Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.
Adult ; Age Factors ; Aged ; Aged, 80 and over ; Antibodies, Antineutrophil Cytoplasmic/blood ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Hemorrhage/etiology ; Humans ; Kidney Failure/etiology ; Korea ; Lung Diseases/etiology ; Male ; Middle Aged ; Polyarteritis Nodosa/*diagnosis/drug therapy/mortality ; Pulmonary Alveoli/blood supply/pathology ; Retrospective Studies ; Steroids/therapeutic use ; Survival Analysis ; Treatment Outcome

A 31-year-old woman was referred to our hospital and diagnosed as overlap syndrome with systemic lupus erythematosus and dermatomyositis. After completing the fourth cycle of intravenous immunoglobulin therapy, the patient developed acute confusional state with the Glasgow Coma Scale of 7. Considering the lack of response to high dose corticosteroid therapy (methylprednisolone 1 g per day for 3 days), rituximab (500 mg per week) was administered twice. The next day after the administration of the first dose of rituximab, the level of consciousness started to improve and 15 days after rituximab, mental status was fully recovered. The proportion of CD19+ B cells started to decrease within 1 week after the administration of rituximab and remained depleted for 14 weeks. There was also a gradual decrease in serum CD40 and CD80 concentration measured by ELISA up to 4 months. This case suggests the effect of rituximab for the treatment of neuropsychiatric lupus.
Female ; Humans

PURPOSE: We investigated the prevalence of lower urinary tract symptoms (LUTS), and the inconvenience of each symptom, using an ICS-male Questionnaire (ICS-mQ) in Korean man. We also evaluated the relationships between symptoms, prostate volume and urodynamic parameters of a bladder outlet obstruction (BOO). MATERIALS AND METHODS: 180 male LUTS patients, from 7 urological institutions, were enrolled in this study. The ICS-mQ was used to measure the severity of the symptoms, with a bothersomeness scale. Transrectal sonography and urodynamic studies, including Schaefer linear passive urethral resistance relation (L-PURR), were performed. RESULTS: The 5 most frequent symptoms were voiding symptoms, such as reduced stream, terminal dribbling, incomplete emptying, intermittency and hesitancy, in that order. However, the 5 most bothersome symptoms included 2 voiding, and 3 storage, symptoms, such as incomplete emptying, urge incontinence, pain in bladder, nocturnal incontinence and hesitancy. 34.4% of the nocturia patients felt quite, or severely, bothered, but only 1.1% felt no problem. The mean value of estimated prostate volume, by sonography, was 29.8+/-14.1ml. There were no correlations between the severity of symptoms and the prostate size. There were also little, or no, correlations between a wide range of symptoms and the urodynamic parameters of BOO. CONCLUSIONS: The most frequent symptoms of male patients with LUTS are voiding symptoms, but they suffered more from storage, than from voiding, symptoms. The severities of the symptoms, or the bothersomeness, were not correlated with the prostate size or the urodynamic parameters of a bladder outlet obstruction. Therefore, the relief of a bladder outlet obstruction is not crucial to relieve the symptoms.
Humans ; Korea* ; Lower Urinary Tract Symptoms* ; Male ; Nocturia ; Prevalence ; Prostate ; Surveys and Questionnaires* ; Rivers ; Urinary Bladder Neck Obstruction* ; Urinary Bladder* ; Urinary Incontinence, Urge ; Urodynamics

PURPOSE: We investigated the prevalence of lower urinary tract symptoms (LUTS), and the inconvenience of each symptom, using an ICS-male Questionnaire (ICS-mQ) in Korean man. We also evaluated the relationships between symptoms, prostate volume and urodynamic parameters of a bladder outlet obstruction (BOO). MATERIALS AND METHODS: 180 male LUTS patients, from 7 urological institutions, were enrolled in this study. The ICS-mQ was used to measure the severity of the symptoms, with a bothersomeness scale. Transrectal sonography and urodynamic studies, including Schaefer linear passive urethral resistance relation (L-PURR), were performed. RESULTS: The 5 most frequent symptoms were voiding symptoms, such as reduced stream, terminal dribbling, incomplete emptying, intermittency and hesitancy, in that order. However, the 5 most bothersome symptoms included 2 voiding, and 3 storage, symptoms, such as incomplete emptying, urge incontinence, pain in bladder, nocturnal incontinence and hesitancy. 34.4% of the nocturia patients felt quite, or severely, bothered, but only 1.1% felt no problem. The mean value of estimated prostate volume, by sonography, was 29.8+/-14.1ml. There were no correlations between the severity of symptoms and the prostate size. There were also little, or no, correlations between a wide range of symptoms and the urodynamic parameters of BOO. CONCLUSIONS: The most frequent symptoms of male patients with LUTS are voiding symptoms, but they suffered more from storage, than from voiding, symptoms. The severities of the symptoms, or the bothersomeness, were not correlated with the prostate size or the urodynamic parameters of a bladder outlet obstruction. Therefore, the relief of a bladder outlet obstruction is not crucial to relieve the symptoms.
Humans ; Korea* ; Lower Urinary Tract Symptoms* ; Male ; Nocturia ; Prevalence ; Prostate ; Surveys and Questionnaires* ; Rivers ; Urinary Bladder Neck Obstruction* ; Urinary Bladder* ; Urinary Incontinence, Urge ; Urodynamics

