Idiopathic hypereosinophilic syndrome (IHES) is a rare disease that is characterized by otherwise unexplained persistent eosinophilia and organ damage caused by eosinophilic infiltration. Its manifestations are highly variable but clinically apparent arthritis is uncommonly observed. Although Korean cases of severe eosinophilia in patients with rheumatoid arthritis (RA) or IHES concurrent with RA have been published, there are no reports of IHES with joint involvement. This paper reports a case of IHES presenting with persistent peripheral eosinophilia, fever, skin rash, multiple lymphadenopathy, and polyarthritis, including the distal interphalangeal joints of the hands.
Arthritis ; Arthritis, Rheumatoid ; Cyclosporine ; Eosinophilia ; Eosinophils ; Exanthema ; Fever ; Finger Joint ; Hand ; Humans ; Hypereosinophilic Syndrome* ; Joints ; Lymphatic Diseases ; Rare Diseases

BACKGROUND AND OBJECTIVES: There are conflicting results about ciliary activity in chronic rhinosinusitis (CRS). And dynamic movements reacting to various stimuli in sinusitis mucosa have been studied rarely. This study was designed to investigate the ciliary activity according to the severity of sinusitis and clinical symptoms. We aimed to identify the dynamic ciliary activity in response to purinergic stimulation in sinusitis. SUBJECTS AND METHOD: Nasal mucosal samples were obtained from 44 CRS and 20 normal subjects. We measured ciliary beat frequency (CBF) in the basal and activated status. For evaluating the correlation of CBF with clinical findings, nasal symptoms, endoscopic findings, CT findings, allergy tests, and olfactory tests were reviewed. RESULTS: No significant differences were found in basal CBF between normal and CRS mucosa. However, the CBF stimulated by ATP in normal and CRS mucosa showed marked differences: both the maximal increase and duration of increased CBF by ATP were significantly reduced in CRS mucosa. The olfactory test showed significant correlation with basal CBF, but the presence of allergy or nasal symptoms did not show significant correlation with the basal CBF. The CT score and presence of nasal polyposis showed negative correlation with the basal CBF (p<0.05). CONCLUSION: The basal CBF of normal and CRS mucosa showed no difference. However, dynamic ciliary activity responding to purinergic stimulation was markedly attenuated in sinusitis mucosa. The severity of sinusitis and presence of polyposis negatively affected CBF.
Adenosine Triphosphate ; Hypersensitivity ; Mucous Membrane ; Sinusitis

OBJECTIVE: To investigate longitudinal changes in the European League Against Rheumatism (EULAR) Sjögren's syndrome patient reported index (ESSPRI) and to study the clinical features associated with favorable ESSPRI changes in primary Sjögren's syndrome (pSS). METHODS: At baseline and after a median period of 6.6 years, 41 pSS patients were evaluated using the ESSPRI, EULAR Sjögren's syndrome disease activity index (ESSDAI), short-form 36, xerostomia inventory (XI), and visual analog scale (VAS) scores for symptoms. The favorable subgroup included patients who were stable or showed improved to satisfactory symptom status (ESSPRI<5) and the unfavorable subgroup included those with stable or worsening to an unsatisfactory symptom status (ESSPRI ≥5). RESULTS: Median ESSPRI increased from 4.11 to 5.33 (p<0.05), although XI scores (p=0.01) and oral dryness (p<0.05) were significantly decreased. Serum immunoglobulin G level was significantly reduced (p<0.001) but ESSDAI scores were unchanged. Six (14.6%) patients showed clinical improvement in ESSDAI, and 11 (26.8%) showed improvement in ESSPRI. On comparing the favorable (n=17) and unfavorable (n=24) subgroups, the former exhibited significantly lower VAS scores for sicca and depression and XI and ESSPRI scores at baseline (all p<0.05) and more lacrimal flow (p<0.05). The favorable subgroup received a significantly lower cumulative dose of pilocarpine and glucocorticoids (both p<0.05). CONCLUSION: About 25% of pSS patients showed clinically significant ESSPRI improvement and about 40% showed a favorable ESSPRI course. Because the favorable subgroup had more lacrimal flow and less sicca symptoms at baseline, long-term patient-derived outcomes could depend on residual exocrine function at pSS diagnosis.
Depression ; Diagnosis ; Glucocorticoids ; Humans ; Immunoglobulin G ; Patient Outcome Assessment ; Pilocarpine ; Quality of Life ; Rheumatic Diseases ; Visual Analog Scale ; Xerophthalmia ; Xerostomia

