Behcet's disease (BD) is a multisystemic disorder, which is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesion. Although intestinal involvement is an uncommon manifestation of the BD, it leads to a poor prognosis, as a result of a high complication, such as intestinal perforation, fistula formation, and massive hemorrhage. Intestinal BD has the tendency for the resistance to conventional medical treatment, and it often requires a surgical intervention because of severe complication. Although the causes of intestinal BD are unknown, some studies show that tumor necrosis factor Alpha (TNF-alpha) plays a key role in the pathogenesis of BD. Therefore, anti-TNF-alpha monoclonal antibody, such as adalimumab, is one of the useful treatment for refractory and relapsed intestinal BD. We describe a patient who had intestinal BD complicated enterocutanous fistula with a good response to adalimumab.
Antibodies, Monoclonal, Humanized ; Fistula ; Hemorrhage ; Humans ; Intestinal Perforation ; Intestines ; Prognosis ; Skin ; Stomatitis, Aphthous ; Tumor Necrosis Factor-alpha ; Ulcer ; Uveitis ; Adalimumab

OBJECTIVE: To estimate drug persistency and the safety of TNF blocker in Korean patients with rheumatoid arthritis. METHODS: Data were extracted from medical records of rheumatoid arthritis patients who had treated with TNF blocker or are currently using TNF blocker at Hanyang University Hospital for Rheumatic Diseases from December 2000 to November 2009 (REtrospective study for Safety and Efficacy of Anti-RA treatment with biologiCs, RESEARCh). Comprehensive chart reviews were undertaken on all patients and data on drug usages and response of TNF blocker was collected at initiation, 3 months and the time of data collection. Persistency with treatment was examined using life-table analysis and multivariate Cox proportional hazard models were developed to examine potential predictors of discontinuation of TNF blocker. RESULTS: A total of 268 patients were enrolled in this retrospective study. Among them 180 patients were included in the analysis of drug persistency. The 1-year and 5-year drug persistency of TNF blocker was 74% and 46%, respectively. Concomitant use of methotrexate (hazard ratio 0.46, 95% CI 0.27-0.80) was associated with higher persistence. Comparing to etanercept, adalimumab is an independent risk factor for discontinuation (hazard ratio 2.63, 95% CI 1.43-4.84). CONCLUSION: Five-year drug persistency of TNF blocker was 46% and concomitant use of methotrexate is associated with higher persistence.
Antibodies, Monoclonal, Humanized ; Arthritis, Rheumatoid ; Biological Agents ; Data Collection ; Humans ; Immunoglobulin G ; Medical Records ; Methotrexate ; Proportional Hazards Models ; Receptors, Tumor Necrosis Factor ; Retrospective Studies ; Rheumatic Diseases ; Risk Factors ; Adalimumab ; Etanercept

Infliximab, a chimeric monoclonal immunoglobulin antibody to tumor necrosis factor (TNF)-alpha is widely used in the treatment of rheumatoid arthritis (RA). The commonly reported cardiac side effects of infliximab include exacerbation of congestive heart failure, hypotension, and syncope. Cardiac arrhythmia and conduction disturbances have been reported only rarely in a few case reports and to the best of our knowledge, there are no previous reports on the occurrence of atrial fibrillation secondary to infliximab use in RA patients. Here, we report a case of acute atrial fibrillation with rapid ventricular response that occurred 24 hr after first dose of infliximab in a 55-year-old female patient with RA, who recovered to sinus rhythm after pharmacologic management.
Antibodies, Monoclonal ; Arrhythmias, Cardiac ; Arthritis, Rheumatoid ; Atrial Fibrillation ; Female ; Heart Failure ; Humans ; Hypotension ; Immunoglobulins ; Middle Aged ; Syncope ; Tumor Necrosis Factor-alpha ; Infliximab

