Adult onset Still's disease (AOSD) is a systemic autoinflammatory disorder that presents with recurrent fever, extreme fatigue, and joint pain. Pulmonary involvement is not uncommon and, although rare, severe pneumonitis can progress to respiratory failure. Still's disease-associated pneumonitis is generally treated with immunosuppressive agents, but improvement in our understanding of systemic inflammatory processes led us to explore alternative agents. Anakinra is an interleukin-1 receptor antagonist used to treat autoinflammatory disorders resistant to immunosuppressive therapy. Several case reports have demonstrated efficacy of anakinra in treating AOSD, but its relevance in cases complicated with severe pneumonitis has not been examined. Our patient's disease activity was not controlled with systemic steroids and cyclophosphamide. Treatment with anakinra led to a dramatic clinical response. This is the first reported case of AOSD with severe pneumonitis refractory to conventional therapy successfully treated with anakinra.
Arthralgia ; Cyclophosphamide ; Fatigue ; Fever ; Immunosuppressive Agents ; Interleukin 1 Receptor Antagonist Protein* ; Interleukin-1 ; Pneumonia* ; Respiratory Insufficiency ; Steroids ; Still's Disease, Adult-Onset*

BACKGROUND/AIMS: We aimed to evaluate the clinical characteristics and prognosis of Crohn's disease (CD) in patients who showed proximal small bowel involvement using a nationwide Korean CD cohort. METHODS: We reviewed the data from a cohort of patients diagnosed with CD. The clinical outcomes of patients were evaluated according to the presence of proximal small bowel involvement. RESULTS: Among 1,329 patients with CD for whom complete disease location data were available, 222 patients (16.7%) showed involvement of the proximal small bowel. Compared to patients without proximal small bowel involvement, those with small bowel involvement were more likely to display stricturing behavior (19.8% vs 12.7%, p=0.020). The surgery-free survival of patients who showed proximal small bowel involvement was inferior to that of patients without proximal small bowel involvement (10-year surgery-free survival: 58.4% vs 67.7%, respectively, p < 0.001). Additionally, upper gastrointestinal involvement was more common in patients with proximal small bowel involvement than in those without involvement (odds ratio, 1.643; 95% confidence interval, 1.008 to 2.677). CONCLUSIONS: Proximal small bowel involvement is a poor prognostic factor for the surgery-free survival of Korean patients with CD. Proximal small bowel involvement should be evaluated in patients with CD for predicting long-term clinical outcomes.
Cohort Studies ; Crohn Disease ; Humans ; Jejunum ; Prognosis

BACKGROUND: The urine dipstick is widely used as an initial screening tool for the evaluation of proteinuria; however, its diagnostic accuracy has not yet been sufficiently evaluated. Therefore, we evaluated its diagnostic accuracy using spot urine albumin/creatinine ratio (ACR) and total protein/creatinine ratio (PCR) in proteinuria. METHODS: Using PCR > or = 0.2g/g or > or = 0.5g/g and ACR > or = 300mg/g or > or = 30mg/g as the reference standard, we calculated the diagnostic accuracy profile: sensitivity, specificity, positive and negative predictive value, and the area under the curve (AUC) of the receiver operating characteristic curve. RESULTS: PCR and ACR were available for 10,348 and 3,873 instances of dipstick testing. The proportions with PCR > or = 0.2g/g, > or = 0.5g/g and ACR > or = 300mg/g, > or = 30mg/g were 38.2%, 24.6% and 8.9%, 31.7%, respectively. The AUCs for PCR > or = 0.2g/g, > or = 0.5g/g, and ACR > or = 300mg/g were 0.935 (trace: closest to ideal point), 0.968 (1+), and 0.983 (1+), respectively. Both sensitivity and specificity were > 80% except for PCR > or = 0.5g/g with trace cutoff. For the reference standard of ACR > or = 30mg/g, the AUC was 0.797 (trace) and the sensitivity was 63.5%. CONCLUSION: Urine dipstick test can be used for screening in older outpatients with ACR > or = 300mg/g or PCR as the reference standard for proteinuria. However, we cannot recommend the test as a screening tool with ACR > or = 30mg/g as the reference owing to its low sensitivity.
Albuminuria ; Area Under Curve ; Humans ; Mass Screening ; Outpatients* ; Polymerase Chain Reaction ; Proteinuria* ; Reagent Strips ; ROC Curve ; Sensitivity and Specificity

Pegylated interferon alpha (PEG-IFN-α) is widely used to treat chronic hepatitis C in combination with ribavirin. Many adverse effects of PEG-IFN-α, such as hematologic, psychologic, dermatologic, immunologic, and other abnormalities, have been reported, and some serious adverse events lead to PEG-IFN-α treatment discontinuation. For very rare adverse events such as panniculitis, there are no established guidelines on whether to continue PEG-IFN-α treatment. Published reports on panniculitis induced by PEG-IFN-α 2a are sparse. Herein we report a case of repeated occurrences of panniculitis in a patient with chronic hepatitis C, leading to treatment cessation.
Hepatitis C ; Hepatitis C, Chronic* ; Hepatitis, Chronic* ; Humans ; Interferon-alpha* ; Interferons* ; Panniculitis* ; Ribavirin ; Withholding Treatment

Background/Aims: Helicobacter pylori (H. pylori) infection highly correlates with erythematous/exudative gastritis, which is one of the endoscopic findings of the Sydney classification system. The present study aimed to evaluate the association between endoscopic severity of erythematous/exudative gastritis and H. pylori infection. Methods: We prospectively enrolled asymptomatic adults who were diagnosed with erythematous/exudative gastritis during screening esophagogastroduodenoscopy. A rapid urease test was performed in all participants to diagnose H. pylori infection. The severity of erythematous/exudative gastritis was determined based on the Sydney classification system. Two investigators independently evaluated the endoscopic findings. The primary endpoint was H. pylori infection rate according to the severity of erythematous/exudative gastritis (mild vs. moderate-to-severe). Results: A total of 177 patients with erythematous/exudative gastritis were included. The rate of H. pyloriinfection was 86.4% in all patients. Of 177 included patients, 78 were at mild degree, 48 were at moderate degree, and 51 were at severe degree. The inter-observer variation was 4.6% and kappa value was 0.593. H. pylori infection rate was similar between patients with mild erythematous/exudative gastritis and those with moderate-to-severe erythematous/exudative gastritis (91.0% vs. 82.8%, p=0.115). Even after adjusting potential confounding variables, the severity of erythematous/exudative gastritis was not associated with H. pylori infection rate. Conclusions H. pylori infection is commonly observed in patients with erythematous/exudative gastritis. However, the severity of erythematous/exudative gastritis is not associated with H. pylori infection rate.