No abstract available.
Arthralgia* ; Hand*

No abstract available.
Cranial Nerve Diseases ; Granuloma, Plasma Cell* ; Polychondritis, Relapsing*

Catabacter hongkongensis is an anaerobic gram-positive coccobacillus that was first isolated in Hong Kong. It is infectious and causes high mortality in patients with rare but underlying diseases. Alistipes indistinctus is an anaerobic gram-negative coccobacillus. This bacterium is a common member of the human intestinal microbiota. We report a case of C. hongkongensis and A. indistinctus isolated from blood cultures of a patient with acute appendicitis. A 35-year-old female patient with no specific medical history was admitted to the hospital due to abdominal pain, vomiting, nausea, and diarrhea experienced on the day before admission. On admission, laboratory tests revealed leukocytosis, neutropenia, and elevated C–reactive protein and procalcitonin levels. Following an abdominal computed tomography showing acute appendicitis with suspected perforation, emergency surgery was performed. Growth was observed in two anaerobic blood culture bottles after four days. After further culturing of the bacteria on Brucella Blood Agar, two types of bacteria were obtained. The two bacterial isolates, one gram-positive and one gram-negative, were unable to be identified using matrix assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS). Thus, 16S rRNA gene sequence analysis was performed, resulting in identification of the bacteria as C. hongkongensis and A. indistinctus. The patient was administered antibiotics and discharged two days after surgery. Although MALDI-TOF MS enables fast and accurate identification of bacteria, C. hongkongensis and A. indistinctus were not listed in the spectral library, and 16S rRNA gene sequence analysis was useful for identifying the two bacteria.
Abdominal Pain ; Adult ; Agar ; Anti-Bacterial Agents ; Appendicitis ; Bacteria ; Brucella ; Diarrhea ; Emergencies ; Female ; Gastrointestinal Microbiome ; Genes, rRNA ; Hong Kong ; Humans ; Leukocytosis ; Mass Spectrometry ; Mortality ; Nausea ; Neutropenia ; Sequence Analysis ; Vomiting

High-mobility group box 1 (HMGB1) protein has been demonstrated to play an important role in chronic inflammatory diseases including rheumatoid arthritis, and systemic lupus erythematosus. This study investigated the association between extracellular HMGB1 expression and disease activity, and clinical features of Behcet's disease (BD). Extracellular HMGB1 expression in the sera of 42 BD patients was measured and was compared to that of 22 age- and sex-matched healthy controls. HMGB1 expression was significantly increased in BD patients compared to healthy controls (78.70 +/- 20.22 vs 10.79 +/- 1.90 ng/mL, P = 0.002). In addition, HMGB1 expression was significantly elevated in BD patients with intestinal involvement compared to those without (179.61 +/- 67.95 vs 61.89 +/- 19.81 ng/mL, P = 0.04). No significant association was observed between HMGB1 concentration and other clinical manifestations, or disease activity. It is suggested that extracellular HMGB1 may play an important role in the pathogenesis of BD.
Adult ; Aged ; Behcet Syndrome/genetics/*metabolism/pathology ; Extracellular Space/metabolism ; Female ; HMGB1 Protein/genetics/*metabolism ; Humans ; Inflammation ; Intestinal Diseases/blood/genetics ; Male ; Middle Aged ; Young Adult

Copy number variation has been associated with various autoimmune diseases. We investigated the copy number (CN) of the DEFA1 gene encoding alpha-defensin-1 in samples from Korean individuals with Behcet's disease (BD) compared to healthy controls (HC). We recruited 55 BD patients and 35 HC. A duplex Taqman(R) real-time PCR assay was used to assess CN. Most samples (31.1%) had a CN of 5 with a mean CN of 5.4 +/- 0.2. There was no significant difference in the CN of the DEFA1 gene between BD patients and HC. A high DEFA1 gene CN was significantly associated with intestinal involvement in BD patients. Variable DEFA1 gene CNs were observed in both BD patients and HC and a high DEFA1 gene CN may be associated with susceptibility to intestinal involvement in BD.
Adult ; Behcet Syndrome/*complications/*genetics ; Female ; Gene Dosage ; Genetic Predisposition to Disease ; Genotype ; Humans ; Intestinal Diseases/*etiology/*genetics ; Male ; Middle Aged ; Real-Time Polymerase Chain Reaction ; alpha-Defensins/*genetics

