Tumor-like Presentation of Cerebral Vasculitis in a Patient With Systemic Lupus Erythematosus: A Biopsy-confirmed Case

Central nervous system (CNS) manifestations of systemic lupus erythematosus (SLE) are diverse and often difficult to distinguish from SLE-unrelated events. CNS vasculitis is a rare manifestation, which is seen in less than 10% of post-mortem studies, and lesions with multifocal cerebral cortical microinfarcts associated with small-vessel vasculitis are the predominant feature. However, CNS vasculitis presenting as a tumor-like mass lesion in SLE has rarely been reported. Herein, we report a case of cerebral vasculitis mimicking a brain tumor in a 39-year-old female with SLE. A biopsy of the brain mass revealed fibrinoid necrosis and leukocytoclastic vasculitis. The neurological deficits and systemic symptoms improved after treatment with corticosteroids and immunosuppressive agents. To the best of our knowledge, there are no reports of biopsy-proven cerebral vasculitis presenting as a brain mass in patients with SLE in Korea.

Case of Renal Microaneurysms and Multiple Renal Infarctions in a Patient with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic inflammatory, heterogeneous autoimmune disease characterized by autoantibody production and the potential involvement of almost every organ system. Although vasculitis usually confined to small vessels is a fairly common feature of SLE, ischemic vasculitis with an aneurysm is an uncommon feature. In particular, renal arterial microaneurysms and multiple renal infarctions are very rarely reported in patients with SLE. Furthermore, to the best of the authors' knowledge, there is no report on renal arterial microaneurysms associated with SLE in Korea. Here, this paper presents a case of renal microaneurysms and multiple renal infarctions in a 41-year-old woman with SLE.

Bilateral Involvement of Juvenile Temporal Arteritis Associated with Kimura Disease

Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.

Qualitative correlation between postoperatively increased vertical dimension and mandibular position in skeletal class III using partial-least-square path modeling / 대한악안면성형재건외과학회지

BACKGROUND: This study constructed a partial-least-square path-modeling (PLS-PM) model and found the pathway by which the postsurgical vertical dimension (VD) affects the extent of the final mandibular setback on the B point at the posttreatment stage for the skeletal class III surgery-first approach (SFA). METHODS: This study re-analyzed the data from the retrospective study by Lee et al. on 40 patients with skeletal class III bimaxillary SFA. Variables were obtained from cone beam computed tomography (CBCT)-generated cephalograms. Authors investigated all variables at each time point to build a PLS-PM model to verify the effect of the VD on the final setback of the mandible. RESULTS: From PLS-PM, an increase in VD10 was found to decrease the absolute value of the final setback amount of the mandible, which reflects the postsurgical physiological responses to both surgery and orthodontic treatment, which, in turn, can be interpreted as an increase in postoperative mandibular changes. CONCLUSIONS: To resolve the issue of collinear cephalometric data, the present study adopted PLS-PM to assess the orthodontic treatment. From PLS-PM, it was able to summarize the effect of increased postsurgery occlusal vertical dimension on the increased changeability of the B point position at the posttreatment stage.

The influence of gonadotropin releasing hormone agonist treatment on the body weight and body mass index in girls with idiopathic precocious puberty and early puberty

PURPOSE: This study aimed to investigate the influence of gonadotropin releasing hormone agonist (GnRHa) treatment on the weight and body mass index (BMI) of girls who were diagnosed with idiopathic central precocious puberty (CPP) or early puberty (EP). METHODS: Patients who were younger than 8 years of age at diagnosis were classified as CPP and patients aged between 8 and 9 years at diagnosis were classified as EP. Of 129 patients, 34 were diagnosed with CPP and 95 were diagnosed with EP. The patients were divided according to pretreatment weight status into normal weight group, an overweight group, or an obese group. RESULTS: No significant changes were observed with respect to the weight standard deviation score (SDS) before and after 1 year, 2 years of treatment, respectively (P>0.05, P>0.05) in all patient groups. No significant changes were observed in relation to the BMI SDS before and after 1 year, 2 years of treatment, respectively (P>0.05, P>0.05) in all patient group. Depending on the degree of obesity, differences with respect to the weight SDS and BMI SDS were observed. CONCLUSION: BMI SDS increased in the GnRHa-treated patients as a whole group, but was not statistically significant. But BMI SDS increased significantly in the normal weight group after 2 years of GnRHa treatment. So, GnRHa treatment may affect the change of BMI SDS depending on degree of obesity.

