Drug survival of tumor necrosis factor α inhibitors in patients with ankylosing spondylitis in Korea

BACKGROUND/AIMS: To evaluate drug survival of the tumor necrosis factor α inhibitors (TNFi) and risk factors for the drug discontinuation in patients with ankylosing spondylitis (AS). METHODS: We retrospectively evaluated 487 AS patients at a single tertiary hospital. Among the TNFi users, drug survival and risk factors of TNFi discontinuation were investigated. RESULTS: Among 487 patients, 128 AS patients were treated with at least one TNFi. Patients who were treated with TNFi were younger at disease onset, had more peripheral manifestations, and had higher level of acute phase reactants and body mass index than those of TNFi non-users at baseline. Of 128 patients, 28 patients (21.9%) discontinued first TNFi therapy during the follow-up period of 65.1 ± 27.9 months. In the multivariable analysis, female (hazard ratio [HR], 6.08; 95% confidence interval [CI], 2.27 to 16.27; p = 0.003), hip involvement (HR, 2.52; 95% CI, 1.08 to 5.87; p = 0.033) and a high C-reactive protein (CRP; HR, 1.10; 95% CI, 1.00 to 1.21; p = 0.044) were risk factors for drug discontinuation. Etanercept showed better survival rate than infliximab. The main reason for discontinuation of TNFi was inefficacy. CONCLUSIONS: TNFi discontinuation rate of Korean patients with AS seems to be similar to those with the European patients. Female sex, hip involvement, CRP, and the type of TNFi were associated with TNFi discontinuation.

Characteristics of hip involvement in patients with ankylosing spondylitis in Korea

BACKGROUND/AIMS: To evaluate the clinical course of hip arthritis and the risk factors for hip joint replacement in patients with ankylosing spondylitis (AS). METHODS: In this retrospective analysis, we evaluated 488 AS patients at a single tertiary hospital. At baseline and the most recent visit to the outpatient clinic from the patients with hip arthritis in AS, radiographic hip arthritis was evaluated using the Bath Ankylosing Spondylitis Radiology Hip Index (BASRI-h). Also the average of the hip joint space width (interbone distance) at three distinct sites between the acetabulum and femoral head was recorded. RESULTS: Among 488 patients with AS, 60 patients (12.3%) had hip arthritis. Erythrocyte sedimentation rate were associated with hip involvement (odds ratio, 1.01; 95% confidence interval [CI], 1.00 to 1.02; p = 0.004). Long disease duration and advanced axial disease were associated with severe hip arthritis (3 ≥ BASRI-h) at baseline. BASRI-h and interbone distance did not significantly change in patients with hip involvement during the follow-up period of 81.4 ± 35.7 months. Five patients had hip joint replacement surgery during follow-up period. The body mass index (BMI) and BASRI-h at baseline were associated with joint replacement surgery (hazard ratio [HR], 1.30; 95% CI, 1.00 to 1.67; p = 0.049 and HR, 20.64; 95% CI, 2.39 to 178.11; p = 0.006, respectively). CONCLUSIONS: Most of the patients with hip arthritis in AS showed no significant radiographic progression during the follow-up period. High BMI and advanced hip arthritis at baseline were associated with hip joint replacement surgery in patients with AS.

Relapsing polychondritis presenting with inflammatory pseudotumor

No abstract available.

Wernicke's encephalopathy after total parenteral nutrition in patients with Crohn's disease

Micronutrient deficiencies in Crohn's disease (CD) patients are not uncommon and usually result in a combination of reduced dietary intake, disease-related malabsorption, and a catabolic state. Decreased serum thiamine levels are often reported in patients with CD. Wernicke's encephalopathy (WE) is a severe form of thiamine deficiency that can cause serious neurologic complications. Although WE is known to occur frequently in alcoholics, a number of non-alcoholic causes have also been reported. Here, we report two cases of non-alcoholic WE that developed in two severely malnourished CD patients who were supported by prolonged total parenteral nutrition without thiamine supplementation. These patients complained of sudden-onset ophthalmopathy, cerebellar dysfunction, and confusion. Magnetic resonance imaging allowed definitive diagnosis for WE despite poor sensitivity. The intravenous administration of thiamine alleviated the symptoms of WE dramatically. We emphasize the importance of thiamine supplementation for malnourished patients even if they are not alcoholics, especially in those with CD.

Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses.

Idiopathic Splenic Vein Thrombosis Presenting as Splenic Infarction and Consequent Gastric Variceal Bleeding

Left-sided portal hypertension and consequent gastric varices can occur in patients with isolated splenic vein thrombosis. It is a rare but clinically significant and curable cause of gastrointestinal hemorrhage. Our patient, a 20-year-old woman, with left flank pain was diagnosed with having idiopathic splenic vein thrombosis with resultant splenic infarction. Thorough workups for the possible etiologies of splenic vein thrombosis were all negative. After six months of anticoagulation, follow-up computed tomography revealed formation of gastric varices; one month following the discovery, she developed gastrointestinal bleeding. Splenectomy was performed, resulting in the resolution of gastric varices.