Bullous pemphigoid (BP) is a chronic relapsing autoimmune blistering disease that typically affects middle-age and elderly patients. It can manifest with varying degrees of mucosal involvement in addition to characteristic skin findings. However, esophageal involvement is very rare. We report a case of a 57-year-old female with BP who presented with epigastric pain and melena. She underwent an esophagogastroduodenoscopy which induced bullae seen only upon withdrawal of the endoscope. This finding is analogous to the dermatological finding of Nikolsky's sign. Gentle insertion and withdrawal of the endoscope is recommended to reduce the risk of bullae formation and mucosal injury.
INTRODUCTION: Bullous pemphigoid (BP) is a chronic relapsing autoimmune blistering disease characterized by circulating antibodies directed against specific epitopes of hemidesmosomes involved in anchoring the epidermis to the dermal layer. We report a case of acute onset esophageal bullae identified in a patient with active skin BP seen only upon withdrawal of the upper endoscope, not present on insertion. This sign is analogous to Nikolsky’s sign where pressure or shearing results in formation of new bullae. CASE DESCRIPTION/METHODS: 57-year-old Caucasian female with Type II DM, BP, CKD stage 3a presented to hospital for 5-6 episodes of melanotic stools for two days. She was diagnosed in September 2017 with BP by skin biopsy and direct immunofluorescence (Figure 1). At the time of presentation, she was normotensive, afebrile, heart rate 102 beats/min and respiratory rate of 14 breaths/min. Skin examination revealed tense bullae on upper extremities (Figure 2). Abdominal exam was notable for tenderness to palpate in the epigastric region. Digital rectal exam revealed melena. Laboratory findings showed hemoglobin 5.7 mg/dl, hematocrit 17.5 mg/dl. The patient received 1 unit of packed RBC, started on a proton pump inhibitor infusion and continued on mycophenolate with oral prednisone. She underwent an upper gastrointestinal endoscopy which revealed segments of sloughing mucosa in the esophagus. Additionally, a large bulla ranging from 20 to 25 cm was seen only upon withdrawal of the endoscope and not during initial insertion (Figure 3). She was discharged home on a high dose oral proton pump inhibitor, mycophenolate, prednisone, and topical steroid. DISCUSSION: Bullous Pemphigoid (BP) is the most common type of acute bullous dermatoses (ABD) affecting older adults without gender predilection. Esophageal manifestations of BP are rare and range from dysphagia, hematemesis or melena. Nikolsky sign is a specific skin finding of autoimmune blistering diseases but is only seen in 56% of the cases. The use of esophagogastroduodenoscopy can be challenging in these patients because of bullae formation after light contact between esophageal mucosa and the endoscope. Only gentle advancement and withdrawal of the endoscope is advised to prevent adverse events such as blood blisters, frank bleeding, or perforation. In cases of significant gastrointestinal bleeding and/ or hemodynamic instability, therapeutic endoscopy can be used to achieve hemostasis.
INTRODUCTION: The availability of noninvasive fibrosis measures coupled with high sustained viral response (SVR) rates has shifted the paradigm in the management of HCV infection in the direct-acting antiviral (DAA) era. Although these noninvasive tests are valuable in evaluating hepatic fibrosis prior to HCV therapy, use of these measures in monitoring fibrosis regression after HCV eradication is currently limited. There is evidence that patients who achieve SVR after therapy have a reduced risk of liver-related complications.1 This is likely attributed to the regression of fibrosis after HCV eradication.2 Other comorbidities, such as obesity, nonalcoholic steatohepatitis and diabetes mellitus may also contribute to liver-related complications.3-4 The aim of this study is to evaluate the effect of metabolic syndrome components on liver stiffness measurement (LSM) after HCV eradication. METHODS: A retrospective study of 65 HCV monoinfected patients who received DAA and achieved SVR located in a safety net hospital in Cleveland. All Patients had pretreatment LSM of more than 7k Pa. Patient’s with Fibrosis stage F4 had CP class A. Fibrosis was evaluate within 12 months before treatment and 12-24 months after SVR using Transient Elastography. Other collected parameters included BMI, history of DM, HLD and HTN, HbA1c, LDL, HDL and TGs. Demographics and hepatitis C genotype (GT) were also collected. Statistical analysis was performed using descriptive analysis, Anova, Univariate regression and Multivariate regression to compare variables for statistical significance. RESULTS: Among all subjects (n = 65). 64.6% were male. 61.5% had Genotype 1a, 26.2% had genotype 1b and 6.2% had genotype 2. 33.8% were diabetic, 64.6% had hyperlipidemia, and 66.2% patients had hypertension. The mean pre-treatment LSM was 14.4kPa (SD 10.0) and the mean post-treatment LSM was 9.33 kPa (SD 5.3). The mean of delta-stiffness was 5.1 kPa (SD 9.7). 53.8% patients dropped their LSM by 3 kPa or more after SVR. In univariate logistic regression, age (P = 0.675), race (P = 0.11), genotype (P = 0.688), BMI (P = 0.709), diabetes (P = 0.661), hyperlipidemia (P = 0.743), and hypertension (P = 0.545) failed to show any significant correlation with significant drop (≥3kPa) in liver stiffness measurement. CONCLUSION: Sustained viral response after direct-acting antiviral (DAA) therapy leads to significant improvement in LSM within 48 months of therapy completion. Metabolic syndrome components has no significant effect on LSM improvement after SVR.
