Arterial Spin-Labeling MR Imaging for the Differential Diagnosis of Venous-Predominant AVMs and Developmental Venous Anomalies.

BACKGROUND AND PURPOSE: Venous-predominant AVMs are almost identical in appearance to developmental venous anomalies on conventional MR imaging. Herein, we compared and analyzed arterial spin-labeling findings in patients with developmental venous anomalies or venous-predominant AVMs, using DSA as the criterion standard. MATERIALS AND METHODS: We retrospectively collected patients with either DVAs or venous-predominant AVMs, each available on both DSA and arterial spin-labeling images. Arterial spin-labeling imaging was visually assessed for the presence of hyperintense signal. CBF measured at the most representative section was normalized to the contralateral gray matter. The temporal phase of developmental venous anomalies or venous-predominant AVMs was measured on DSA as a delay between the first appearance of the intracranial artery and the lesion. Correlation between the normalized CBF and the temporal phase was evaluated. RESULTS: Analysis of 15 lesions (13 patients) resulted in categorization into 3 groups: typical venous-predominant AVMs (temporal phase, <2 seconds), intermediate group (temporal phase between 2.5 and 5 seconds), and classic developmental venous anomalies (temporal phase, >10 seconds). Arterial spin-labeling signal was markedly increased in the typical venous-predominant AVM group, while there was no discernible signal in the classic developmental venous anomaly group. In the intermediate group, however, 3 of 6 lesions showed mildly increased arterial spin-labeling signal. The normalized CBF on arterial spin-labeling and the temporal phase on DSA were moderately negatively correlated: r(13) = 0.66, P = .008. CONCLUSIONS: Arterial spin-labeling may predict the presence and amount of arteriovenous shunting in venous-predominant AVMs, and using arterial spin-labeling enables confirmation of typical venous-predominant AVMs without DSA. However, lesions with an intermediate amount of shunting suggest a spectrum of vascular malformations ranging from purely vein-draining developmental venous anomalies to venous-predominant AVMs with overt arteriovenous shunting.

Suspected Metallic Embolization Distal to Coiled Intracranial Aneurysms Detectable by Susceptibility-Weighted MR Imaging.

BACKGROUND AND PURPOSE: After endovascular coiling of intracranial aneurysms, round dark parenchymal lesions believed to be particulate metal are sometimes encountered in MR imaging studies of the brain. We used SWI to assess the frequency of such occurrences, in addition to exploring likely causes and clinical implications. MATERIALS AND METHODS: We reviewed 700 MR imaging studies performed between September 2018 and March 2019 at our institution as follow-up monitoring of coiled intracranial aneurysms. Any sizeable (>5 mm) rounded dark-signal lesions encountered were presumed to be metallic. The magnitudes and locations of such lesions were recorded. In patients with these lesions, pertinent procedural documentation was screened for devices used, including coils, microcatheters, microguidewires, and stents. Medical records were also examined to determine whether any related symptoms ensued. RESULTS: Twenty patients (2.8%) exhibited a total of 25 lesions on SWI. Diameters ranged from 5 to 11 mm (median, 8 mm). All except 2 lesions were located in brain regions downstream from aneurysms, but all lesions occupied vascular territories of vessels used to place guiding catheters. Other than the Synchro 14, which was routinely deployed, no device was regularly used in patients with SWI-detectable lesions; and none of the affected patients developed focal neurologic symptoms as a consequence. CONCLUSIONS: Although the origins remain unclear, distal embolization of particulate metal distal to coiled cerebral aneurysms is occasionally observed on follow-up MR imaging studies. Such lesions, however, seem to have no apparent clinical impact.

Contralateral Approach to Coil Embolization of Proximal A1 Aneurysms Using the Anterior Communicating Artery.

