Sweet syndrome is an unusual disease characterized by the sudden onset of fever, leukocytosis, and painful erythematous plaques, and the dermal infiltration of neutrophils at the site of skin lesions. Although Sweet syndrome can also present with extra-cutaneous manifestations, involvement of the central nervous system (CNS) is rarely reported. We describe a case of Sweet syndrome involving the CNS in a 46-year-old male with a disturbance of consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid examination disclosed neutrophilic pleocytosis without decreased glucose and protein levels. HLA typing showed B54, which is frequently seen in Sweet syndrome. Brain magnetic resonance imaging showed abnormal signal intensity lesions in the left temporal lobe. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet syndrome. The confused mentality, fever, and erythematous skin plaques resolved after the administration of systemic corticosteroids.
Biopsy ; Brain ; Central Nervous System ; Consciousness ; Extremities ; Fever ; Glucose ; Histocompatibility Testing ; Humans ; Leukocytosis ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neutrophils ; Skin ; Sweet Syndrome ; Temporal Lobe

Nontuberculous mycobacteria are ubiquitous organisms that are frequently present in the water, soil and animal reservoirs. Nontuberculous mycobacterial infections of the musculoskeletal system are rare and usually associated with predisposing factors, such as prior joint disease, trauma, use of intraarticular or oral corticosteroids, or an immunocompromised state. A sixty five-year-old patient with rheumatoid arthritis was hospitalized due to swelling on the left wrist. M. intracellulare was cultured from the aspirated joint fluid. The patient was successfully treated with clarithromycin, ethambutol, and rifampin. We report this case with review, emphasizing high suspicion for nontuberculous mycobacterial infection in patients with predisposing risk factors.
Adrenal Cortex Hormones ; Animals ; Arthritis, Rheumatoid* ; Causality ; Clarithromycin ; Ethambutol ; Humans ; Joint Diseases ; Joints ; Musculoskeletal System ; Mycobacterium avium Complex* ; Mycobacterium* ; Nontuberculous Mycobacteria ; Rifampin ; Risk Factors ; Soil ; Tenosynovitis* ; Wrist

Nontuberculous mycobacteria are ubiquitous organisms that are frequently present in the water, soil and animal reservoirs. Nontuberculous mycobacterial infections of the musculoskeletal system are rare and usually associated with predisposing factors, such as prior joint disease, trauma, use of intraarticular or oral corticosteroids, or an immunocompromised state. A sixty five-year-old patient with rheumatoid arthritis was hospitalized due to swelling on the left wrist. M. intracellulare was cultured from the aspirated joint fluid. The patient was successfully treated with clarithromycin, ethambutol, and rifampin. We report this case with review, emphasizing high suspicion for nontuberculous mycobacterial infection in patients with predisposing risk factors.
Adrenal Cortex Hormones ; Animals ; Arthritis, Rheumatoid* ; Causality ; Clarithromycin ; Ethambutol ; Humans ; Joint Diseases ; Joints ; Musculoskeletal System ; Mycobacterium avium Complex* ; Mycobacterium* ; Nontuberculous Mycobacteria ; Rifampin ; Risk Factors ; Soil ; Tenosynovitis* ; Wrist

Kikuchi-Fujimoto disease (KFD) is a rare self-limiting necrotizing lymphadenitis found mainly in young women. Patients typically present with lymphadenopathy and often with a high temperature. The etiology of the disease remains unknown, but various viral infection and autoimmune processes have been postulated to be the cause. One theory proposes that KFD may be a self-limiting form of systemic lupus erythematosus (SLE). This theory is strongly supported by the fact that microscopic features of KFD can be very similar to those found in lupus lymphadenitis. The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. No treatment is usually needed and relapse, although possible, is uncommon. Our case describes a young woman, originally diagnosed as having SLE, who subsequently developed recurrent KFD with axillary lymphadenopathy and fever.
Diagnosis ; Female ; Fever ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Lupus Erythematosus, Systemic ; Lymphadenitis ; Lymphatic Diseases ; Recurrence

BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.
Arteries ; Atherosclerosis ; Carotid Arteries ; Cerebrovascular Disorders ; Demography ; Epidemiology ; Humans ; Hypertension ; Korea ; Magnetic Resonance Imaging ; Neuroimaging ; Prospective Studies* ; Registries ; Risk Factors ; Smoke ; Smoking ; Stroke*

BACKGROUND: H. pylori-associated gastrointestinal diseases have been widely recognized. The aim of this study was to investigate the seropositivity of H. pylori in health check-up subjects and to find out the relating factors. METHODS: From November 2004 through June 2005, total 7,676 health check-up subjects (age > or =20) responded to the self administered questionnaires. The prevalence of H. pylori was assessed by measuring anti-H. pylori IgG antibodies. RESULTS: The overall seropositivity was 56.7% in 7,676 and 1,137 (14.8%) has been found to have history of H. pylori eradication therapy. The seropositivity rate of H. pylori was 61.3% (2,653) in 4,328 subjects without history of H. pylori eradication and current gastrointestinal symptoms. Seroprevalence of H. pylori was significantly lower in 20~29 years old, female, high income and subjects from Seoul respectively. CONCLUSIONS: The seropositivity of H. pylori in 2004~2005 is found to be 61.3% in subjects without history of H. pylori eradication and current gastrointestinal symptoms. This seems to be lower than 66.9%, the seroprevalence rate in asymptomatic Korean population in the age of > or =16 years in 1998. This decrease might be caused by improvement of socioeconomic status.
Antibodies ; Female ; Gastrointestinal Diseases ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoglobulin G ; Prevalence ; Seoul ; Seroepidemiologic Studies* ; Social Class ; Surveys and Questionnaires