Clinical Characteristics of Enteroviral Meningitis without Cerebrospinal Fluid Pleocytosis in Children

PURPOSE: The purpose of this study was to investigate the clinical features and laboratory findings of enteroviral meningitis according to the presence of cerebrospinal fluid (CSF) pleocytosis, and to investigate the factors influencing pleocytosis in children. METHODS: A retrospective study was conducted in 300 children with enteroviral meningitis confirmed by CSF reverse transcriptase polymerase chain reaction (RT-PCR) between 2012 and 2016. Electronic medical records including clinical and laboratory data were assessed. Clinical factors were compared between patients with and without pleocytosis. RESULTS: Of the total 300 children with enteroviral meningitis, 73 had no pleocytosis. The proportion of infants under the age of two years was higher in patients without pleocytosis than in patients with pleocytosis (39.7% vs 14.5%, P<0.001). Patients without pleocytosis were younger (43.4 months vs 66.2 months, P<0.001), had less prevalence of headache and vomiting, lower peripheral white blood cell count (9,740±3,555/mm³ vs 11,632±3,872/mm³) and a shorter interval between the onset and lumbar puncture than those with pleocytosis. In multiple regression analysis, younger age, vomiting, lower white blood cell count, and interval less than 24 hours from onset to spinal tap were associated with the absence of pleocytosis. CONCLUSION: This study showed that children younger than two years of age had high proportion of absence of pleocytosis and some of the factors analyzed correlated with CSF inflammation. Therefore, absence of pleocytosis in the initial CSF sample cannot exclude meningitis, and rapid RT-PCR testing should be performed with high suspicion in young infants.

Focal Eosinophilic Myositis Associated with Behçet's Disease

Behçet's disease (BD) is a systemic vasculitis commonly accompanied by recurrent mucosal ulceration and other systemic manifestations, but rarely by myositis. Focal eosinophilic myositis is the most limited idiopathic eosinophilic myopathy characterized by peripheral blood eosinophilia and/or eosinophilic muscle infiltration. Clinical manifestations include myalgia, muscle weakness, and cutaneous lesions, such as subcutaneous induration and erythema. Given that BD can mimic deep vein thrombosis or pseudotumor, muscle biopsy should be performed to enhance the accuracy of diagnosis. Microscopic examination reveals extensive infiltration of eosinophils and mononuclear cells into muscle, myofiber necrosis, and regeneration. To the best of our knowledge, there have not been any published reports on MEDLINE regarding focal eosinophilic myositis associated with BD. Here, we presented a case of focal eosinophilic myositis associated with intestinal BD in a 23-year-old man who suffered from a large ulcer in the terminal ileum.

Characteristics and Psychiatric Symptoms of Internet Gaming Disorder among Adults Using Self-Reported DSM-5 Criteria