BACKGROUND AND PURPOSE: Aneurysms arising from the proximal A1 segment of the anterior cerebral artery are rare, and their distinctive configurations often pose technical challenges during endovascular embolization. Herein, we present 11 patients with proximal A1 aneurysms requiring a contralateral approach (via the anterior communicating artery) to coil embolization. MATERIALS AND METHODS: From a prospectively collected data repository, we retrieved records of 11 patients consecutively treated for proximal A1 aneurysms between January 2011 and March 2018. In each instance, coil embolization was performed by the contralateral route. Outcomes were analyzed in terms of morphologic features and clinical status. RESULTS: Aneurysms in all 11 patients were directed posteriorly and were small (<5 mm). A contralateral approach (via the anterior communicating artery) was used after ipsilateral attempts at aneurysm selection failed in each instance, despite using a variety of microcatheters. Single punctures and single guiding catheters sufficed in 9 patients, but 2 patients required dual punctures and 2 guiding catheters. All endovascular treatments ultimately yielded excellent outcomes. Although 1 symptomatic infarct was manifested in the course of ipsilateral treatment, no morbidity or mortality resulted from the contralateral access. CONCLUSIONS: Due to angio-anatomic constraints, a contralateral strategy for coil embolization of proximal A1 aneurysms is acceptable if ipsilateral access is technically prohibitive and the vessels (contralateral A1 and anterior communicating artery) are amenable to the passage of microdevices.

Comparison of Clinical Outcomes of Intracranial Aneurysms: Procedural Rupture versus Spontaneous Rupture.

BACKGROUND AND PURPOSE: Procedural rupture of an intracranial aneurysm is a devastating complication in endovascular treatment. The purpose of this study was to evaluate the clinical outcomes of patients with procedural rupture of unruptured saccular intracranial aneurysms compared with those with spontaneously ruptured aneurysms. MATERIALS AND METHODS: A retrospective review was performed for 1340 patients with 1595 unruptured saccular intracranial aneurysms that underwent endovascular coil embolization between February 2010 and December 2014. The clinical outcomes of patients with procedural rupture of unruptured saccular intracranial aneurysms were compared with those of 198 patients presenting with spontaneously ruptured aneurysms. RESULTS: In this series, procedural rupture developed in 19 patients (1.4% per patient and 1.2% per aneurysm), and the morbidity related to procedural rupture was 26.3% (95% confidence interval, 8.5%-61.4%) with no mortality. Hunt and Hess scale grades and hospitalization days of patients with procedural rupture were equivalent to those of patients presenting with spontaneous aneurysm rupture. Subsequent treatment procedures after hemorrhage (including lumbar drainage, extraventricular drainage, decompressive craniectomy, and permanent shunt) showed no difference between the 2 groups. The hemorrhage volumes were smaller in the procedural-rupture group (P = .03), and the endovascular vasospasm therapies tended to be more frequently required in the spontaneous aneurysm-rupture group (P = .08). At postictus 6 months, the proportion of modified Rankin Scale scores of ≥2 were lower in the procedural-rupture group (5.3% versus 26.8%, P = .049). In multivariate analysis, spontaneous aneurysm rupture was a significant risk factor for worse clinical outcome (OR = 14.9; 95% CI, 1.2-193.1; P = .039). CONCLUSIONS: This study showed better clinical outcomes in the procedural-rupture group. Even though there is a potential chance of aneurysm rupture during treatment, the clinical outcomes after procedural bleeds seem to be more favorable than those of spontaneous rupture.

Risk Factor Analysis of Recanalization Timing in Coiled Aneurysms: Early versus Late Recanalization.

BACKGROUND AND PURPOSE: Long-term documentation of anatomic and angiographic characteristics pertaining to the timing of recanalization in coiled aneurysms has been insufficient. Our intent was to analyze and compare early and late-phase recanalization after coiling, identifying respective risk factors. MATERIALS AND METHODS: A total of 870 coiled saccular aneurysms were monitored for extended periods (mean, 30.8 ± 8.3 months). Medical records and radiologic data were also reviewed, stratifying patients as either early (n = 128) or late (n = 52) recanalization or as complete occlusion (n = 690). Early recanalization was equated with confirmed recanalization within 6 months after the procedure, whereas late recanalization was defined as verifiable recanalization after imaging confirmation of complete occlusion at 6 months. A multinomial regression model served to assess potential risk factors, the reference point being early recanalization. RESULTS: Posterior circulation (P = .009), subarachnoid hemorrhage at presentation (P = .011), second attempt for recanalized aneurysm (P < .001), and aneurysm size >7 mm (P < .001) emerged as variables significantly linked with early recanalization (versus complete occlusion). Late (versus early) recanalization corresponded with aneurysms ≤7 mm (P = .013), and in a separate subanalysis of lesions ≤7 mm, aneurysms 4-7 mm showed a significant predilection for late recanalization (P = .008). However, the propensity for complete occlusion in smaller lesions (≤7 mm) increased as the size diminished. CONCLUSIONS: Although long-term complete occlusion after coiling was more likely in aneurysms ≤7 mm, such lesions were more prone to late (versus early) recanalization, particularly those of 4-7 mm in size. Long-term follow-up imaging is thus appropriate in aneurysms >4 mm to detect late recanalization of those formerly demonstrating complete occlusion.

