Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition of which IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC, namely, primary sclerosing cholangitis and cholangiocarcinoma, outline diagnostic criteria for IgG4-SC, provide insight into possible pathophysiological mechanisms underlying the disease and discuss short and long-term management options of this recently described disease.

Multi-vessel coronary artery disease (MVD) frequently features ambiguous or intermediate lesions that may be both serial and complex, suggesting that multiple regions require revascularization. Percutaneous coronary intervention (PCI) is associated with various challenges such as appropriate identification of lesions that should be treated, the choice of an optimum revascularization method, and limitations of long-term outcomes. Optimal patient selection and careful targeting of lesions are key when planning treatment. Physiology-guided decision-making (based on the fractional flow reserve) can overcome the current limitations of PCI used to treat MVD regardless of clinical presentation or disease subtype, as confirmed in recent clinical trials. Here, we review the use of physiology-guided PCI for patients with MVD, and their early and late outcomes.

Transplantation research has focused on cytotoxic T-cell and plasma cell/ B-cell-targeted strategies, but little attention has been paid to the role of T helper 17 (Th17) cells in allograft dysfunction. However, accumulating evidence suggests that Th17 cells contribute to the development of acute and chronic allograft injury after transplantation of various organs, including the kidney. This review summarizes recent reports on the role of Th17 cells in kidney transplantation. Means of improving allograft outcomes by targeting the Th17 pathway are also suggested.

Adverse drug reactions can cause considerable discomfort. They can be life-threatening in severe cases, requiring or prolonging hospitalization, impeding proper treatment, and increasing treatment costs considerably. Although the incidence of severe cutaneous adverse reactions (SCARs) is low, they can be serious, have permanent sequelae, or lead to death. A recent pharmacogenomic study confirmed that genetic factors can predispose an individual to SCARs. Genetic markers enable not only elucidation of the pathogenesis of SCARs, but also screening of susceptible subjects. The human leukocyte antigen (HLA) genotypes associated with SCARs include HLA-B*57:01 for abacavir (Caucasians), HLA-B*58:01 for allopurinol (Asians), HLA-B*15:02 (Han Chinese) and HLA-A*31:01 (Europeans and Koreans) for carbamazepine, HLA-B*59:01 for methazolamide (Koreans and Japanese), and HLA-B*13:01 for dapsone (Asians). Therefore, prescreening genetic testing could prevent severe drug hypersensitivity reactions. Large-scale epidemiologic studies are required to demonstrate the usefulness and cost-effectiveness of screening tests because their efficacy is affected by the genetic differences among ethnicities.

Background/Aims: Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) and brushing cytology are used worldwide to diagnose pancreatic and biliary malignant tumors. Liquid-based cytology (LBC) has been developed and it is currently used to overcome the limitations of conventional smears (CS). In this study, the authors aimed to compare the diagnostic value of the CellPrepPlus (CP; Biodyne) LBC method with CS in samples obtained using EUS-FNA and brushing cytology. Methods: This study prospectively enrolled 75 patients with pancreatic or biliary lesions from June 2012 to October 2013. For cytological analyses, including inadequate specimens, benign and atypical were further classified into benign, and suspicious and malignant were subcategorized as malignant. Sensitivity, specificity, accuracy, and positive predictive values (PPV) and negative predictive values (NPV) were evaluated. Results: In the EUS-FNA based cytological analysis of pancreatic specimens, CP had a sensitivity of 60.7%; specificity, 100%; accuracy, 77.1%; PPV, 100%; and NPV, 64.5%. CS had a sensitivity of 85.7%; specificity, 100%; accuracy, 91.7%; PPV, 100%; and NPV, 83.3%. In the brushing cytology based analysis of biliary specimens, CP had sensitivity of 53.1%; specificity, 100%; accuracy, 54.5%; PPV, 100%; and NPV, 6.3%. CS had a sensitivity of 78.1%; specificity, 100%; accuracy, 78.8%; PPV, 100%; and NPV, 12.5%. Conclusions: Our study found that CP had a lower sensitivity because of low cellularity compared with CS. Therefore, CP (LBC) has a lower diagnostic accuracy for pancreatic EUS-FNA based and biliary brush cytology based analyses compared with CS.