OBJECTIVE: The Section III of the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) proposed nine diagnostic criteria and five cut-point criteria for Internet Gaming Disorder (IGD). We aimed to examine the efficacy of such criteria. METHODS: Adults (n=3041, men: 1824, women: 1217) who engaged in internet gaming within last 6 months completed a self-report online survey using the suggested wordings of the criteria in DSM-5. Major characteristics, gaming behavior, and psychiatric symptoms of IGD were analyzed using ANOVA, chi-square, and correlation analyses. RESULTS: The sociodemographic variables were not statistically significant between the healthy controls and the risk group. Among the participants, 419 (13.8%) were identified and labeled as the IGD risk group. The IGD risk group scored significantly higher on all motivation subscales (p<0.001). The IGD risk group showed significantly higher scores than healthy controls in all nine psychiatric symptom dimensions, i.e., somatization, obsession-compulsion, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, and psychoticism (p<0.001). CONCLUSION: The IGD risk group showed differential psychopathological manifestations according to DSM-5 IGD diagnostic criteria. Further studies are needed to evaluate the reliability and validity of the specific criteria, especially for developing screening instruments.

Review of Disaster Mental Health System in Japan / 신경정신의학

Mental health is one of the most important issues for disaster survivors, and many studies have reported higher rates of mental health problems after disasters. Because Japan has experienced frequent earthquakes, tsunami, typhoons, and volcanoes, mental health problems have been a matter of great concern after disasters in Japan. To serve the psychiatric services after the Hanshin-Awaji (Kobe) earthquake, 'Hyogo institute for traumatic stress' was established. And when the disaster caused by the great east Japan earthquake and tsunami occurred, 'National information center of disaster mental health' and 'Disaster psychiatric assistance team' had the important role of reducing mental health problems.

Complex repetitive discharge on electromyography as a risk factor for malignancy in idiopathic inflammatory myopathy

BACKGROUND/AIMS: We investigated the electromyography (EMG) findings and demographic, clinical, and laboratory features that may predict the development of malignancy in patients with idiopathic inflammatory myopathy (IIM). METHODS: In total, 61 patients, 36 with dermatomyositis and 25 with polymyositis, were included. Patients were divided into those with and without malignancies, and comparisons were made between the groups in terms of their demographic, clinical, laboratory, and EMG findings. RESULTS: The frequencies of malignancies associated with dermatomyositis and polymyositis were 22% and 8%, respectively. Patients with malignancies showed a significantly higher incidence of dysphagia (odds ratio [OR], 21.50; 95% confidence interval [CI], 3.84 to 120.49), absence of interstitial lung disease (ILD; OR, 0.12; 95% CI, 0.01 to 0.98), and complex repetitive discharge (CRD) on the EMG (OR, 26.25; 95% CI, 2.67 to 258.52), versus those without. After adjustment for age, dysphagia and CRD remained significant, while ILD showed a trend for a difference but was not statistically significant. Multivariate analysis revealed that the CRD conferred an OR of 25.99 (95% CI, 1.27 to 531.86) for malignancy. When the frequency of malignancy was analyzed according to the number of risk factors, patients with three risk factors showed a significantly higher incidence of malignancy, versus those with fewer than two (p = 0.014). CONCLUSIONS: We demonstrated for the first time that CRD on the EMG was an additional independent risk factor for malignancy in IIM. Further studies on a larger scale are needed to confirm the importance of CRD as a risk factor for malignancy in IIM.

A Case of Prostate Cancer Associated with Dermatomyositis and Interstitial Lung Disease / 대한내과학회지

Dermatomyositis (DM) is a kind of systemic autoimmune disease characterized by chronic inflammation leading to progressive weakness of proximal muscles and typical cutaneous lesions. DM has been known to be strongly associated with malignancies, such as ovarian, lung, and gastric cancers. Prostate cancer is rarely associated with DM and, to our knowledge, no case of prostate cancer has been reported in patients with DM combined with interstitial lung disease in Korea. Here, we report a case of DM with bronchiolitis obliterans organizing pneumonia that was simultaneously diagnosed as advanced prostate cancer by a thorough evaluation for hidden malignancy.