Do Contrast-Fill Patterns Immediately After Coil Embolization of Small Saccular Aneurysms Impact Long-Term Results?

PURPOSE: It is generally accepted that filling of a saccular aneurysm with contrast immediately after coil embolization predisposes to later recanalization. However, not all such scenarios evolve similarly over time. We investigated outcomes of small (≤ 7 mm) aneurysms with contrast-filled sacs immediately after coil embolization, evaluating the impact of pattern and degree of filling on subsequent recanalization. METHODS: Between January, 2008 and December, 2010, 186 small (≤ 7 mm) saccular aneurysms that retained contrast after coil embolization accrued for this study. Lesions were categorized by pattern (eccentric vs. concentric) and degree of filling on working projections. Clinical and morphologic factors were also analyzed to assess impact on subsequent recanalization. Morphologic outcomes at 6 months or more were assessed. RESULTS: In 93.5 % (174/186) of aneurysms with visible contrast retention, complete occlusion was evident on follow-up imaging studies at 6 months. Multiple logistic regression analysis indicated that eccentric (vs. concentric) contrast filling carried greater risk of subsequent recanalization (p = 0.020). Stent placement and progressive occlusion were also linked, falling short of statistical significance (p = 0.089). Of 166 progressively occluded aneurysms followed for more than 12 months (mean, 30.8 ± 7.3 months), 158 (95.2 %) exhibited stable occlusion. CONCLUSION: Small (≤ 7 mm) aneurysms that retain contrast immediately after coil embolization are more likely to become completely occluded over time through progressive thrombosis. However, an eccentric fill pattern may predispose to recanalization.

Fate of Coiled Aneurysms with Minor Recanalization at 6 Months: Rate of Progression to Further Recanalization and Related Risk Factors.

BACKGROUND AND PURPOSE: Minor recanalization in coiled aneurysms may remain stable with time or may progress to major recanalization. Our aim was to monitor the aneurysms displaying minor recanalization in imaging studies at 6 months, gauging major recanalization rates and related risk factors through extended follow-up. MATERIALS AND METHODS: Sixty-five aneurysms (in 65 patients) showing minor recanalization in follow-up imaging at 6 months were reviewed retrospectively. Medical records and radiologic data accruing during extended monitoring (mean, 24.8 ± 8.2 months) were assessed. Univariate and multivariate analyses were conducted to identify risk factors for progression from minor-to-major recanalization. RESULTS: Progression to major recanalization was observed in 24 (36.9%) of the initially qualifying aneurysms during a follow-up of 112.5 aneurysm-years, for an annual rate of 17.84% per aneurysm-year. Progression was determined chronologically as follows: 14 (58.3%) at 6 months, 8 (33.3%) at 18 months, and 2 (8.4%) at 30 months. Stent deployment significantly decreased the occurrence of major recanalization (OR = 0.22, P = .03), whereas antiplatelet therapy (OR = 0.82, P = .75), posterior location (OR = 0.24, P = .20), and second coiling for recanalized aneurysms (OR = 0.96, P = .96) were unrelated. CONCLUSIONS: Our analysis determined a 36.9% rate of major recanalization during a follow-up of 112.5 aneurysm-years in coiled aneurysms showing minor recanalization at 6 months. Stent deployment alone conferred a protective effect, preventing further recanalization without additional treatment. Given the fair probability of late major recanalization, aneurysms showing minor recanalization at 6 months should be monitored diligently, particularly in the absence of stent placement.

A prospective randomised comparison of two insertion methods for i-gel placement in anaesthetised paralysed patients: standard vs. rotational technique.