Background/Aims: In countries with a higher risk of gastric atrophic gastritis, noninvasive tests are helpful for a more reliable diagnosis of Helicobacter pylori infection. The aim of this study was to evaluate the characteristics of seropositive subjects according to their stool H. pylori antigen test, serum pepsinogen (PG) assay, and endoscopic findings. Methods: Consecutive subjects who visited Konkuk University Medical Center for upper gastrointestinal endoscopy for a regular check-up were included in a prospective setting if the serum anti-H. pylori immunoglobulin G assay was positive. A H. pylori antigen stool test was measured using a stool H. pylori antigen enzyme-linked immunosorbent assay kit on the same day as a serum PG assay and endoscopy. Results: Of 318 seropositive subjects, 256 (80.5%) showed positive stool test findings. Subjects with a negative stool test result showed lower serum PG I (p < 0.001) and PG II (p < 0.001) levels and higher PG I/II ratio (p < 0.001) than those with a positive stool test. Chronic atrophic gastritis was more common in the positive stool test group than the negative stool test group on endoscopic finding (p = 0.009). A higher serum PG I level (p = 0.001) and a lower serum PG I/II ratio (p = 0.001) were independent risk factors for the presence of H. pylori antigen in stool. Conclusions: A high serum PG level denotes an ongoing current H. pylori infection with positive stool H. pylori antigen test findings. Seropositive subjects with increased gastric secreting ability tend to have H. pylori in their fecal material as reflected by a positive stool H. pylori antigen test finding.

Background/Aims: E-health technologies have been implemented for the management of Crohn's disease (CD). We aimed to identify differences between patient activities at home and at routine clinic visits using a web-based self-reporting CD symptom diary (CDSD) and to determine the impact of this disparity on clinical outcomes. Methods: Patients with CD from three tertiary hospitals were invited to assess their symptoms at least once a week using CDSD. We identified patients who showed disparities in disease activity (high activity at home but normal at the next hospital visit) and evaluated clinical outcomes of these patients such as unscheduled visits due to flares using Kaplan-Meier analyses. Results: One hundred and forty-three patients recorded their symptoms weekly for at least 3 consecutive months and were included. Forty-eight patients (33.6%) showed disparate disease activities between at home and at the next outpatient clinic visit. The cumulative risk of unscheduled visits was significantly higher in this disparity group than in the concordant group (p = 0.001). Disparity in activity (p = 0.003), and anti-tumor necrosis factor use (p = 0.002) were independent risk factors of unscheduled visits due to disease flares. Conclusions: Disparity in disease activity is considerable in CD patients and is related to the risk of unscheduled hospital visit.

Background/Aims: Fluid retention occurs in patients with heart failure, accounting for dyspnea. We investigated the diagnostic implication of body fluid status, assessed by bio-impedance analysis (BIA), in acute heart failure (AHF) among patients who presented with dyspnea. Methods: A total of 100 patients who presented with dyspnea and suspected with AHF were analyzed in this study. We enrolled 50 AHF and 50 non-AHF patients discriminated through echocardiographic analysis and Framingham criteria and were matched by age and sex. Body composition was analyzed using a multifrequency BIA. Results: AHF patients demonstrated higher extracellular water (ECW)/total body water (TBW) compared with non-AHF patients ($0.412{\pm}0.017$ vs. $0.388{\pm}0.023$, p < 0.001). A significant difference of ECW/TBW between AHF patients and non-AHF patients was noted when the upper extremities, trunk, and lower extremities were analyzed (all p < 0.001, respectively). ECW/TBW was not different between patients with reduced ejection fraction (EF) and preserved EF along body compartments. The best cut-off value to predict AHF was > 0.412 at lower extremities with sensitivity and specificity of 0.780 and 0.960. The ECW/TBW of the lower extremities ($ECW/TBW_L$) was correlated with log B-type natriuretic peptide (BNP) levels (r = 0.603, p < 0.001) and also improved the net reclassification improvement and integrated discriminated improvement when added to log BNP level. Multivariate analysis revealed that $ECW/TBW_L$ > 0.412 had an independent association with AHF patients (p = 0.011). Conclusions: The $ECW/TBW_L$ was higher in patients with dyspnea caused by AHF than their counterparts and demonstrated an independent diagnostic implication. It may be a promising marker to diagnose AHF at bedside.