OBJECTIVE: Multipotent bone marrow stromal cells have the ability to differentiate toward a variety of connective tissue lineages including cartilage. The future use of adult mesenchymal stem cells (MSCs) for human therapies depends on the establishment of preclinical studies. Therefore, in this preclinical study we demonstrated the expression of MSC surface markers CD29, CD105, and CD44 on human bone marrow derived stromal cells during chondrogenic differentiation. METHODS: Adult human bone marrow was collected from the iliac crest of 7 donors following informed consent. Mononuclear cells were isolated, incubated in monolayers, and embedded in alginate beads for three-dimensional cultures. Cellualr viability was assessed by MTT assay. Flow cytometry of alginate bead cultures was performed on days 0, 7, 14, 21, and 28 using monoclonal antibody against surface molecules, CD105, CD29, CD44, CD34 and CD45. Total contents of collagen and glycosaminoglycan (GAG) of the alginate beads was measured. SPSS 11.0 was used for data analysis. RESULTS: After 7 days of culture, 89% of the cells expressed the human integrin beta 1 antibody, CD29. The CD29-positive cells remained elevated at 83% on days 28. However, while only 18% expressed the type II TGF-beta receptor endoglin, CD105 on day 7, the CD105-positive cells increased abruptly 65% on day 14 remaining elevated up to day 28. The expression of CD44 was maximal in the first passage cell (63%). High concentration of TGF-beta 3 (10 ng/mL) was more favorable for sustaining cell viability than a low concentration (0.5 ng/mL)(n=4, p= 0.002, day 21). The total contents of collagen and GAG in the MSC-alginate beads increased during the three-dimensional culture (n=4, p=0.02, p=0.006) suggesting its differentiation into a chondrogenic lineage. CONCLUSION: CD29 was expressed earlier than CD105 during chondrogenic differentiation of human bone marrow MSC. CD44 expression was highest in the first passage cells and gradually decreased afterwards.
Adult ; Bone Marrow* ; Cartilage ; Cell Survival ; Collagen ; Connective Tissue ; Flow Cytometry ; Humans* ; Informed Consent ; Mesenchymal Stromal Cells ; Receptors, Transforming Growth Factor beta ; Statistics as Topic ; Stromal Cells* ; Tissue Donors ; Transforming Growth Factor beta

An "overlap syndrome" is used to describe patients who have two or more well-defined connective tissue diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and polymyositis. Their coexistence is defined clinically and often by specific serologic tests. We report a case of dermatomyositis associated with IgA nephropathy that had overlapping features of Sjogren's syndrome. This dermatomyositis and Sjogren's overlap is a rarely reported overlap syndrome worldwide.
Arthritis, Rheumatoid ; Connective Tissue Diseases ; Dermatomyositis* ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Lupus Erythematosus, Systemic ; Polymyositis ; Scleroderma, Systemic ; Serologic Tests ; Sjogren's Syndrome*

Up to 70% of patients with systemic lupus erythematosus (SLE) are afflicted with neurologic manifestations. However, there are only a few reports documenting acute leukoencephalopathy in SLE. We describe a 20-year-old woman who was recently diagnosed as SLE, suffering from headache, fever and arthritis. She developed an acute onset of consciousness disturbance with seizure followed by prolonged coma, which recovered completely after 1 month of steroid therapy. Her brain MRI showed diffuse high signal intensity in the periventricular and subcortical white matter on T2-weighted and FLAIR images, whereas cerebral cortex, basal ganglia, and thalamus were spared. Acute leukoencephalopathy may be recognized as a subtype of lupus involving the central nervous system.
Arthritis ; Basal Ganglia ; Brain ; Central Nervous System ; Cerebral Cortex ; Coma ; Consciousness ; Female ; Fever ; Headache ; Humans ; Leukoencephalopathies* ; Lupus Erythematosus, Systemic* ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Seizures ; Thalamus ; Young Adult

BACKGROUND: Leflunomide, a novel immunoregulatory drug, has been shown to be effective in rheumatoid arthritis (RA) as monotherapy and as combination therapy with methotrexate (MTX). The aims of this study were to investigate the efficacy and safety of combination therapy with leflunomide and MTX in active RA patients and to identify the patients with a better response to this combination. METHODS: The patients received a maintenance dose of 20 mg of leflunomide with or without a loading dose. Parameters for disease activity in RA were measured at baseline and at 12 and 24 weeks after initiation of leflunomide. At 24 weeks, the baseline data from the patients classified as leflunomide responders were compared with data from nonresponders and analyzed to determine the potential predisposing factors for treatment response. RESULTS: A total of 103 patients with RA were included and 93 (90.3%) patients received leflunomide for 24 weeks. At 24 weeks, 67 (65.1%) patients were DAS28 responders; 14 (13.6%) were good responders and 53 (51.5%) moderate responders. At 12 weeks, significant improvements were noticeable in the individual efficacy measures of diseases activity. There were also significant improvements between 12 and 24 weeks in swollen joint count, tender joint count, HAQ disability index, and patients' and physicians' global assessments of diseases activity; but no further improvements in ESR or CRP could be seen after the first 12 weeks. When comparing the baseline data from responders with the nonresponders, patients on a higher MTX dose and patients with a higher disease activity at baseline responded better to leflunomide. However, age, sex, disease duration of RA, functional status, loading dosage of leflunomide, and previous number of DMARDs used did not affect the patients' response to leflunomide. CONCLUSION: Combination therapy with leflunomide and MTX is effective and safe across a wide range of patients, especially those with a high disease activity in spite of treatment with other traditional DMARDs.
Antirheumatic Agents ; Arthritis, Rheumatoid* ; Causality* ; Humans ; Joints ; Methotrexate*