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

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: 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

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

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

OBJECTIVES: To elucidate the genetic basis for Thiopurine methyltransferase (TPMT) polymorphism and investigate the relationship between TPMT mutant and the adverse effect in patients with systemic lupus erythematosis (SLE) taking azathioprine (AZA) in Korea. METHODS: The TPMT genotype was determined in two hundred healthy adults and 342 patients with SLE by MALDI-TOF and correlated with the effects of clinical exposure to AZA. RESUTLS: TPMT polymorphism were detected in 2/200 healthy adults (1%), which were heterozygotes with TPMT*3C and TPMT*6 allele, respectively, and 17/342 (4.97%), which were 12 heterozygotes with TPMT*3C and 5 heterozygotes with TPMT*6 allele, respectively, which had a higher frequency of TPMT mutant alleles compared to the healthy controls (p=0.015). Severe nausea occurred in 4 patient with TPMT*3C allele, and severe bone marrow toxicity in a patient with TPMT*6 allele taking AZA. Twenty three in 94 (24.47%) SLE patients taking AZA were suspicious of the adverse effects such as leucopenia (n=17), nausea (n=4) and abnormal liver function test (n=1). AZA was relatively well tolerated among the rest of them. CONCLUSION: The heterozygote with TPMT*3C and *6 were frequently detected in the patient with SLE compared to healthy adults and there was no statistical correlation between TPMT genotype and AZA toxicity. TMPT genotyping cannot replace regular blood monitoring in SLE patients on AZA treatment.
Adult ; Alleles* ; Azathioprine* ; Bone Marrow ; Genotype ; Heterozygote ; Humans ; Korea ; Liver Function Tests ; Lupus Erythematosus, Systemic* ; Nausea

Gold salts have been used for many years in the treatment of rheumatoid arthritis. The common side effects are mucocutaneous reactions, but hepatotoxic reaction and isolated neutropenia are rare complications. We report a 62-year-old woman with rheumatoid arthritis who had developed hepatitis and neutropenia simultaneously after receiving 137.5 mg of sodium aurothiomalate.
Antirheumatic Agents, Gold/adverse effects+ACo- ; Arthritis, Rheumatoid/drug therapy+ACo- ; Case Report ; Female ; Gold Sodium Thiomalate/adverse effects+ACo- ; Hepatitis, Toxic/etiology+ACo- ; Human ; Injections ; Middle Age ; Neutropenia/chemically induced+ACo-

Behcet's disease can elicit serious vascular complications such as thrombophlebitis, arterial occlusion, and arterial aneurysm. The most common cause of death in Behcet's disease is secondary to vascular complication. The treatment for abdominal aortic aneurysm which was frequently complicated by fatal rupture (60%) is mainly a surgical management. If surgical treatment is not possible due to various causes, alternative medical therapy such as immunosuppressives and steroid would be recommended. We report here the case of medically controlled abdominal aortic aneurysm in a 47-year-old patient with Behcet's disease who refused to undergo surgical treatment. Immunosuppressive therapy and steroid could be an alternative treatment of aneurysmal manifestations in Behcet's disease.
Aneurysm ; Aortic Aneurysm ; Aortic Aneurysm, Abdominal* ; Cause of Death ; Humans ; Immunosuppressive Agents* ; Middle Aged ; Rupture ; Thrombophlebitis

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