In this prospective randomised study, we compared two techniques for i-gel(™) insertion. One hundred and eighty-one anaesthetised, paralysed adult patients were randomly allocated into one of two groups. In the standard group (n = 91), the i-gel was inserted using the standard technique. In the rotation group (n = 90), the i-gel was rotated 90° anticlockwise in the mouth and re-rotated in the hypopharynx to the original alignment. The success rate, insertion time, air leak pressure and complications were assessed. The success rate for insertion at the first attempt was lower for the standard technique, 78 (86%) vs. 87 (97%; p = 0.016). The mean (SD) insertion time was longer (26.9 (14.5) s vs. 22.4 (10.2) s; p = 0.016) and air leak pressure was lower (22.5 (10.4) cm H2O vs. 27.1 (9.4) cm H2O; p = 0.002) in the standard group. The incidence of bloodstaining was higher with the standard technique (8 (9%) vs. 1 (1%); p = 0.034). This study suggests that the rotational technique is superior to the standard technique for i-gel insertion.

Interleukin-18 as an efficient marker for remission and follow-up in patients with inactive adult-onset Still's disease.

OBJECTIVES: Diagnosis of adult-onset Still's disease (AOSD) is difficult because of a lack of pathognomonic findings and markers. The aim of this study was to investigate the efficacy of interleukin (IL)-18 and free IL-18 in the diagnosis and follow-up of patients with AOSD. METHOD: Levels of inflammatory cytokines, IL-18, IL-18 binding protein (IL-18BP), and free IL-18 were compared in 80 AOSD patients and 90 controls. The AOSD patients were divided into active and inactive groups according to disease activity, and the inactive patients were subdivided into a remission subgroup and a low disease activity subgroup. We compared erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, IL-18, and free IL-18 as disease activity markers in the AOSD patients. Serial serum levels of activity markers were measured in 52 of the 80 AOSD patients at 3- to 6-month intervals. RESULTS: There were significantly higher levels of IL-18 and free IL-18 in the AOSD patients than in the controls. IL-18 and free IL-18 were significantly higher in the active group than the inactive group (p < 0.001 for all). Unlike other activity markers, IL-18 and free IL-18 levels in the low disease activity subgroup were significantly higher than those in the remission subgroup within the inactive group (p = 0.004 and 0.005, respectively). During serial follow-up, ferritin and IL-18 showed a significant decrease in the responder and remission subgroup. CONCLUSIONS: IL-18 might be an efficient marker for diagnosis and follow-up of AOSD and might also be a useful predictor of remission, especially in clinically inactive patients.

Multiple-electrode radiofrequency ablations using Octopus® electrodes in an in vivo porcine liver model.

OBJECTIVES: The objective of this study was to determine the in vivo efficacy of radiofrequency ablation (RFA) in porcine liver using Octopus® electrodes for creating a large coagulation compared with RFA using clustered electrodes. METHODS: A total of 39 coagulations were created using a 200-W generator and clustered electrodes or Octopus electrodes during laparotomy in 19 pigs. Radiofrequency was applied to the livers using four protocols: (1) Group A-1, monopolar mode using a clustered electrode (n=11); (2) Group A-2, monopolar mode using an Octopus electrode (n=11); (3) Group B-1, consecutive monopolar mode using three, clustered electrodes (n=8); and (4) Group B-2, switching monopolar mode using two Octopus electrodes (n=9). The energy efficiency, shape, diameters (D) and volume (V) of the coagulation volume were compared in each of the two groups. RESULTS: The mean maximum D and V of the coagulations in Group A-2 (4.7 cm and 33.1 cm(3), respectively) were significantly larger than those in Group A-1 (4.1 cm and 20.3 cm(3), respectively) (p<0.05). Furthermore, the mean minimum D, maximum D and V of the coagulations in Group B-2 were significantly larger than those in Group B-1, i.e. 5.3 vs 4.0 cm, 6.6 vs 4.9 cm and 66.9 vs 30.2 cm(3), respectively (p<0.05). The energy efficiencies were also significantly higher in Groups A-2 and B-2 than in Groups A-1 and B-1 (p<0.05). CONCLUSION: The Octopus electrodes were more efficient for creating a large ablation zone than clustered electrodes, and the efficacy of RFA with Octopus electrodes can be amplified in the switching monopolar mode.

Anticytokine therapy in systemic lupus erythematosus.