A Phase I Study of Human Cord Blood-Derived Mesenchymal Stem Cell Therapy in Patients with Peripheral Arterial Occlusive Disease

BACKGROUND AND OBJECTIVES: Half of patients with critical limb ischemia (CLI) are ineligible for revascularization at diagnosis. The aim of this study was to assess the safety and feasibility of intramuscular human umbilical cord blood-derived mesenchymal stem cell (hUCB-MSC) therapy in patients with CLI due to atherosclerosis obliterans (ASO) or thromboangiitis obliterans (TAO). METHODS AND RESULTS: A total of eight patients (all male, median age 52 years, range 31~77) with CLI were enrolled in this phase I trial. All patients were considered ineligible for further revascularization to improve CLI. We injected 1x10(7) hUCB-MSCs per single dose intramuscularly into the affected limb. The primary end points of safety were occurrence of adverse events (procedure-related complication, allergic reaction to hUCB-MSCs, graft-versus-host disease, cardiovascular and cerebrovascular events) and improvement of symptoms/clinical parameters (healing of foot ulcer, ankle-brachial index, and pain-free walking distance). Angiogenesis was measured with conventional angiography and scored by an independent reviewer. There were four adverse events in three patients. One patient, developed whole body urticaria after injection on treatment day, which disappeared after one day of antihistamine treatment. The other adverse events included diarrhea, oral ulceration, and elevation of serum creatinine level; all conditions improved without treatment. Abnormal results of laboratory parameters were not detected in any patients. Three of four ulcerations (75%) healed completely. Angiographic scores increased in three of eight patients. CONCLUSIONS: This phase I study demonstrates that intramuscular hUCB-MSC injection is a safe and well tolerated treatment for patients with end-stage CLI due to ASO and TAO.

Hypercalcemia Refractory to Pamidronate Associated with Metastatic Calcifications in Multiple Organs / 대한내과학회지

Paget's disease of bone (PDB) is a disorder featuring high-level bone turnover associated with the presence of disorganized and immature bone tissue with excessive levels of fibrosis. The risk of deformity is very high. The etiology of PDB is not well understood, but includes both genetic and environmental factors among which is bone trauma. Hypercalcemia can occur as a complication of PDB in patients who are immobilized and dehydrated. However, to date, no case of severe hypercalcemia with metastatic calcifications in multiple organs has been reported in any PDB patient. The drugs of choice for treatment of PDB are bisphosphonates. These drugs effectively suppress bone turnover. Patients with extensive PDB may require higher doses of bisphosphonates, and acquired resistance to a particular bisphosphonate may be overcome by use an alternative drug. Here, we report a case of suspicion of PDB. The patient presented with hypercalcemia and metastatic calcifications and his condition improved dramatically after treatment with zoledronic acid.

Hypercalcemia Refractory to Pamidronate Associated with Metastatic Calcifications in Multiple Organs / 대한내과학회지

Paget's disease of bone (PDB) is a disorder featuring high-level bone turnover associated with the presence of disorganized and immature bone tissue with excessive levels of fibrosis. The risk of deformity is very high. The etiology of PDB is not well understood, but includes both genetic and environmental factors among which is bone trauma. Hypercalcemia can occur as a complication of PDB in patients who are immobilized and dehydrated. However, to date, no case of severe hypercalcemia with metastatic calcifications in multiple organs has been reported in any PDB patient. The drugs of choice for treatment of PDB are bisphosphonates. These drugs effectively suppress bone turnover. Patients with extensive PDB may require higher doses of bisphosphonates, and acquired resistance to a particular bisphosphonate may be overcome by use an alternative drug. Here, we report a case of suspicion of PDB. The patient presented with hypercalcemia and metastatic calcifications and his condition improved dramatically after treatment with zoledronic acid.