OBJECTIVE: Pneumomediastinum (PnM), a rare complication of dermatomyositis and polymyositis (DM/PM), is sporadic and has an unclear pathogenesis. PnM is almost always associated with interstitial lung disease (ILD), and is a poor prognostic factor in inflammatory myositis patients. We studied the prevalence of PnM in Korean DM/PM and its clinical significance. METHODS: We retrospectively studied the medical records of 161 patients diagnosed with DM/PM meeting Bohan-Peter's criteria at Hanyang University Hospital for Rheumatic Diseases from 1995 to 2010. We collected following findings; demographic data, diagnosis, lung involvement, cause of death, and duration from diagnosis to death. RESULTS: One hundred nineteen patients (73.9%) were DM and 42 patients (26.1%) were PM. Eighty three patients (51.6%) developed ILD at diagnosis or during follow up. Eighteen patients (11.2%) died because of ILD aggravation, infection, or malignancy. The mean duration from diagnosis to death was 11.5 months, with 10 patients (6.2%) dying from from ILD aggravation but none with spontaneous PnM. 6 patients (3.7%) presented with PnM, and it was associated with ILD worsening in all cases. PnM resolved with O2 inhalation, corticosteroids, and/or immunosuppressive agents after 11 weeks (mean) of therapy. CONCLUSION: PnM is rare but associates with DM and aggravation of ILD. PnM does not usually cause fatalities and can be cured by appropriate therapy.
Adrenal Cortex Hormones ; Cause of Death ; Dermatomyositis ; Follow-Up Studies ; Humans ; Immunosuppressive Agents ; Inhalation ; Lung ; Lung Diseases, Interstitial ; Mediastinal Emphysema ; Medical Records ; Myositis ; Polymyositis ; Prevalence ; Prognosis ; Retrospective Studies ; Rheumatic Diseases

PURPOSE: The aim of this study was to investigate the ability of thoracic computed tomography (CT) to predict esophageal stricture formation and risk factors associated with the development of stricture induced by ingestion of caustic materials. METHODS:This was a study of 41 patients who had visited an emergency care center following ingestion of caustic substances sometime between January 1998 and August 2008. A retrospective analysis of medical records was performed. Findings for the esophageal lesion were classified according to changes in the esophageal wall and the infiltration of peri-esophageal soft tissue. Also, clinical, laboratory, and endoscopic data from these patients were reviewed. The correlation between the degree of esophageal damage seen on CT scans and esophageal constriction seen on esophagography were then evaluated. RESULTS: A total of 41 cases of caustic ingestion were identified (age range, 20~82 years). The most common caustic agent ingested was acid (70%). The most frequent cause for ingestion was attempted suicide (70%); the other cases (30%) were accidental ingestion. The findings on thoracic CT in the 41 patients were as follows: first-degree esophageal injury in 4 (9.8%), second-degree in 8 (19.5%), third-degree in 17 (41.6%), fourth-degree in 12 (29.3%). Fourteen patients (34.1%) developed caustic esophageal stricture. The closer the degree of esophageal damage was to grade IV, the more prevalent the esophageal constriction became. This correlation was statistically significant (p<0.001). Of the 41 patients, 26 underwent endoscopy at an early stage after they visited an emergency care center. An analysis of the correlation between the degree of esophageal damage seen on endoscopy and that seen on CT scans was performed. This revealed a significant correlation (p=0.002, r=0.585). CONCLUSION: Thoracic CT grading suggesting periesophageal soft tissue infiltration and fluid collection (grade III to IV) rather than only edema (grade I) may be associated with stricture formation. Early CT grading is safe and useful for predicting the development of stricture induced by caustic ingestion.
Caustics ; Constriction ; Constriction, Pathologic ; Eating ; Edema ; Emergency Medical Services ; Endoscopy ; Esophageal Stenosis ; Humans ; Medical Records ; Retrospective Studies ; Risk Factors ; Suicide, Attempted ; Thorax