OBJECTIVE: Although increased expression of receptor for advanced glycation end products (AGE) in osteoarthritis (OA) has been reported, little is known concerning the role of AGEs in the pathogenesis of OA. This study was undertaken to determine the effect of AGEs on the regulation of matrix metalloproteinase (MMP) expressions and activities in human OA chondrocytes METHODS: OA chondrocytes were treated with increasing doses of AGE-bovine serum albumin (AGE-BSA). The expressions of MMPs were determined by both enzyme-linked immunosorbent assay (ELISA) and immunoblot analysis. The activities of MMPs were evaluated by both gelatin and casein zymography assays. In addition, electrophoretic mobility shift assay (EMSA) was employed to investigate the DNA binding activity of nuclear factor-kappa B (NF-kappaB) by AGE-BSA treatment. RESULTS: The productions of MMP-1, -3, and -13 were significantly elevated by AGE-BSA in a dose dependent manner. The elevated activities of MMP-1, -3, and -13, and TNF-alpha by AGE-BSA were also observed. DNA binding activity of NF-kappaB was markedly increased by AGE-BSA treatment implicating possible involvement of NF-kappaB mediated pathway in the AGE-BSA induced MMP-1, -3, and -13, and TNF-alpha productions in OA chondrocytes. Taken together, this study demonstrates the stimulatory effect of AGE-BSA on the productions of MMPs and TNF-alpha and suggests the possible involvement of NF-kappaB mediated pathway in OA chondrocytes. CONCLUSION: These results suggest that AGE may play a role in pathogenesis of OA.
Caseins ; Chondrocytes* ; DNA ; Electrophoretic Mobility Shift Assay ; Enzyme-Linked Immunosorbent Assay ; Gelatin ; Glycosylation End Products, Advanced* ; Humans* ; Matrix Metalloproteinases* ; NF-kappa B ; Osteoarthritis ; Serum Albumin ; Tumor Necrosis Factor-alpha

Macrophage activation syndrome (MAS) is a secondary hemophagocytic lymphohistiocytosis caused by autoimmune diseases, such as systemic lupus erythematosus (SLE). It is characterized by fever, cytopenia, coagulopathy, hepatosplenomegaly, elevated liver enzyme, and high ferritin, typically combined with hemophagocytic histiocyte proliferation in the bone marrow. Here, we report a case of MAS in a patient with SLE treated successfully by tocilizumab. She was transferred to our hospital due to persistent fever of unknown origin. Initial blood tests revealed cytopenia, elevated liver enzyme, and high ferritin. Bone marrow histology revealed the presence of hemophagocytic histiocytes. The patient was initially treated with high dose corticosteroids; however, fever and cytopenia were not controlled. Additional treatments with cyclosporine, intravenous immunoglobulin, and rituximab were applied consecutively, but the fever and cytopenia persisted. Symptom resolution was finally achieved following treatment with tocilizumab, resulting in rapid improved of fever, and resolution of pancytopenia within 2 months.
Adrenal Cortex Hormones ; Autoimmune Diseases ; Bone Marrow ; Cyclosporine ; Ferritins ; Fever ; Fever of Unknown Origin ; Hematologic Tests ; Histiocytes ; Humans ; Immunoglobulins ; Liver ; Lupus Erythematosus, Systemic* ; Lymphohistiocytosis, Hemophagocytic ; Macrophage Activation Syndrome* ; Macrophage Activation* ; Macrophages* ; Pancytopenia ; Rituximab