Over-expression of extracellular superoxide dismutase in mouse synovial tissue attenuates the inflammatory arthritis

Oxidative stress such as reactive oxygen species (ROS) within the inflamed joint have been indicated as being involved as inflammatory mediators in the induction of arthritis. Correlations between extracellular-superoxide dismutase (EC-SOD) and inflammatory arthritis have been shown in several animal models of RA. However, there is a question whether the over-expression of EC-SOD on arthritic joint also could suppress the progression of disease or not. In the present study, the effect on the synovial tissue of experimental arthritis was investigated using EC-SOD over-expressing transgenic mice. The over-expression of EC-SOD in joint tissue was confirmed by RT-PCR and immunohistochemistry. The degree of the inflammation in EC-SOD transgenic mice was suppressed in the collagen-induced arthritis model. In a cytokine assay, the production of pro-inflammatory cytokines such as, IL-1beta, TNFalpha, and matrix metalloproteinases (MMPs) was decreased in fibroblast-like synoviocyte (FLS) but not in peripheral blood. Histological examination also showed repressed cartilage destruction and bone in EC-SOD transgenic mice. In conclusion, these data suggest that the over-expression of EC-SOD in FLS contributes to the activation of FLS and protection from joint destruction by depressing the production of the pro-inflammatory cytokines and MMPs. These results provide EC-SOD transgenic mice with a useful animal model for inflammatory arthritis research.

Identification of Independent Predictive Factors for Atherosclerosis in Rheumatoid Arthritis: Based on KARRA Cohort Study

OBJECTIVE: This study sought to investigate independent predictive factors for subclinical atherosclerosis in Korean patients with rheumatoid arthritis (RA). METHODS: We used high-resolution B-mode ultrasonography to measure the carotid artery intima-media thickness (IMT) and carotid plaque in 367 patients with RA. Detailed information on the demographic characteristics, cardiovascular (CV) risk factors, and RA disease characteristics were collected on all subjects. The relationship of the carotid artery IMT and carotid plaque to relevant clinical and laboratory variables were examined. RESULTS: Old age and male sex had the most significant association with increased IMT and presence of plaque than other factors. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and mKHAQ (Korean version of modified health assessment questionnaire) were significantly associated with both increased IMT and presence of plaque after univariate analysis adjusting for age and sex. A multivariable logistic regression analysis revealed that ESR and TJC68 were independent factors associated with the presence of plaque (p<0.001 and p=0.019, respectively). There was a significant linear correlation between the number of plaques and ESR (p<0.001 and R2=0.07). CONCLUSION: Our results indicated that markers of systemic inflammation contributed significantly to subclinical atherosclerosis in patients with RA. We emphasize the need for aggressive control of RA disease activity in patients who persistently demonstrate highly elevated ESR levels.

Natural History of Type II Endoleaks after Endovascular Aneurysm Repair in Abdominal Aortic Aneurysm / 대한혈관외과학회지

PURPOSE: Endovascular aneurysm repair (EVAR) for the treatment of abdominal aortic aneurysm (AAA) has shown excellent early outcomes. However, long-term durability continues to be questioned because of endoleaks. The optimal management of type II endoleaks remains controversial because little is known about their long-term natural history. The purpose of this study was to evaluate the natural history of type II endoleaks and to study factors associated with persistent type II endoleaks. METHODS: On a retrospective basis, we analyzed 98 patients who underwent EVAR for incidence and outcome of type II endoleaks. Patients with type II endoleaks were evaluated using computed tomography angiography or Duplex scans at one, 6 and 12 months after their EVAR, and annually thereafter to evaluate both the persistence of the endoleak and the size of the aneurysm sac. RESULTS: Type II endoleaks were detected during the follow-up periods in 38 patients (39.8%), who underwent EVAR (mean follow-up: 23.7 months). Spontaneous sealing of type II endoleaks by 6 months after EVAR occurred in 15 patients (39.4%), meaning that there were 23 patients (60.6%) whose leaks were ongoing for more than 6 months. Four patients were treated using embolization because of an enlarging aneurysm sac. CONCLUSION: Most type II endoleaks are transient and do not require intervention. However, particularly persistent endoleaks could lead to aneurysm enlargement and to delayed aortic rupture. We did not find a significant difference in any number of preoperative patient factors between patients with transient, persistent, or no type II endoleaks. Further studies based on independent data sets are needed to validate these results.