Systemic lupus erythematosus is a prototype of heterogeneous autoimmune disease. There have been few newly approved therapeutic agents in lupus treatment for many reasons. Several animal studies and human data have shown that many potential cytokines are related to the pathogenesis and disease activity of systemic lupus erythematosus. Cytokines are produced by many immune cell types and have variable functions in the immune system. Following the success of biological agents in the treatment of inflammatory arthritis, inflammatory bowel disease, and psoriasis, biological targeting to specific cytokines or receptor molecules is now promising in the treatment of systemic lupus erythematosus. In addition to B-cell deleting modalities, clinical trials targeting potential cytokines associated with disease pathogenesis are underway at various clinical stages. Among potential cytokines, targeting agents against B-cell activating factor and interferon-alpha are in the most advanced stage, and belimumab (anti-B-cell activating factor antibody) could be the first biological agent approved in the treatment of systemic lupus erythematosus. Anti-tumor necrosis factor was tried with some success, but with a potential risk of infection in a small number of patients. In this review, we discuss the rationale for anticytokine therapies and review agents currently in clinical trials, and those that could be developed in the near future for systemic lupus erythematosus. We present the results mostly from published trials and data from http://clinicaltrials.gov/ct2/

Effects of gonadotropins on in vitro maturation and of electrical stimulation on parthenogenesis of canine oocytes.

The objective of this study was to determine the effects of gonadotropins on in vitro maturation (IVM) and electrical stimulation on the parthenogenesis of canine oocytes. In experiment I, cumulus oocyte complexes were collected from ovaries at a random phase of the oestrus cycle and cultured on maturation medium treated with hCG or eCG for 48 or 72 h. There were no significant differences in the effects on the metaphase II (MII) rate between the hCG and eCG treatment groups over 48 h (5.4% vs 5.5%). The MII rate in the co-treatment group of hCG and eCG for 48 h was higher than in each hormone treated group (15.5%, p < 0.05). In experiment 2, the parthenogenetic effect on oocyte development, at various electrical field strengths (1.0, 1.5, 2.0 kV/cm DC) for 60 or 80 mus with a single DC pulse after IVM on the co-treatment of hCG and eCG, was examined. The rate of pronuclear formation (37.1%) in electrical activation at 1.5 kV/60 mus without cytochalasin B (CB) was higher than that of oocytes activated in the other groups (p < 0.05). However, we did not observe the cleavage stages. Also, CB did not influence parthenogenesis of canine oocytes. The results showed that the pronucleus formation rate, indicative of the parthenogenesis start point, could be increased by electrical stimulation. Therefore, these results can provide important data for the parthenogenesis of canine oocytes and suggest the probability of parthenogenesis in canines.

Parthenogenetic induction of canine oocytes by electrical stimulation and Ca-EDTA.

In this study, we investigated parthenogenetic induction of canine oocytes by electrical stimulation following Ca-EDTA treatment. Oocyte maturation, parthenogenetic development, and cleavage rate in canine after various electrical stimulations (1.5, 1.8, 2.1 kV/cm) for 50 mus with single DC pulse following 1 mM Ca-EDTA treatment were investigated. In oocyte activated electrically at the voltage of 1.5 kV/cm after 1 mM Ca-EDTA treatment, the rate of pronucleus and two-cell was 4.1% and 2.7%, respectively. Although electrical stimulation could parthenogenetically induce immature oocyte to cleavage stage, degeneration rate in all experimental groups was more than 60%. This means that electrical stimulation after Ca-EDTA treatment could cause canine oocytes to be degenerated. However, two-cell in canine oocyte by parthenogenesis was for the first time induced. Therefore, we suggested that electrical stimulation for canine oocytes could induce parthenogenetically early embryonic cleavage. This result can be used as a basic data for parthenogenesis study in canine. Also, to perform more developed embryonic development, further study to parthenogenesis in canine need to be developed.

Interleukin-1beta and interleukin-1 receptor antagonist gene polymorphisms in Korean patients with adult-onset Still's disease.