BACKGROUND: Auditory evoked potentials (AEPs) have assumed an essential role in the clinical practice of auditory and several other professions. AEP classification and nomenclature systems are generally based on such aspects as the time domain (short, middle, long), anatomic origin, stimulus-response relationship. The P300 component of the human event related potential is an endogenous positive wave with a latency of 300msec or greater. OBJECTIVES: Our purpose of this study was to estimate normal values of auditory late response (ALR) and P300 in adults and use them in mapping of the brain. MATERIALS AND MEHTODS: An auditory evoked potential was performed on 40 normal adults, and a scalp surface electrode with 30 electrodes was attempted. Forty adults with normal hearing were divided into 2 groups, one consisting of thirty persons under 30 years of age and the other consisting of ten persons over 30 years of age. RESULTS: The latencies of P1, N1, P2, N1-P2 were 53.17 +/- 19.57 msec, 98.25 +/- 32.68 msec, 166.12 +/- 44.0 msec, 65.12 +/- 23.68 msec, respectively, in the whole group and 54.23 +/- 20.86 msec, 98.7 +/- 34.35 msec, 160.93 +/- 44.02 msec 62.23 +/- 25.36 msec, respectively, in the group under 30 years old, and 50.0 +/- 15.57 msec, 96.9 +/- 28.69 msec, 181.7 +/- 42.28 msec 73.8 +/- 15.69 msec, respectively, in the group over 30 years old. The P300 latencies for the whole group and the two groups were 299.37 +/- 34.01 msec, 295.16 +/- 33.36 msec, 312.0 +/- 34.49 msec, respectively. There was no significant difference in the latency and amplitude of the ALR and P300 between the two groups (p>0.05). CONCLUSION: ALR and P300 were measured in 40 normal adults. Description of the normal topography of P300 may facilitate the examination of the P300 topography in cognitive disorders. Such an examination might help ALR and P300 to serve as better diagnostic tools of cognitive disorder in adults.
Adult* ; Brain ; Brain Mapping ; Classification ; Electrodes ; Event-Related Potentials, P300 ; Evoked Potentials ; Evoked Potentials, Auditory ; Hearing ; Humans ; Reference Values ; Scalp

Menetrier' disease is a rare disease characterized, histologically, by epithelial hyperplasia involving the surface and foveolar mucous cells, accompanied by atrophic or normal oxyntic glands of the stomach. The 48 year-old man with epigastric discomfort and peripheral edema was admitted to St. Paul Hospital. Gastrofiberscopic examination revealed extremely thickened mucosal fold in fundus and body especially greater curvature of stomach, and microscopically there was marked hyperplasia of foveolae and cystic dilatation. Serum albumin concentration was decreased and clearance of a1-antitrypsin was markedly increased in the stool, suggesting the protein-losing enteropathy. He was diagnosed to Menetrier's disease and treated with H2 blocker, antacids, diuretics and albumin replacement.
Antacids ; Dilatation ; Diuretics ; Edema ; Esophagus* ; Gastritis, Hypertrophic* ; Humans ; Hyperplasia ; Intestines* ; Middle Aged ; Protein-Losing Enteropathies ; Rare Diseases ; Serum Albumin ; Stomach*

PURPOSE: The porosity of the bone cement is the most important cause of fatigue failure, the most common mode of failure of bone cement using widely in arthroplasty. It is important to evaluate the porosity of bone cement for improvement or development of bone cement, but the conventional 'stain, 'cut, and 'polish, manual method takes long time and efforts. So it is necessary to develop a new technique for evaluation of porosity of bone cement. We tried a technique using computer image analysis system to evaluate the porosity of bone cement specimen and assess efficacy of the method. Simultaneously we evaluated the relationship between the porosity of bone cement and fatigue failure. MATERIAL AND METHODS: We made 59, 2.5inch-length bone cement specimens(30 Simplex P, 29 Palacos R) using Simplex P and Palaces R which are widely using in clinical situation and checked radiogram using mammography film. After scanning the mammography film, we measured the porosity of the bone cement specimens using NIH(National Institute for Health) Image 1.6 version image analysis program. We also, measured the porosity of the bone cement specimens with conventional 'stain','cut' and 'polish' method, after then compared the results of two methods. Simultaneously, we evaluated the relationship between porosity & fatigue failure by loading 9.0, 10.0, 12.5 and 15.0 MPa load with frequency of 10Hz to the bone cement specimens under the physiologic condition. RESULTS: The coefficient of relation of simplex P and palaces R was 0.729 and 0.713 respectively, so there was high relationship between the image analysis system method and conventional one. It was easy and took shorter time to measure the porosity of bone cement specimens with image analysis system. There was high correlation between cement porosity and fatigue failure, regardless of level of load. CONCLUSION: It was very easy and fast to measure the porosity of the bone cement specimens with image analysis system and there was high correlation between cement porosity and fatigue failure.
Arthroplasty ; Fatigue ; Mammography ; Methylmethacrylate ; Porosity*

