Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder associated with fibroinflammatory conditions that can affect multiple organs. Hallmark histopathological findings of IgG4-RD include lymphocytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. However, little is known about central nervous system involvement of IgG4-RD.Hypertrophic pachymeningitis (HP) has recently been reported as a manifestation of IgG4-RD, which may have previously been demonstrated in a significant percentage of idiopathic cases. Herein, we report a rare case of a 63-year-old male who presented with a scalp mass that mimicked a brain tumor. He was diagnosed with IgG4-related HP (IgG4-RP) after surgery. This case suggests that awareness of a possibility of IgG4-RP in patients with isolated scalp masses, even in the absence of systemic symptoms, is crucial. A combination of careful history taking, evaluation of serum IgG4-levels and imaging as an initial work-up, followed by tissue biopsy, is important for the differential diagnosis of IgG4-RP, malignancy, and other infectious diseases.

BACKGROUND/AIMS: We prepared a cross-cultural adaptation of the Behcet's Syndrome Activity Scale (BSAS) and evaluated its reliability and validity in Korea. METHODS: Fifty patients with Behcet's disease (BD) who attended the Rheumatology Clinic of Gachon University Gil Medical Center were included in this study. The first BSAS questionnaire was administered at each clinic visit, and the second questionnaire was completed at home within 24 hours of the visit. A Behcet's Disease Current Activity Form (BDCAF) and a Behcet's Disease Quality of Life (BDQOL) form were also given to patients. The test-retest reliability was analyzed by intraclass correlation coefficients (ICC). To assess the validity, the total BSAS score was compared with the BDCAF score, the patient/physician global assessment, and the BDQOL by Spearman rank correlation. RESULTS: Twelve males and 38 females were enrolled. The mean age was 48.5 years and the mean disease duration was 6.7 years. Thirty-eight patients (76.0%) returned the questionnaire by mail. For the test-retest reliability, the two assessments were significantly correlated on all 10 items of the BSAS questionnaire (p < 0.05) and the total BSAS score (ICC, 0.925; p < 0.001). The total BSAS score was statistically correlated with the BDQOL, BDCAF, and patient/physician global assessment (p < 0.01). CONCLUSIONS: The Korean version of BSAS is a reliable and valid instrument to measure BD activity.
Academic Medical Centers ; Adult ; Behcet Syndrome/*diagnosis/physiopathology/psychology ; Cost of Illness ; Cultural Characteristics ; Female ; Humans ; Male ; Middle Aged ; *Patient Reported Outcome Measures ; Predictive Value of Tests ; Quality of Life ; Reproducibility of Results ; Republic of Korea ; Severity of Illness Index

OBJECTIVE: This study measured the reliability of the Behcet's Disease Current Activity Form (BDCAF) questionnaire used as a patient self-report form. METHODS: A study was conducted among 63 patients with Behcet's disease who attended our rheumatology clinic. First, a physician administered a BDCAF questionnaire. Second, the patient completed a self-administered questionnaire at home within 24 hours of the visit. The test-retest reliability was analyzed using kappa tests. Kappa scores of >0.6 indicated good agreement. The BDCAF score was compared with the patient's/clinician's perception of disease activity and the Korean version of Behcet's Disease Quality of Life (BDQOL). RESULTS: The study included 17 males and 46 females. The mean age of participants was 47.7 years and the mean disease duration was 5.3 years at the first assessment. Fifty-three patients (84.1%) returned the questionnaires to us by mail. For test-retest reliability, good agreement was achieved with the items including headache, oral/genital ulceration, erythema, arthritis, and diarrhea with altered/frank blood per rectum; moderate agreement with skin pustules, arthralgia, and eye involvement; fair agreement with nausea/vomiting/abdominal pain, nervous system, and major vessel involvement. Significant associations were observed between BDCAF scores with the patient's/clinician's perception of disease activity and BDQOL (p<0.05). CONCLUSION: The BDCAF appears useful as a patient self-report instrument for assessment of disease status.
Arthralgia ; Arthritis ; Diarrhea ; Erythema ; Female ; Headache ; Humans ; Male ; Nervous System ; Postal Service ; Quality of Life ; Rectum ; Rheumatology ; Skin ; Ulcer

The spondyloarthritis (SpA) is a group of chronic inflammatory rheumatic diseases in association with HLA-B27. They share the clinical features including sacroiliitis, spondylitis, oligoarthritis, enthesitis and extra-articular involvement. Recently ASAS proposed new classification criteria sets of axial and peripheral SpA. They were designed to include non-radiographic SpA, thus can guide the early diagnosis of disease before the structural damage occurs. SpA has a strong genetic predisposition. Non-MHC genes, such as IL23R and ERAP1, as well as HLA-B27 were confirmed as susceptibility genes through several GWAS. Major pathology in SpA is entheseal inflammation and new bone formation. Intrinsic ability of HLA-B27 to trigger innate immune response and several proinflammtory cytokines may contribute to the inflammation in SpA. New bone formation could be explained by a mechanism, partly or completely independent of the inflammatory process.
Cytokines ; Early Diagnosis ; Genetic Predisposition to Disease ; HLA-B27 Antigen ; Immunity, Innate ; Inflammation ; Osteogenesis ; Rheumatic Diseases ; Sacroiliitis ; Spondylitis ; Spondylitis, Ankylosing