OBJECTIVE: This cross-sectional study aimed to investigate the association between rheumatoid factor (RF) positivity and bone mineral density (BMD) in male Korean subjects without any history of joint disease. METHODS: Of 84,344 males who had undergone a comprehensive health checkup program in 2012, 1,390 male health examinees were recruited, whose BMD and RF results were available. A RF titer ≥20 IU/mL was considered positive. BMD was measured at lumbar spine (L1~L4) or hip (femoral neck and total hip) by dual-energy X-ray absorptiometry. RESULTS: The association between RF positivity and BMD was assessed by multiple linear regression analysis. The mean age was 52.7±10.9 years (range 19~88 years), and RF was detected in 64 subjects (4.6%). Demographics and laboratory data were not different between RF-positive and -negative subjects except hepatitis B surface antigen (HBsAg), which was more frequently seen in RF-positive subjects (15.6% vs. 4.3%, p=0.001). RF-positive subjects had significantly lower BMD compared to RF-negative subjects in lumbar spine but not in total hip regardless of the existence of HBsAg (1.17±0.16 g/cm2 vs. 1.10±0.18 g/cm2, p=0.002 in total subjects; 1.17±0.16 g/cm2 vs. 1.10±0.18 g/cm2, p=0.004 in HBsAg-negative subjects). After adjusting for multiple confounders, RF positivity was negatively associated with lumbar spine BMD (B=−0.088 and standard error=0.035, p=0.011). CONCLUSION: Our results show that the presence of RF could have an unfavorable impact on bone density in apparently normal males. Additional studies to elucidate the osteoimmunological mechanism of rheumatoid factor are warranted.
Absorptiometry, Photon ; Arthritis* ; Bone Density ; Cross-Sectional Studies ; Demography ; Hepatitis B Surface Antigens ; Hip ; Humans ; Joint Diseases ; Linear Models ; Male* ; Men's Health ; Neck ; Rheumatoid Factor* ; Spine

We report on a case of limited form of granulomatosis with polyangiitis (GPA) with pituitary involvement which presented with central diabetes insipidus. This rare form of GPA has not been reported in Korea. The patient presented with fever, headache, productive cough, nasal symptoms, and polyuria. Laboratory data and imaging studies demonstrated inflammatory lesions in nasal sinus and lungs. Pituitary stalk thickening and enhancement were observed on brain magnetic resonance imaging. The histopathology of the lung lesions showed chronic active granulomatous inflammation. Polyuria, hyperosmolar hypernatremia, and decreased urine osmolality which responded to synthetic vasopressin analog were consistent with central diabetes insipidus. Based on the clinical findings and histopathological results, a diagnosis of GPA with pituitary involvement was established. Treatment with desmopressin as well as concurrent glucocorticoids and immunosuppressant resulted in clinical improvement.
Brain ; Cough ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic* ; Diagnosis ; Fever ; Glucocorticoids ; Headache ; Humans ; Hypernatremia ; Inflammation ; Korea ; Lung ; Magnetic Resonance Imaging ; Osmolar Concentration ; Pituitary Gland ; Polyuria ; Vasopressins