Background/Aims: Compared with metallic drug-eluting stents (DES), bioresorbable vascular scaffolds (BVS) may further improve long-term outcomes of percutaneous coronary intervention (PCI) in patients with coronary artery disease. We report our early experience with BVS in Korea. Methods: We evaluated 105 consecutive patients with BVS implanted at Asan Medical Center, Korea between October 21, 2015 and June 3, 2016. Angiographic results, and in-hospital and 6-month clinical outcomes were assessed. Results: A total of 134 BVS were implanted to treat 115 lesions. The mean age was $62{\pm}10.5years$; 85 patients (81%) were males, 26 patients (25%) were presented with acute coronary syndrome. Among 115 lesions treated with BVS, 76 (66.1%) were B2/C type, 27 (23.5%) were bifurcation lesions, and four (3.5%) were chronic total occlusion. Pre-dilation and post-dilation using high-pressure non-compliant balloon was performed in 104 lesions (90.4%) and 113 lesions (98.2%), respectively. During the procedure, intravascular imaging was used for all patients (100%; intravascular ultrasound 89 and optical coherence tomography 40 patients). Device success rate was 100%. In-segment and in-scaffold acute again were $1.1{\pm}0.6$ and $1.3{\pm}0.5mm$, respectively. Periprocedural myocardial infraction occurred in four patients (3.8%). No deaths, stent thrombosis, or urgent revascularizations occurred either during hospitalization or the follow-up period. Conclusions: In this single-center experience, implantation of BVS with intravascular imaging support was feasible and early clinical outcomes were excellent. Evaluation of long-term efficacy and safety of BVS and its feasibility in clinical use for a broader range of lesions is warranted.

Background/Aims: Patients with diabetes mellitus (DM) and tuberculosis (TB) have increased morbidity and a high risk of treatment failure or recurrence. It is important to manage both diseases simultaneously. Among anti-diabetic drugs, metformin inhibits intracellular growth of mycobacteria. Therefore, we examined the effects of metformin on TB treatment, especially in patients with DM. Methods: This retrospective cohort study included patients with culture-positive pulmonary TB diagnosed between 2011 and 2012. The primary study outcome was sputum culture conversion after 2 months of treatment. Results: Of 499 patients diagnosed with culture-positive pulmonary TB, 105 (21%) had DM at diagnosis. Among them, 62 (59.5%) were treated with metformin. Baseline characteristics, except for the presence of chronic renal disease, were not significantly different between the metformin and non-metformin groups. Metformin treatment had no significant effect on sputum culture conversion (p = 0.60) and recurrence within 1 year after TB treatment completion (p = 0.39). However, metformin improved the sputum culture conversion rate in patients with cavitary pulmonary TB, who have higher bacterial loads (odds ratio, 10.8; 95% confidence interval, 1.22 to 95.63). Conclusions: Among cavitary pulmonary TB patients with DM, metformin can be an effective adjunctive anti-TB agent to improve sputum culture conversion after 2 months of treatment.

Background/Aims: To use serological and multiplex polymerase chain reaction (PCR) assays to examine sputum samples from patients experiencing acute exacerbation of chronic obstructive pulmonary disease (AECOPD) for the presence of atypical pathogens, including Mycoplasma pneumoniae, Chlamydia pneumoniae, and Legionella pneumophila. Methods: From September 2012 to February 2014, 341 patients with AECOPD attending outpatient clinics were enrolled as part of a randomized, double-blind, multicenter study. A commercial enzyme-linked immunosorbent assay was used to measure serum immunoglobulin M (IgM) and IgG antibody titers on the first day of the study and at 36 days post-enrollment. Multiplex PCR was used to test sputum samples for the presence of atypical pathogens. A urinary antigen test for L. pneumophila was performed on the first day. Results: Nineteen patients (5.6%) showed serological evidence of acute infection with M. pneumoniae. Also, one and seven patients (2%) showed serological evidence of acute infection with C. pneumoniae and L. pneumophila, respectively. All DNA samples were negative for M. pneumoniae, C. pneumoniae, and L. pneumophila according to PCR. Only one urine sample was positive for L. pneumophila antigen, but serologic evidence was lacking. Conclusions: Serological testing suggested that infection by atypical pathogens during AECOPD was relatively uncommon. In addition, PCR provided no direct evidence of infection by atypical pathogens. Thus, atypical pathogens may not be a major cause of AECOPD in South Korea.