BACKGROUND/AIMS: This study was undertaken to perform a cross-cultural adaptation of the Behcet's Disease Current Activity Form (BDCAF, version 2006) questionnaire to the Korean language and to evaluate its reliability and validity in a population of Korean patients with Behcet's disease (BD). METHODS: A cross-cultural study was conducted among patients with BD who attended our rheumatology clinic between November 2012 and March 2013. There were 11 males and 35 females in the group. The mean age of the participants was 48.5 years and the mean disease duration was 6.4 years. The first BDCAF questionnaire was completed on arrival and the second assessment was performed 20 minutes later by a different physician. The test-retest reliability was analyzed by computing kappa statistics. Kappa scores of > 0.6 indicated a good agreement. To assess the validity, we compared the total BDCAF score with the patient's/clinician's perception of disease activity and the Korean version of the Behcet's Disease Quality of Life (BDQOL). RESULTS: For the test-retest reliability, good agreements were achieved on items such as headache, oral/genital ulceration, erythema, skin pustules, arthralgia, nausea/vomiting/abdominal pain, and diarrhea with altered/frank blood per rectum. Moderate agreement was observed for eye and nervous system involvement. We achieved a fair agreement for arthritis and major vessel involvement. Significant correlations were obtained between the total BDCAF score with the BDQOL and the patient's/clinician's perception of disease activity p < 0.05). CONCLUSIONS: The Korean version of the BDCAF is a reliable and valid instrument for measuring current disease activity in Korean BD patients.
Adult ; Asian Continental Ancestry Group/psychology ; Behcet Syndrome/*diagnosis/physiopathology/psychology ; Comprehension ; Cultural Characteristics ; Female ; Humans ; Language ; Male ; Middle Aged ; Observer Variation ; Predictive Value of Tests ; Reproducibility of Results ; Republic of Korea/epidemiology ; Severity of Illness Index ; *Surveys and Questionnaires

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 ± 11.1 and 14.2 ± 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 ± 11.4 years vs. 33.8±11.6 years, 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.
Age of Onset* ; Arthritis ; Behcet Syndrome* ; Continental Population Groups ; Female ; Gender Identity ; HLA-B51 Antigen* ; Humans ; Leukocytes ; Low Back Pain ; Male ; Phenotype* ; Retrospective Studies ; Rheumatology ; Skin ; Ulcer

Mucormycosis is a rare and fulminating opportunistic fungal infection that occurs almost in immunocom-promised patients. It is also a highly virulent and rapidly progressive disease with poor prognosis. Its incidence has been increasing in recent years. We have experienced a case of solitary duodenal mucormycosis in a 45 year-old male patient. Mucormycosis was diagnosed by pathology & culture and he was treated with amphotericin B and discharged with clinical improvement. We report this case with a literature review.
Amphotericin B ; Humans ; Incidence ; Male ; Middle Aged ; Mucormycosis* ; Pathology ; Prognosis

Klinefelter's syndrome is a disorder of sexual differentiation in males, characterized by the presence of two or more X-chromosomes, hypogonadism, and lack of secondary sexual characteristics. The association between Klinefelter's syndrome and systemic lupus erythematous has been described, while cases of rheumatoid arthritis associated with Klinefelter's syndrome are rare. We report the first Korean case: a 29-year-old man with Klinefelter's syndrome who developed rheumatoid arthritis. The sex hormone imbalance might have influenced the onset and course of his disease.
Adult ; Arthritis, Rheumatoid ; Gonadal Steroid Hormones ; Humans ; Hypogonadism ; Klinefelter Syndrome ; Male ; Sex Differentiation

Phlegmonous esophagitis is an uncommon disease characterized by purulent infection of the esophageal wall, sparing the mucosa. Bacterial infection of the eosphagus is usually presented as a superimposed infection upon a preexisting viral or fungal esophagitis and most victims are immunocompromised hosts. A case was experienced involving an acute phlegmonous esophagitis in an 21-year-old man who was immunologically normal and whose main symptoms were epigastric pain and fever for one day. Esophagographic examination revealed a large ulceration of the eosphagus with exudation, and submucosal lesions. Due to its rarity, this case is herein reported with a review of the corresponding literature.
Bacterial Infections ; Cellulitis* ; Esophagitis* ; Fever ; Humans ; Immunocompromised Host ; Mucous Membrane ; Ulcer ; Young Adult

PURPOSE: Bacille Calmette-Guerin (BCG) vaccination has been reported to be an effective treatment for asthma in several animal models. This study investigated whether the response to BCG treatment in asthma depends on subject clinical characteristics. METHODS: Stable asthma patients were vaccinated with BCG. One month later, alterations in pulmonary function after vaccination and their relationships with subject clinical characteristics were determined. RESULTS: Of 149 patients with asthma, 54 (36.2%) showed a good or fair response to BCG. The DeltaFEV1 after vaccination was significantly related to age (r=-0.348, P<0.001), peripheral blood eosinophil counts (r=0.315, P<0.001) and baseline FEV1, expressed as % personal best value (r=-0.474, P<0.001), but not to FEV1 % predicted value (r=-0.066, P>0.05). A good/fair response was highly prevalent in atopic females compared with atopic males, especially among those aged < or =50 years (90.9% vs. 40.0%, P=0.024). Age (P<0.001, odds ratios (OR)=0.92, confidence interval (CI)=0.88-0.96) and atopy (P<0.01, OR=4.95, CI=1.70-14.44) were significant predictors for a good/fair response in females. However, blood eosinophil counts (P<0.05, OR=1.18, CI=1.01-1.39) and FEV1 % best (P<0.001, OR=0.86, CI=0.79-0.94), but not age or atopy, were significant predictors in males. Approximately three-quarters of the males were smokers. CONCLUSIONS: The therapeutic effect of BCG in asthma may differ according to patient clinical characteristics. The greatest benefit occurred in young atopic females. Asthma activity indices, such as eosinophilia and FEV1 % best, were more predictive of a good/fair response in males; this may have been related to cigarette smoking.
Aged ; Asthma ; Eosinophilia ; Eosinophils ; Female ; Humans ; Male ; Models, Animal ; Mycobacterium bovis ; Odds Ratio ; Smoking ; Vaccination