We aimed to evaluate the safety and clinical responses in Korean ankylosing spondylitis (AS) patients after three months of etanercept therapy. AS patients satisfying the Modified New York Criteria were enrolled. They were assessed for safety and clinical responses at enrollment and after three months of etanercept therapy. A total of 124 patients completed the study. After three months, the rate of ASsessment in AS International Working Group 20% improvement (ASAS 20) response was 79.8%. The rates of ASAS 40 and ASAS 5/6 responses were 58.5 and 62.8%, respectively. Significant improvement of Korean version of Bath AS Disease Activity Index (KBASDAI) (p<0.0001), Bath AS Functional Activity Index (BASFI) (p<0.0001), and Bath AS Metrology Index (BASMI) (p=0.0009) were achieved after three months. Quality of life was also significantly improved after three months, as demonstrated by scores for SF-36 (p<0.0001) and EQ-5D (p<0.0001). Erythrocyte sedimentation rate and C-reactive protein were significantly decreased (p<0.0001, p<0.0001, respectively). None of the patients developed tuberculosis and there were no serious adverse event. AS patients with inadequate response to conventional therapy showed significant clinical improvement without serious adverse events after three months of etanercept therapy.
Adult ; Blood Sedimentation ; C-Reactive Protein/metabolism ; Cohort Studies ; Female ; Humans ; Immunoglobulin G/*pharmacology ; Immunosuppressive Agents/pharmacology ; Korea ; Male ; Prospective Studies ; Quality of Life ; Receptors, Tumor Necrosis Factor ; Remission Induction ; Spondylitis, Ankylosing/*therapy ; Treatment Outcome

Panniculitis is an inflammation within adipose tissue and most commonly affects the subcutaneous fat. Frequently, the panniculitis is associated with certain drugs and systemic diseases, such as various rheumatologic diseases, idiopathic Weber-Christian disease, infection and malignancy. Panniculitis in dermatomyositis (DM) is most commonly an incidental histopathologic finding that less commonly manifests a clinical component. We report the case of a woman with DM who presented with panniculitis as a clinical finding. A 51-year-old woman was admitted to our hospital with diffuse nodular, indurated, painful erythematous plaques on buttocks, back and chest. 18-months ago, she had been diagnosed DM at our hospital. Her medication on admission was azathioprine. High-dose glucocorticoid was prescribed after the diagnosis of lobular panniculitis confirmed by skin biopsy. After then, the patient was getting better and discharged with the medication tapered.
Female ; Humans ; Biopsy

We report here a case of vascular Behcet's disease. 32-year-old young woman who suffered from chest pain was diagnosed with acute myocardial infarction four years before admission. At that time she manifested symtoms of Behcet's disease, such as oral ulcers, genital ulcers, multiple arthralgia, and a peculiar hyperirritability reaction to needle puncture. At this time she was admitted due to acute chest pain and intermittent abdominal pain. The diagnosis of an acute anteroseptal myocardial infarction was made on the basis of the electrocardiography findings. Cardiac catheterization revealed segmental stenosis up to 95% at the mid-left anterior descending artery without aneurysm formation at other coronary trees and there were no atherosclerotic changes. Coronary stent was inserted in the lesion. Abdominal CT showed inferior vena cava and right hepatic vein obstruction, which supports the diagnosis of Budd-Chiari syndrome. Our case demonstrated the followings which are not common to vasculo-Behcet disease; 1) initial clinicopathologic manifestations at the coronary artery, 2) abrupt total occlusion with clear cut lesion at left anterior descending artery, 3) recurrent myocardial infarctions, 4) Budd-Chiari syndrome, 5) combined superior mesenteric artery, inferior mesenteric artery and celiac trunk obstruction. Among the systemic manifestation of Behect's disease, cardiac involvement is very rare but should be considered as one of the most important features that influences the prognosis.
Abdominal Pain ; Adult ; Aneurysm ; Anterior Wall Myocardial Infarction ; Arteries ; Arthralgia ; Budd-Chiari Syndrome* ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Constriction, Pathologic ; Coronary Vessels ; Diagnosis ; Electrocardiography ; Female ; Heart Diseases ; Hepatic Veins ; Humans ; Mesenteric Artery, Inferior ; Mesenteric Artery, Superior ; Myocardial Infarction* ; Needles ; Oral Ulcer ; Prognosis ; Punctures ; Stents ; Tomography, X-Ray Computed ; Ulcer ; Vena Cava, Inferior