TNF-alpha antagonists have been successfully utilized in the treatment of autoimmune diseases, including psoriasis and psoriatic arthritis. Paradoxically, new onset or exacerbation of psoriatic lesions during treatment with TNF-alpha antagonists have been reported. It has been postulated that TNF-alpha blockade may cause disruption in the balance between TNF-alpha and type 1 interferon (IFN)-alpha, which are the key players in the pathogenesis of psoriasis. We report a case of psoriasis exacerbation during TNF-alpha antagonist therapy in a 53-years-old man with ankylosing spondylitis. The patient has been treated with etanercept for 3 years and 7 months when he developed accelerated deterioration of psoriasis. His condition was previously under control solely by local treatment. Physical examination revealed vigorous desquamative lesions with silvery scale in both lower legs. Deterioration of psoriasis was attributed to etanercept therapy and was subsequently discontinued. Clinical improvement of psoriasis has been observed 2 months following cessation of etanercept.
Arthritis, Psoriatic ; Autoimmune Diseases ; Humans ; Etanercept ; Immunoglobulin G ; Interferons ; Leg ; Physical Examination ; Psoriasis ; Receptors, Tumor Necrosis Factor ; Skin ; Spondylitis, Ankylosing ; Tumor Necrosis Factor-alpha

This study aimed to evaluate the frequency and clinical characteristics of hydroxychloroquine (HCQ) retinopathy in Korean patients with rheumatologic diseases. We retrospectively reviewed medical records of 310 patients taking HCQ. Ophthalmic examinations included spectral-domain optical coherence tomography (SD-OCT), automated visual field test, and fundus autofluorescence. The severity of retinopathy was categorized as early, moderate, or severe, and the location was categorized as parafoveal, pericentral, or mixed pattern. Among 310 patients, 9 patients (2.9%) were diagnosed as HCQ retinopathy. Among the patients with HCQ use ≥ 5 years (n = 174), the frequency was 5.2%. Only 1 (11.1%) of the 9 patients was symptomatic. The mean daily dose per kilogram of real body weight of the 9 patients was 5.6 mg, and only 3 had used 6.5 mg or more. Four of the 9 patients had severe HCQ retinopathy. Six of the 9 patients showed pericentral or mixed pattern of retinal damage. Consequently, the frequency of HCQ retinopathy in Korean patients was not low, especially when administered at a high cumulative dose and for a long duration. Screening of HCQ retinopathy by the recommended guidelines that include SD-OCT seems useful and should be done to detect retinal damage earlier in patients with chronic exposure to HCQ.
Body Weight ; Humans ; Hydroxychloroquine* ; Mass Screening ; Medical Records ; Retinaldehyde ; Retrospective Studies ; Tomography, Optical Coherence ; Visual Field Tests

OBJECTIVE: Polymyalgia rheumatica is a chronic inflammatory disease that affects people older than 50 years of age. The diagnosis is made based on clinical features, and the current standard of treatment is low-dose glucocorticoids. PMR is more commonly reported in Caucasians and females. However, epidemiological studies of PMR in Asian countries are scarce. We aimed to estimate the epidemiology of PMR in Korea. METHODS: This study was conducted by analyzing the Health Insurance Review and Assessment databases. We verified all claims between 2007 and 2012. Cases were included when PMR ICD-10 code (M 35.3) was recorded more than twice, and glucocorticoids were prescribed for > or =30 days. RESULTS: We identified 1,463 newly diagnosed cases of PMR during the 5 years. Among them, 992 (67.8%) were female, and the mean age at diagnosis was 66.9 years old. The annual incidence rate was 2.06 per 100,000 individuals aged over 50 years. The prevalence rate was 8.21 per 100,000 individuals in 2012. Incidence and prevalence appeared to increase with age. Prednisolone was the most commonly prescribed glucocorticoid. In half of the patients, the daily starting dose was 6~15 mg as prednisolone equivalents. CONCLUSION: This is the first study to investigate the epidemiology of PMR in Korea. The incidence and prevalence appeared to be considerably lower than those in Western populations. Both genetic and environmental factors might influence disease occurrence. In addition, the actual incidence may have been underestimated due to lack of awareness of PMR in clinical practice.
Asian Continental Ancestry Group ; Diagnosis ; Epidemiologic Studies ; Epidemiology* ; Female ; Glucocorticoids ; Humans ; Incidence ; Insurance, Health ; International Classification of Diseases ; Korea ; Polymyalgia Rheumatica* ; Prednisolone ; Prevalence