Background/Aims: As the prevalence of diabetes mellitus and its complications increase rapidly, diabetic foot ulcers (DFUs), which are a major diabetic complication, are expected to increase. For prevention and effective treatment, it is important to understand the clinical course of DFUs. The aim of this study was to investigate the natural course and predictors of amputation in patients with DFUs who required hospitalization Methods: A total of 209 patients with type 2 diabetes, aged 30 to 85 years, who visited emergency department or needed hospitalization due to DFUs were consecutively enrolled from May 2012 to January 2016, by retrospective medical record review. The main outcome was lower extremity amputation (LEA). Results: Among 192 patients who completed follow-up, 113 patients (58.9%) required LEAs. Compared to patients without amputation, baseline levels of white blood cell counts and C-reactive protein were higher in patients with amputation. In addition, bone and joint involvement was more frequently observed in patients with amputation. Multivariable regression analysis revealed that combined infection (odds ratio [OR], 11.39; 95% confidence interval [CI], 2.55 to 50.93; p = 0.001) and bone or joint involvement (OR, 3.74; 95% CI, 1.10 to 12.70; p = 0.035) were significantly associated with an increased risk of LEA. Conclusions: The depth of the wound and combined infection of DFU, rather than the extent of the wound, were significant prognostic factors of LEAs in patients with type 2 diabetes.

Background/Aims: The true incidence of aristolochic acid nephropathy (AAN) is thought to be underestimated because numerous ingredients known or suspected to contain aristolochic acid (AA) are used in traditional medicine in Korea. Methods: We collected data on cases of AAN since 1996 via a database in Korea. We evaluated the year of AAN development, route to obtaining AA-containing herbal medicine, gender, reason for taking AA-containing herbal medicine, clinical manifestations, histological findings, phytochemical analysis, and prognosis of patients with AAN. Results: Data on 16 cases of AAN were collected. Thirteen cases developed AAN before and three cases after the prohibition of AA-containing herbal medicine by the Korea Food and Drug Administration. Patients were prescribed AA-containing herbal medicine from oriental clinics or had purchased it from traditional markets. AAN was distributed in all age groups. Young females were most commonly exposed to AA-containing herbal medicine for slimming purposes and postpartum health promotion, while older adults took AA-containing compounds for the treatment of chronic diseases. The most common symptoms presented at hospitalization were nausea and vomiting, and acute kidney injury was accompanied by Fanconi syndrome in almost half of the patients. Phytochemical analysis of AA in herbal medicine was available in six cases. Progression to end stage renal disease (ESRD) was observed in seven patients (43.8%), and five patients (31.3%) had progressed to ESRD within 6 months of diagnosis. Conclusions: Our report shows that patients were still exposed to AA-containing herbal medicine and that there is a possibility of underdiagnosis of AAN in Korea. A stronger national supervision system of herbal ingredients and remedies in oriental medicine is needed to prevent AAN.

Background/Aims: Predialysis hyponatremia has been recently reported to be associated with mortality in incident hemodialysis patients. However, whether hyponatremia is associated with unfavorable outcomes in elderly patients remains unknown. We hypothesized that nephrology referral inf luences hyponatremia, and aimed to define how nephrology referral affects the association between hyponatremia and mortality in the elderly. Methods: We retrospectively assessed mortality in 599 incident hemodialysis patients aged ${\geq}70$ at a tertiary university hospital, between 2000 and 2010. We analyzed 90-day and 1-year all-cause mortality (ACM) in relation to predialysis serum sodium (sNa). We divided the patients into two groups according to predialysis glucose-corrected sNa: hyponatremia (< 135 mmol/L) and normonatremia (135 to 145 mmol/L). Results: Low estimated glomerular filtration rate, high phosphorus, low albumin, nonpreparation of arteriovenous fistula or graft, and late referral were associated with a low sNa in the elderly. Among 599 patients, 106 and 174 patients died at the 90-day and 1-year follow-ups, respectively. Each 10-mmol/L increase in predialysis sNa tended to be associated with lower 90-day and 1-year ACM. When patients were stratified by nephrology referral, hyponatremia was associated with increased mortality in early referral group (90-day ACM: hazard ratio [HR] = 2.335, p = 0.041; 1-year ACM: HR = 1.790, p = 0.024). However, hyponatremia was not associated with mortality in late referral group. Conclusions: Predialysis hyponatremia at hemodialysis initiation is associated with late referra.