OBJECTIVE: Juvenile rheumatoid arthritis (JRA) is classified as polyarticular, oligoarticular, and systemic onset type by clinical symptoms presented during first six months. This study was performed to investigate the clinical features and course of systemic onset JRA. METHODS: We performed a retrospective study for patients who were diagnosed as JRA between March 2000 and March 2006 according to the JRA criteria of the International League of Association for Rheumatology (ILAR). RESULTS: Of the 216 JRA patients, 33 patients (11 male/ 22 female) were systemic onset type. Because of insufficient data, 6 patients were excluded. Chief complaints at the time of diagnosis were fever (81.5%) and arthralgia (77.7%). During the disease course, all patients manifested fever and arthritis, rash (59.2%) and splenomegaly (22.2%) also occurred. Most patients had symmetric (81.5%) arthritis, and involved more than five joints (59.3%) including knee and wrist. Anemia, leukocytosis, and thrombocytosis were common laboratory abnormalities. Almost all patients had elevated level of C-reactive protein and erythrocyte sediment rate. Some patients had positive results about immunologic marker such as rheumatoid factor (3.8%), antinuclear antibody (57.7%), and antiperinuclear factor (9.5%). Therapeutic regimens included glucocorticoids (88.9%), nonsteroidal anti-inflammatory drugs (81.5%), methotrexate (81.5%), and hydroxychloroquine (55.6%). Biologic agents were applied in 5 patients, and 3 showed improvement of disease activity. Combination therapy was introduced in 18.5% of patients, and 63% of patients still required medications. CONCLUSION: In Korea, systemic onset JRA patients had variable clinical manifestations and chronic course of disease, which often extended into adulthood.
Anemia ; Antibodies, Antinuclear ; Arthralgia ; Arthritis ; Arthritis, Juvenile* ; Biological Factors ; Biomarkers ; C-Reactive Protein ; Diagnosis ; Erythrocytes ; Exanthema ; Fever ; Glucocorticoids ; Humans ; Hydroxychloroquine ; Joints ; Knee ; Korea ; Leukocytosis ; Methotrexate ; Retrospective Studies ; Rheumatoid Factor ; Rheumatology ; Splenomegaly ; Thrombocytosis ; Wrist

OBJECTIVE: It has been suggested that overproduction of interleukin -18 (IL-18) may contribute to the pathogenesis of adult onset Still's disease (AOSD). Recently, positive association between a polymorphism in the IL-18 gene and different diseases like diabetes, sarcoidosis and asthma has been reported. The aim of the present study was to investigate the potential association of two single-nucleotide polymorphisms (SNPs) at position -137 (G/C) and -607 (C/A) in the promoter region of the IL-18 gene with susceptibility and clinical feature of AOSD in the Korean population. METHODS: We examined two SNPs of IL-18 in 70 patients with AOSD and 204 healthy control individuals. The genotyping were performed using sequence specific PCR. Haplotypes were analyzed by the estimated haplotype program. The patients with AOSD were subdivided into groups according to disease course: monocyclic systemic, polycyclic systemic, and chronic articular type. RESULTS: As for the -607 genotypes, 13 of the 69 patients had CC genotype (18.8%), 36 the CA type (52.2%) and 20 the AA type (29.0%). AOSD patients had higher frequency of A allele at -607 when compared to controls (OR 1.48, 95% CI 1.00~2.18, p=0.048). AOSD patients had significantly higher frequency of AA genotypes at -607 when compared to controls (AA vs CA& CC, OR 1.90, 95% CI 1.01~3.58, p=0.044). As for the -137 genotypes, of the 68 patients, 57 had GG genotype (83.8%), 9 the GC type (13.2%) and 2 (2.9%) had the CC type. No differences were found in allele and genotype frequencies between two groups. The haplotype frequencies of the IL-18 polymorphism were not significantly different between patients with AOSD and controls. The frequency of -137 GG genotype was significantly increased in chronic articular type compared to healthy control and systemic type of AOSD. CONCLUSION: In IL-18 gene polymorphisms, the A allele and AA genotye at position -607 might be genetic risk factors for the development of AOSD in Korean population. Further investigation in larger groups is required to provide more conclusive evidence regarding the role of the IL-18 gene polymorphism in AOSD.
Adult* ; Alleles ; Asthma ; Genotype ; Haplotypes ; Humans ; Interleukin-18* ; Interleukins ; Korea* ; Polymerase Chain Reaction ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Risk Factors ; Sarcoidosis ; Still's Disease, Adult-Onset*