OBJECTIVE: Interleukin-1 (IL-1) has been implicated in the pathogenesis of several rheumatic inflammatory diseases, including adult-onset Still's disease (AOSD) and systemic-onset juvenile idiopathic arthritis (SoJIA). Several clinical trials also suggest that anakinra, a human recombinant interleukin-1 receptor antagonist (IL-1Ra), is effective in patients with AOSD and SoJIA. We have therefore investigated whether IL-1beta and IL-1Ra gene polymorphisms are associated with the development and clinical features of AOSD. METHODS: Genomic DNA was isolated from 83 AOSD patients and 144 healthy controls. Genotyping of the two IL-1beta gene (IL-1B+3954 and IL-1B-511) polymorphisms was performed using polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP). Genotyping of the IL-1Ra gene (intron 2, VNTR) polymorphism was performed using PCR-based analysis. To compare genotype and allele frequencies, the chi2-test or Fisher's exact test was used. Haplotype frequencies and pairwise linkage disequilibrium were also estimated. A p-value <0.05 was considered significant. RESULTS: There were no significant differences in the genotype and allele frequencies of the IL-1beta and IL-1Ra gene polymorphisms. No differences were also found in the IL-1 gene cluster haplotypes between both groups. IL-1 gene cluster polymorphisms had no effect on the clinical course and joint involvement pattern. Nevertheless, the IL-1B-511 and IL-1RN (VNTR) polymorphic sites were in linkage disequilibrium. CONCLUSION: These results suggest that IL-1beta and IL-1Ra gene polymorphisms are not associated with the development and clinical features of AOSD in Korean patients.

In vitro maturation, in vitro fertilization and embryonic development of canine oocytes.

In this study we have investigated the efficiency of in vitro maturation (IVM) as a basic way to study the development of canine oocytes after in vitro fertilization (IVF). We decided, therefore, to perform two-part experiments. Firstly, experiment I compared the effects of TCM199 without fetal bovine serum (FBS) with TCM199 supplemented with 5% FBS on the in vitro nuclear maturation rate of canine oocytes. For the efficiency of meiotic development to the metaphase II (MII) stage, we found that 4.7% (4/64) of all oocytes grown in TCM199 without FBS developed to the MII stage compared with only 1.7% (1/59) of those grown in TCM199 with 5% FBS for 48 h. Therefore, FBS did not increase in vitro nuclear maturation. In experiment II, the cleavage rate of canine oocytes used for IVF was investigated following heparin treatment. Canine oocytes were fertilized in four groups: Fert-TALP medium without heparin (Fert I) or Fert-TALP medium supplemented with 10, 20 or 30 microg/ml heparin (Fert II, Fert III, Fert IV, respectively). Oocytes that were grown for 24 h in Fert I following fertilization showed the highest rate of all of the groups, 6.5% (5/77) and developed to the early morula stage. Markedly, the oocytes cultured in Fert I for 24 h following insemination had a higher rate of embryonic development than other groups. We can assert that, unlike findings in other mammals, heparin treatment in canine IVF does not increase the efficiency of the fertilization rate and is therefore not an important factor.

SLC11A1 gene polymorphisms in Korean patients with Behçet's disease.

OBJECTIVE: To investigate the potential susceptibility to the solute carrier family 11 member 1 (SLC11A1) gene polymorphisms of Korean patients with Behçet's disease (BD). METHODS: Ninety-nine patients with BD and 98 controls were recruited. Analyses of three polymorphisms of the SLC11A1 gene [the 5'-promoter (GT)n, D543N and A318V] were performed, either by denaturing high-performance liquid chromatography for D543N and A318V or by using automatic DNA sequencing for the (GT)n. The genotypes and alleles between patients with BD and the controls were compared using the chi2 test and Yate's correction test. RESULTS: No significant differences were found in the distribution of genotypes and alleles of the (GT)n polymorphism between BD patients and the controls. However, subjects with the allele 3 or the genotype allele 3/allele 3 of this polymorphism had a significantly lower risk of developing BD than those without this allele or genotype [allele: p = 0.029, pc = 0.039, odds ratio (OR) = 0.60, 95% confidence interval (CI) 0.37-0.95; genotype: p = 0.036, pc = 0.048, OR = 0.54, 95% CI 0.31-0.96]. In addition, the distributions of genotypes and alleles of D543N were similar between BD patients and controls (p>0.05). In the case of A318V, all of the BD patients and controls had a wild-type genotype. CONCLUSION: The allele 3 and the genotype allele 3/allele 3 of the 5'-promoter (GT)n in the SLC11A1 gene may have a protective effect for the development of BD in the Korean population. Further studies in other populations are required to confirm our results.

Association between the HLA-DRB1 gene and clinical features of systemic sclerosis in Korea.