Background/Aims: Adoptive therapy with regulatory T (Treg) cells to prevent graft-versus-host disease (GVHD) would benefit from a strategy to improve homing to the sites of inf lammation following hematopoietic stem cell transplantation (HSCT). Although donor-derived Treg cells have mainly been used in these models, third-party-derived Treg cells are a promising alternative for cell-based immunotherapy, as they can be screened for pathogens and cell activity, and banked for GVHD prevention. In this study, we explored major histocompatibility complex (MHC) disparities between Treg cells and conventional T cells in HSCT to evaluate the impact of these different cell populations on the prevention of acute GVHD, as well as survival after allogeneic transplantation. Methods: To induce acute GVHD, lethally irradiated BALB/c (H-2d) mice were transplanted with $5{\times}10^5$ T cell-depleted bone marrow cells and $5{\times}10^5$ CD4+CD25-splenic T cells from C57BL/6 (H-2b) mice. Recipients were injected with $5{\times}10^5$ cultured donor-, host-, or third-party-derived CD4+CD25+CD62L+ Treg cells (bone marrow transplantation + day 1). Results: Systemic infusion of three groups of Treg cell improved clinicopathological manifestations and survival in an acute GVHD model. Although donor-derived Treg cells were immunologically the most effective, the third-party-derived Treg cell therapy group displayed equal regulation of expansion of CD4+CD25+Foxp3+Treg cells and suppressive CD4+IL-17+ T-helper (Th17) cells in ex vivo assays compared with the donor- and host-derived groups. Conclusions: Our findings demonstrate that the use of third-party Treg cells is a viable alternative to donor-derived Treg cellular therapy in clinical settings, in which human leukocyte antigen-matched donors are not always readily available.

Background/Aims: Markers of inflammation have been associated with outcomes in various cancers. The purpose of this study was to evaluate whether systemic inf lammatory markers and their f luctuations can predict survival and chemotherapy response in patients with metastatic gastric cancer (mGC). Methods: We retrospectively reviewed the records of 502 patients who received first-line palliative chemotherapy for mGC between 2007 and 2013. The neutrophil-to-lymphocyte ratio (NLR) and modified Glasgow prognostic score (mGPS) were assessed before and after chemotherapy to evaluate their association with survival. The NLR values were categorized into two groups based on a cut-off value of 3; mGPS values were classified as high versus low. Results: High prechemotherapy NLR was significantly associated with poor overall survival on univariate analysis (p = 0.002). On multivariate analysis, high prechemotherapy NLR (hazard ratio, 1.43; p < 0.001) was an independent prognostic factor for poor overall survival. However, the prechemotherapy mGPS was not significantly associated with survival. Continuously high NLR or a shift to high NLR postchemotherapy was associated with poor chemotherapy response as well as survival, while NLR reduction was associated with a good response (linear by linear association, p < 0.001) and a favorable prognosis. Conclusions: Prechemotherapy NLR can be used as a prognostic factor in mGC, while the postchemotherapy NLR value may predict the chemotherapeutic response and prognosis. In contrast, mGPS has limited prognostic utility in mGC.

Background/Aims: We evaluated the effects of a multifaceted intervention for controlling a carbapenem-resistant Acinetobacter baumannii (CRAB) epidemic in an intensive care unit (ICU) with no restrictions on carbapenem use. Methods: This study was conducted in a medical ICU between April 2012 and June 2016. The baseline infection control programs included surveillance cultures, contact precautions, and environmental cleaning. However, increases in the detection of CRAB isolates beginning in May 2013 led to the implementation of a new protocol, consisting of universal glove and gown use with daily chlorhexidine bathing for all patients in combination with baseline programs. The efficacy of the intervention was subjected to interrupted time series (ITS) analysis. Results: The multifaceted intervention led to a decrease in the incidence of CRAB from 17.65 to 0.89 cases per 1,000 patient-days, during the study period (p < 0.001). ITS analysis revealed a significant change in the trend of CRAB incidence (-0.413, p < 0.001). Over this same period, mean monthly use of carbapenems increased from $143.54{\pm}95.73$ to $204.95{\pm}78.43$ doses per 1,000 patient-days (p = 0.018). Conclusions: Our multifaceted intervention reduced the incidence of CRAB acquisition in an ICU where carbapenem use was not restricted.

