs and anti-p140 and anti-p155/140 antibodies were mutually exclusive. Anti-p140 (23.7%, 9/38), anti-p155/140 (21.1%, 8/38), and anti-Mi2 (18.4%, 3/38) antibodies were found exclusively in DM patients. Anti-p140 antibody was associated with rapidly progressive interstitial lung disease (ILD) (p = 0.001), with a sensitivity of 100.0% (4/4) and a specificity of 85.3% (29/34) in DM patients. Anti-p155/140 antibody was associated with cancer-associated DM (p = 0.009), with a sensitivity of 55.6% (5/9) and a specificity of 89.7% (26/29). Cancer-associated survival was significantly worse when anti-p155/140 antibody was present (19.2 ± 7.6 vs. 65.0 ± 3.5 months, p = 0.032). Finally, anti-ARS antibodies were associated with stable or slowly progressive ILD in PM and DM patients (p = 0.005).Conclusions: Anti-p140 and anti-p155/140 antibodies were commonly found autoantibodies in Korean patients with inflammatory myositis. Despite the lack of clinically amyopathic DM patients in the study subjects, a strong association was observed between anti-p140 antibody and rapidly progressive ILD. Anti-p155/140 antibody was associated with cancer-associated myositis and poor survival. [ABSTRACT FROM AUTHOR]

Purpose: The value of intensity-modulated radiotherapy (IMRT) for early-stage nasal NK/T-cell lymphoma has not been previously reported. The aim of the present study was to assess the dosimetric parameters, toxicity, and treatment outcomes of patients with nasal NK/T-cell lymphoma. Methods and Materials: Between 2003 and 2008, 42 patients with early-stage nasal NK/T-cell lymphoma underwent definitive high-dose and extended involved-field IMRT with or without combination chemotherapy. The median radiation dose to the primary tumor was 50 Gy. The dose-volume histograms of the target volume and critical normal structures were evaluated in all patients. The locoregional control, overall survival, and progression-free survival were calculated using the Kaplan-Meier method. Results: The average mean dose delivered to the planning target volume was 55.5 Gy. Only 1.3% and 2.5% of the planning target volume received <90% and 95% of the prescribed dose, respectively, indicating excellent planning target volume coverage. The mean dose and average dose to the parotid glands was 15 Gy and 14 Gy, respectively. With a median follow-up time of 27 months, the 2-year locoregional control, overall survival, and progression-free survivalrate was 93%, 78%, and 74%, respectively. No Grade 4 or 5 acute or late toxicity was reported. Conclusions: High-dose and extended involved-field IMRT for patients with early-stage nasal NK/T-cell lymphoma showed favorable locoregional control, overall survival, and progression-free survival, with mild toxicity. The dose constraints of IMRT for the parotid glands can be limited to <20 Gy in these patients. [ABSTRACT FROM AUTHOR]

Background:S-adenosylmethionine (SAMe) has antiinflammatory and analgesie effects and has been reported to ameliorate the pain and dysfunction of osteoarthritis (OA). The metabolism of SAMe can be affected by geographic or ethnic factors. However, its efficacy and tolerability versus NSAIDs have not been reported in an Asian population. Objective: This study compared the efficacy and tolerability of SAMe 1200 mg/d and nabumetone 1000 mg/d in Korean patients with knee OA. Methods: This study was an 8-week, multicenter, randomized, double-blind, double-dummy, Phase IV clinical trial. Eligible patients were aged >18 years and had knee OA according to the clinical and radiologic criteria of the American College of Rheumatology, with a symptom duration of greater than or equal to 3 months and with a baseline pain rating of >40 mm on a visual analog scale (VAS) or a pain rating on the VAS that was increased by >10 mm or 20% during the washout period compared with the screening visit. After a washout period of 2 weeks, patients with OA were randomly assigned to receive SAMe 1200 mg/d (400 mg TID) or nabumetone 1000 mg once a day in the evening for 8 weeks. The primary end point was the patient's assessment of pain intensity using a VAS at week 8, and the secondary end points were functional class, patient's global assessment of disease status, physician's global assessment of response to therapy, and the Western Ontario and McMaster Universities (WOMAC) index. Adverse events were assessed based on spontaneous reports by patients during interviews and by laboratory tests. Results: One hundred thirty-four patients, all Asians, were randomly allocated to 1 of 2 treatment groups: 67 patients (56 women, 11 men; mean [SD] age, 63.9 [8.2] years) received SAMe 400 mg TID, and 67 patients (60 women, 7 men; mean age, 62.1 [8.4] years) received nabumetone 1000 mg once daily for 8 weeks. An analysis of changes in pain intensity between weeks 0 and 8 found that both SAMe and nabumetone effectively reduced pain intensity from baseline in each group (mean [SD] change: SAMe, -13.0 [20.8] mm, P < 0.001; nabumetone, -15.7 [20.9] mm, P < 0.001), and the degree of decrease in pain intensity was not significantly different between groups. Secondary end points showed significant improvements from baseline to 8 weeks in both groups. The patient's global assessment of disease status, physician's global assessment of response to therapy, and WOMAC index scores were not significantly different between the groups. Use of acetaminophen as rescue medication did not differ significantly between the groups during weeks 0 to 4 (SAMe, 88.5% [54/61]; nabumetone, 81.3% [52/64]) or weeks 4 to 8 (SAMe, 79.5% [35/44]; nabumetone, 68.5% [37/54]). No significant differences were observed between the treatments in the proportions of patients with all adverse events (SAMe, 35.8% [24/67]; nabumetone, 31.3% [21/67]), drugrelated clinical or laboratory-determined adverse events (SAMe, 22.4% [15/67]; nabumetone, 25.4% [17/67]), or discontinuations due to any adverse events (SAMe, 13.4% [9/67]; nabumetone, 10.4% [7/67]). Conclusion: This study found no significant differences in pain relief or tolerability between treatment with SAMe or nabumetone over 8 weeks in Korean patients with knee OA. [ABSTRACT FROM AUTHOR]