INTRODUCTION: Patients with liver cirrhosis are at risk for life-threatening hemorrhage from esophageal varices. Variceal hemorrhage can be prevented with medical and endoscopic therapies, so identifying patients through a screening process is paramount. Traditionally, screening was offered to all patients with cirrhosis; however, not all patients with cirrhosis are at the same risk to develop variceal hemorrhage. Using the Baveno VI criteria, cirrhotic patients with low risk for esophageal varices can be identified and spared the risks associated with invasive endoscopic screening. Previous studies have not evaluated these criteria in a safety net hospital population. METHODS: We enrolled 75 patients with cirrhosis for a retrospective chart review. Inclusion criteria were F3-4 or F4 by transient elastography and esophagogastroduodenoscopy (EGD) within 12 months of the elastography procedure. RESULTS: Mean age was 58 years, 54.7% were male and 53% of patients identified as black, African-American, or Hispanic. 67% had hepatitis C, 16% had alcoholic liver disease, and 16% had non-alcoholic fatty liver disease. 42.6% of patients had obesity (BMI > 30) and 56% had metabolic syndrome. 21 patients met Baveno VI criteria and 12 had esophageal varices on upper endoscopy. 4 additional patients had esophageal varices, yet were not identified as a high risk patient per Baveno VI criteria. 16 patients in total had esophageal varices (21.3%). 11 had small esophageal varices (without high risk stigmata) and 5 had high risk varices requiring intervention (large varices or varices with high risk stigmata). For Baveno VI criteria predicting the presence of any varices, the sensitivity was 75% (12/16), specificity was 84.8% (50/59), positive predictive value was 57.1% (12/21), and the negative predictive value was 92.6% (50/54). Baveno VI criteria identified all patients with high risk varices (5/5). CONCLUSION: With a robust negative predictive value in a safety net hospital population, Baveno VI criteria seems to be an adequate method for identifying patients who can avoid screening endoscopy for esophageal varices.
Objectives: Lack of medication persistence with antidiabetic drug therapy increases risk of diabetes complications and hospitalizations. This study assessed the impact of type of initial antidiabetic medications, demographic and clinical factors on persistence among adult patients with type 2 diabetes (T2D). Methods: We identified adults aged over 26 years with T2D who initiated treatment with oral antidiabetic therapy using Optum's De-identified Clinformatics® Data Mart database [2007][2008][2009][2010][2011][2012][2013][2014][2015][2016][2017][2018]. Patients were required to have continuous enrollment .=12 months before and .=12 months after the index prescription. The initial treatment regimen was defined as all medications used in the 6-week period following the first antidiabetic medication to capture adjustments that are common in real world practice. Persistence was measured using time to discontinuation [time to a gap in all therapy .=60 days]. The primary independent variable of interest was the medication class(es) used as initial therapy. Demographic and clinical characteristics included age, gender, health insurance, prior healthcare utilization, diagnostic mix and prior use of nondiabetic medications. The association between initial therapy and patients' characteristics and the likelihood of discontinuation was estimated using Cox proportional hazards models. Results: A total of 229,485 patients meeting study criteria were identified. Patients initiating treatment using sulfonylurea monotherapy, thiazolidinedione (TZD) monotherapy, or combination therapy were 5.2%, 28.7%, and 19.9% significantly less likely to discontinue initial therapy than patients initiating treatment on metformin monotherapy. Patients who were older, had lower prior medical costs, and higher prior drug costs were significantly less likely to discontinue their initial therapy. Patients who received anti-infectives, autonomic drugs, central nervous agents, muscle relaxants, and respiratory agents were significantly more likely to discontinue while patients who received cardiovascular drugs and electrolytic agents were significantly less likely to discontinue. Conclusions: Patients initiating sulfonylurea, TZD, or combination therapy were significantly less likely to discontinue their initial treatment regimen.
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