Three dimensional cone-beam CT study of upper airway change after mandibular setback surgery for skeletal Class III malocclusion patients / 대한치과교정학회지

OBJECTIVE: Lateral cephalometric radiographs have been the main form of resource for assessing two dimensional anteroposterior airway changes. The purpose of this study was to evaluate the three dimensional volumetric change in the upper airway space in Class III malocclusion patients who underwent mandibular setback surgery. METHODS: Three dimensional cone-beam computed tomographs (CBCT) and their three dimensional reconstruction images were analyzed. The samples consisted of 20 adult patients (12 males and 8 females) who were diagnosed as skeletal Class III and underwent mandibular setback surgery. CBCTs were taken at 3 stages - Baseline (1.8 weeks before surgery), T1 (2.3 months after surgery), and T2 (1 year after surgery). Pharyngeal airway was separated according to the reference planes and reconstructed into the nasopharynx, the oropharynx and the hypopharynx. Measurements at Baseline, T1, and T2 were compared between groups. RESULTS: The result showed the volume of the pharyngeal airway decreased significantly 2.3 months after surgery (p < 0.001) and the diminished airway did not recover after 1 year post-surgery. The oropharynx was the most decreased area. CONCLUSIONS: These findings suggest that mandibular setback surgery causes both short-term and long-term decrease in the upper airway space.

Prevalence and Correlates of Depressive Symptoms among North Korean Defectors Living in South Korea for More than One Year

OBJECTIVE: This study examined the prevalence and correlates of depressive symptoms in North Korean defectors who have been living in South Korea for more than one year. METHODS: We used questionnaires developed by the authors to collect sociodemographic data in addition to the Center for Epidemiologic Studies Depression Scale (CES-D), the Psychosocial Well-being Index to measure stress, and a social support scale. A total of 367 subjects were included in this study. RESULTS: The results showed that 30.5% of the men and 34.7% of the women reported depressive symptoms, and 33.1% of the men and 36.1% of the women exhibited signs of severe distress. Correlates of depressive symptoms were lack of occupation [odds ratio (OR)=2.198, 95% confidence interval (CI), 1.247-3.873], having escaped without family (OR=1.725, 95% CI, 1.006-2.959), and a poor subjective sense of health status (OR=3.111, 95% CI, 1.591-6.085). CONCLUSION: Continuing vocational training and career management, psychological support programs, and intensive physical health services are needed to improve the mental health of this population.

A Case of Steroid-responsive C1q Nephropathy Presenting as Focal Segmental Glomerulosclerosis / 대한신장학회지

C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobins and complement deposits, most notably C1q, and the absence of clinical and laboratory evidence of systemic lupus erythematosus. Most patients with C1q nephropathy present nephrotic-range proteinuria, which has a poor response to steroid. Some patients may experience decreased renal function and progress to end stage renal disease. A 27year-old man presented with proteinuria and decreased mental state. The patient was hypertensive, with a blood pressure of 180/120 mmHg. Serum BUN/creatine was 18/1.8 mg/dL, and urinalysis revealed proteinuria (3+). Brain computed tomography showed right basal ganglial and intraventricular hemorrhage. The patient was treated with craniotomy and hematoma removal, and he also received carvedilol, losartan, nifedipine, and doxazosin for control of BP. Although his mental status recovered and blood pressure was controlled, the patient still showed subnephrotic proteinuria; therefore, renal biopsy was performed. Kidney biopsy showed segmental sclerosis in 3 out of 14 glomeruli, and mesangial C1q immunofluorescence positive staining. Electron microscopic findings revealed electron-dense deposits in the mesangium. The patient was treated with oral prednisolone, and proteinuria was alleviated after 8 weeks and remains in complete remission.