OBJECTIVE: To determine whether HLA-DR alleles are associated with the development and clinical features of systemic sclerosis (SSc) in Koreans. METHODS: Seventy-nine patients (74 women and five men; 45 diffuse types and 34 limited types; mean age at diagnosis 43.9 years) fulfilling the American College of Rheumatology (ACR) classification criteria for SSc were enrolled. The controls were 144 healthy, disease-free Koreans. HLA-DRB1 genotypes were assessed by the polymerase chain reaction-sequence specific oligonucleotide probe (PCR-SSOP) method. RESULTS: The HLA-DRB1*15 allele was increased in anti-topoisomerase I autoantibody (anti-topo I)-positive SSc patients [p = 0.003, p corrected (p(corr)) = 0.039, odds ratio (OR) = 3.43, 95% confidence interval (CI) 1.45-8.13] compared with controls. The DRB1*11 allele was also observed more frequently in anti-topo I-positive SSc than in controls (13.3% vs. 4.2%) but not statistically significant (p = 0.053, p(corr) = 0.689). In patients with SSc, the DRB1*04 allele was associated with subcutaneous calcinosis (p = 0.048, OR = 4.56, 95% CI 1.07-19.37). Patients with overlap syndrome showed a negative association with the DRB1*04 allele (p = 0.036, OR = 0.26, 95% CI 0.08-0.91). CONCLUSION: The HLA-DRB1*15 allele was associated with the development of anti-topo I-positive SSc in Koreans. In addition, the DRB1*04 allele was associated with certain clinical features in SSc patients.

Palisaded neutrophilic granulomatous dermatitis presenting as an unusual skin manifestation in a patient with Behçet's disease.

A 32-year-old Korean woman with painful oral ulcers and a sore throat presented with multiple erythematosus papules on both legs. Histological examination of the papular lesions on the legs demonstrated palisaded granuloma with degeneration of collagen fibres in the dermis, compatible with palisaded neutrophilic granulomatous dermatitis (PNGD). This condition is known to be an unusual disease entity associated with various systemic autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, other connective tissue diseases, and systemic vasculitis. To our knowledge a case with typical Behçet's disease coinciding with PNGD among systemic autoimmune diseases has not been described before.

Muscular amyloidoma presenting as inguinal masses in multiple myeloma.

We report a case with protruding inguinal masses for 6 months, in whom muscular amyloidoma was not suspected before muscle biopsy. On pelvic magnetic resonance imaging (MRI), round masses showing peripheral rim enhancement with gadolinium were observed in iliopsoas and iliacus muscles of both inguinal areas. The same lesions were also observed in gluteus muscles. The biopsy showed Congo red positive materials in a dense fibrous background. Serum and urine electrophoresis showed Bence Jones protein, lambda type. In bone marrow section, myeloma cells were found. Peripheral blood stem cell transplantation (PBSCT) following four cycles of VAD (vincristine, adriamycin, dexamethasone) chemotherapy was performed and the result was satisfactory. Amyloidoma lesions decreased in size and number on the following MRI.

The association between fcgammaRIIIB polymorphisms and systemic lupus erythematosus in Korea.

Polymorphisms of FcgammaR have been proposed as genetic factors that influence susceptibility to SLE. FcgammaRIIIB polymorphism in systemic lupus erythematosus (SLE) have been studied in various populations, but the results were inconsistent. The aim of this study was to determine the association of FcgammaRIIIB polymorphism in Korean lupus patients. One-hundred and eighty-three SLE patients (166 female, 17 male) meeting 1982 ACR criteria and 300 Korean disease-free controls were enrolled. Genotyping for the FcgammaRIIIB NA1/NA2 was performed by PCR of genomic DNA using allele-specific primers. There was no significant skewing in the distribution of the three FcgammaRIIIB genotypes, and alleles between SLE and the controls. The frequency of FcgammaRIIIB genotypes in SLE patients and controls was FcgammaRIIIB NA1/NA1 27.9% versus 26%, NA1/NA2 55.2% versus 51.7%, NA2/NA2 16.9% versus 22.3%, respectively. The gene frequencies of NA1 allele were 0.56 in the SLE and 0.52 in controls, respectively. Among clinical manifestations, thrombocytopenia was more common in FcgammaRIIIB NA2/NA2 genotype (P = 0.04, OR 2.4, 95% CI 1.0-5.4), and NA2 allele (P = 0.03, OR 1.7, 95% CI 1.1-2.8). Although FcgammaRIIIB polymorphism was not associated with the development of SLE in Korean, thrombocytopenia was associated with FcgammaRIIIB NA2/NA2 genotype, and NA2 allele.