Background/Aims: We evaluated the management and educational status of adult anaphylaxis patients at emergency departments (EDs). Methods: Anaphylaxis patients who visited ED from 2011 to 2013 were enrolled from three hospitals. We analyzed clinical features, prior history of anaphylaxis, management and provided education for etiology and/or prevention. For analyzing associated factors with epinephrine injection, Pearson chi-square test was used by SPSS version 21 (IBM Co.). Results: A total of 194 anaphylaxis patients were enrolled. Ninety-nine patients (51%) visited ED by themselves. Time interval from symptom onset to ED visit was $62{\pm}70.5$ minutes. Drug (56.2%) was the most frequent cause of anaphylaxis. Forty-seven patients (24.2%) had prior history of anaphylaxis and 33 patients had same suspicious cause with current anaphylaxis. Cutaneous (88.7%) and respiratory (72.7%) symptoms were frequent. Hypotension was presented in 114 patients (58.8%). Mean observation time in ED was $12{\pm}25.7$ hours and epinephrine was injected in 114 patients (62%). In 68 patients, epinephrine was injected intramuscularly with mean dose of $0.3{\pm}0.10mg$. Associated factor with epinephrine injection was hypotension (p = 0.000). Twenty-three patients (13%) were educated about avoidance of suspicious agent. Epinephrine auto-injectors were prescribed only in five patients. Only 34 (19%) and 72 (40%) patients were consulted to allergist at ED and outpatient allergy department respectively. Conclusions: We suggested that management and education of anaphylaxis were not fully carried out in ED. An education and promotion program on anaphylaxis is needed for medical staff.

Background/Aims: To evaluate the impact of isoniazid (INH) treatment for latent tuberculosis infection (LTBI) on the development of liver function test (LFT) abnormality and the persistence of tumor necrosis factor (TNF) inhibitors in rheumatoid arthritis (RA) patients. Methods: We retrospectively enrolled patients with RA who were treated with TNF inhibitors at a university hospital between December 2000 and November 2011. After dividing the patients into two groups based on the occurrence of LFT abnormality during follow-up, we compared demographic and clinical features between the two groups. A multivariable logistic regression analysis was performed to identify the impact of INH treatment on LFT abnormality. The impact of INH treatment on the persistence of TNF inhibitors was also evaluated with the log-rank test and the Cox-proportional hazards model. Results: A total of 312 RA patients including 96 patients (30.9%) who took INH for LTBI were included in this analysis. Thirty-nine patients (12.5%) experienced LFT abnormalities while using TNF inhibitors. The use of INH was associated with LFT abnormalities (odds ratio, 3.01; 95% confidence interval [CI], 1.39 to 6.48) after adjusting for covariates, including methotrexate use. However, the persistence of TNF inhibitors over 5 years did not differ between patients receiving or not receiving INH treatment (49.4 vs. 54.6%, p = 0.79). INH treatment was not a risk factor for discontinuation of TNF inhibitors (hazard ratio, 1.01; 95% CI, 0.66 to 1.57). Conclusion: INH treatment for LTBI in RA patients who started TNF inhibitors is associated with the occurrence of LFT abnormality; however, it does not lead to discontinuation of TNF inhibitors.

Background/Aims: The clinical manifestations of Behcet disease (BD) have been reported to differ according to country, region, and race. Gender, onset age, and human leukocyte antigen (HLA)-B51 have also been known as the factors that influence the clinical features of BD. The aim of this study is to investigate the clinical phenotypes of Korean patients who visited the rheumatology clinic with BD with respect to gender, onset age, and HLA-B51. Methods: Total 193 Korean patients (129 females and 64 males) fulfilling the international criteria for BD were retrospectively assessed. Results: The mean age at disease onset and disease duration of the BD patients were $32.2{\pm}11.1$ and $14.2{\pm}9.3$ years, retrospectively. Females suffered more frequently from genital ulcers (90.7% vs. 75.0%, p < 0.01), peripheral arthritis (67.4% vs. 43.8%, p < 0.01), and inf lammatory low back pain (38.8% vs. 23.4%, p = 0.03) than males, while skin involvement was more frequent in males than in females (90.6% vs. 75.2%, p = 0.01). The patients with late onset of BD (> 40 years) suffered from neurologic involvement (15.9% vs. 4.2%, p = 0.007) more frequently than those with early onset of BD. The patients with HLA-B51 showed earlier onset of disease than without HLA-B51 ($28.3{\pm}11.4years$ vs. $33.8{\pm}11.6years$, p = 0.02) and the neurologic and gastrointestinal involvements were more frequent in the patients without HLA-B51 than with HLA-B51 (17.2% vs. 2.5%, p = 0.02 and 20.7% vs. 2.5%, p = 0.01, respectively). Conclusions: The clinical phenotypes in Korean patients with BD may be influenced by gender, onset age and HLA-B51.