Plasma cell granuloma, a form of inflammatory pseudotumor, is a rare nonneoplastic lesion that is characterized by cellular proliferation composed predominantly of polyclonal plasma cells with other inflammatory cells in fibrovascular background. We have experienced an unusual case of plasma cell granuloma of skull that developed in a patient with systemic lupus erythematosus (SLE). A 42-year-old female diagnosed with SLE 13 years ago has complained of palpable scalp mass and headache beginning ten days previously. A brain magnetic resonance imaging showed intensely enhanced soft tissue mass with focal bone defect in right parietal bone and whole body positron emission tomography suggested high possibility of malignancy. Surgical tumor removal was performed. Biopsy specimen revealed inflammatory proliferation predominantly with mature plasma cells which were determined to be polyclonal in immunostaining. Currently, 4 months after surgery, the patient are on regular follow-up with oral medications (prednisolone, hydroxychloroquine) for SLE without evidence of recurrence.
Adult ; Biopsy ; Brain ; Cell Proliferation ; Female ; Follow-Up Studies ; Granuloma, Plasma Cell* ; Headache ; Humans ; Lupus Erythematosus, Systemic* ; Magnetic Resonance Imaging ; Parietal Bone ; Plasma Cells* ; Plasma* ; Positron-Emission Tomography ; Recurrence ; Scalp ; Skull*

OBJECTIVE: Despite the high risk for disease-related morbidity and mortality in Behcet's disease (BD) with cardiovascular (CV) manifestations, only a few studies concerning BD with CV involvements are available. We conducted study to evaluate the clinical manifestations of CV BD (cardiovascular Behcet's disease) and the clinical outcome according to the different treatment modalities, especially focusing on the immunosuppressive agents. METHODS: We retrospectively reviewed 1,812 patients diagnosed with BD at tertiary hospital. All patients with vascular involvements were classified into three groups by lesion site. We assessed clinical characteristics, treatments, outcome and recurrence in each group. RESULTS: Of 1,812 patients, 79 patients showed CV involvements. Male to female ratio was 65 (82.3%) to 14 (17.7%). Venous involvements occurred in 57 cases (72.2%), arterial lesions in 22 (27.8%), cardiac involvements 16 (20.3%). In clinical manifestation, only hypertension and arthritis were more frequently found in cardiac lesion than in venous lesion (p=0.01, p=0.01, respectively). CV lesions recurred in 16 patients (20.3%), mostly at the same sites as previous involvements. There was no association of recurrence with site of lesion (p=0.49). Recurrent rate was significant different in three medication group (p=0.028). Recurrences were more frequent in patients treated with no immunosuppressive agent and colchicines only or colchicines with prednisolone than in patients treated with additional immunosuppressive agent (p=0.024, R.R, 7.16 (95% CI, 1.55 to 32.99)). CONCLUSION: Recurrence rate was lower in patients with aggressive immunosuppressive treatment. Although most of patients improved, more efforts to decrease the relatively high rate of the recurrence (20.3%) would be needed.
Arthritis ; Cardiovascular Diseases ; Female ; Humans ; Hypertension ; Immunosuppressive Agents* ; Male ; Mortality ; Prednisolone ; Recurrence* ; Retrospective Studies ; Tertiary Care Centers

BACKGROUND AND PURPOSE: Thrombectomy within 24 hours can improve outcomes in selected patients with a clinical-infarct mismatch. We devised an easy-to-use visual estimation tool that allows infarct volume estimation in centers with limited resources. METHODS: We identified 1,031 patients with cardioembolic or large-artery atherosclerosis infarction on diffusion-weighted images (DWIs) obtained before recanalization therapy and within 24 hours of onset, and occlusion of the internal carotid or middle cerebral artery. Acute DWIs were mapped onto a standard template and used to create visual reference maps with known lesion volumes, which were then used in a validation study (with 130 cases) against software estimates of infarct volume. RESULTS: The DWI reference map chart comprises 144 maps corresponding to 12 different infarct volumes (0.5, 1, 2, 3, 5, 7, 9, 11, 13, 15, 17, and 19 mL) in each of 12 template slices (Montreal Neurological Institute z-axis –15 to 51 mm). Infarct volume in a patient is estimated by selecting a slice with a similar infarct size at the corresponding z-axis level on the reference maps and then adding up over all slices. The method yielded good correlations to software volumetrics and was easily learned by both experienced and junior physicians, with approximately 1 to 2 minutes spent per case. The sensitivity, specificity, and accuracy for detecting threshold infarct volumes ( < 21, < 31, and < 51 mL) were very high (all about >90%). CONCLUSIONS: We developed easy-to-use reference maps that allow prompt and reliable visual estimation of infarct volumes for triaging patients to thrombectomy in acute stroke.
Atherosclerosis ; Cerebral Infarction ; Decision Making ; Diffusion Magnetic Resonance Imaging ; Humans ; Infarction ; Medical Staff, Hospital ; Methods ; Middle Cerebral Artery ; Sensitivity and Specificity ; Stroke ; Thrombectomy