We investigated the compliance of Korean patients using tumor necrosis factor (TNF) inhibitors to treat rheumatoid arthritis (RA) and ankylosing spondylitis (AS), and identified potential predictors associated with treatment discontinuation. The study population comprised 114 RA and 310 AS patients treated with TNF inhibitors at a single tertiary center for at least 1 yr from December 2002 to November 2011. Of the 114 RA patients, 64 (56.1%) discontinued their first TNF inhibitors with a mean duration of 18.1 months. By contrast, 65 of 310 patients (21.0%) with AS discontinued their first TNF inhibitors, with a mean duration of 84 months. Although the survival rate did not differ among the three TNF inhibitors in the AS patients, the etanercept group had a lower discontinuation rate than the infliximab group in the RA patients. In addition, RA patients who received corticosteroids in combination with TNF inhibitors were more likely to discontinue their TNF inhibitors. The independent predictors of drug discontinuation in AS patients were male gender and complete ankylosis on radiographs of the sacroiliac joint. Our results provide further evidence that real-life treatment outcomes of RA and AS patients may be different from those observed in randomized clinical trials.
Adult ; Aged ; Antibodies, Monoclonal/therapeutic use ; Antibodies, Monoclonal, Humanized/therapeutic use ; Antirheumatic Agents/*therapeutic use ; Arthritis, Rheumatoid/*drug therapy/mortality ; Cohort Studies ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin G/therapeutic use ; Male ; Middle Aged ; Proportional Hazards Models ; Receptors, Tumor Necrosis Factor/therapeutic use ; Sex Factors ; Spondylitis, Ankylosing/*drug therapy/mortality/radiography ; Tertiary Care Centers ; Treatment Refusal ; Tumor Necrosis Factors/*antagonists & inhibitors/metabolism

We investigated the compliance of Korean patients using tumor necrosis factor (TNF) inhibitors to treat rheumatoid arthritis (RA) and ankylosing spondylitis (AS), and identified potential predictors associated with treatment discontinuation. The study population comprised 114 RA and 310 AS patients treated with TNF inhibitors at a single tertiary center for at least 1 yr from December 2002 to November 2011. Of the 114 RA patients, 64 (56.1%) discontinued their first TNF inhibitors with a mean duration of 18.1 months. By contrast, 65 of 310 patients (21.0%) with AS discontinued their first TNF inhibitors, with a mean duration of 84 months. Although the survival rate did not differ among the three TNF inhibitors in the AS patients, the etanercept group had a lower discontinuation rate than the infliximab group in the RA patients. In addition, RA patients who received corticosteroids in combination with TNF inhibitors were more likely to discontinue their TNF inhibitors. The independent predictors of drug discontinuation in AS patients were male gender and complete ankylosis on radiographs of the sacroiliac joint. Our results provide further evidence that real-life treatment outcomes of RA and AS patients may be different from those observed in randomized clinical trials.
Adult ; Aged ; Antibodies, Monoclonal/therapeutic use ; Antibodies, Monoclonal, Humanized/therapeutic use ; Antirheumatic Agents/*therapeutic use ; Arthritis, Rheumatoid/*drug therapy/mortality ; Cohort Studies ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin G/therapeutic use ; Male ; Middle Aged ; Proportional Hazards Models ; Receptors, Tumor Necrosis Factor/therapeutic use ; Sex Factors ; Spondylitis, Ankylosing/*drug therapy/mortality/radiography ; Tertiary Care Centers ; Treatment Refusal ; Tumor Necrosis Factors/*antagonists & inhibitors/metabolism

Bone marrow edema is a common and nonspecific finding on magnetic resonance imaging (MRI) and results from various diseases including infection, inflammation, neoplasm, injury, and osteoarthritis. However, bone marrow edema syndrome (BMES) represents a distinct entity with specific clinical and imaging features such as diffuse extensions, the lack of other morphologic alterations, no history of trauma, and reversible disease courses. BMES is caused by ischemic changes, and thus, it is postulated to occur as a result of the thrombosis in a patient with primary anti-phospholipid syndrome (APS). Here, we present a case of 67-year-old male with a history of stroke, being presented with fever of unknown origin and pain on both knees. He had high titers of IgG and IgM anti-cardiolipin antibodies which titers didn't change after 3 months. He was diagnosed as having a BMES due to typical MRI findings. After the introduction of anticoagulation, fever and joint pain were subsided. Our case suggests that BMES can develop within a patient with APS, thus early detection of differentiations is essential to avoid unnecessary treatments.
Antibodies ; Antiphospholipid Syndrome ; Arthralgia ; Bone Marrow ; Edema ; Fever ; Fever of Unknown Origin ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Inflammation ; Knee ; Magnetic Resonance Imaging ; Male ; Osteoarthritis ; Stroke ; Thrombosis

We present a case of a 47-year-old woman with benign solitary fibrous tumor of the pleura originated in the diaphragm which was discovered incidentally on a chest radiograph. Chest radiograph, sonograph, computed tomographic scan and magnetic resonance image studies proved a well circumscribed and lobulated fibrous tumor of the pleura. During the subsequent right thoracotomy, the tumor was found to be encapsulated and consisted of firm mass. It was connected to the diaphragm with 5x4 cm area by pedicle. Most of the tumor was free of adhesion. It was excised completely together with attached diaphragm. The tumor measured 23.5x3.5x8.0 cm and the pathologic diagnosis was benign solitary fibrous tumor and the attached diaphragm was free of disease.
Diagnosis ; Diaphragm ; Female ; Humans ; Middle Aged ; Pleura* ; Pleural Neoplasms ; Radiography, Thoracic ; Solitary Fibrous Tumor, Pleural ; Solitary Fibrous Tumors* ; Thoracotomy