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    Alex Straumann

    Strictures are a frequent complication of eosinophilic esophagitis. The efficacy and safety of topical corticosteroids and of dilation of eosinophilic esophagitis-associated strictures have not yet been thoroughly clarified. We present a... more
    Strictures are a frequent complication of eosinophilic esophagitis. The efficacy and safety of topical corticosteroids and of dilation of eosinophilic esophagitis-associated strictures have not yet been thoroughly clarified. We present a retrospective analysis of 10 adult patients with eosinophilic esophagitis who had symptomatic esophageal stenosis that was unresponsive to topical corticosteroids, and who were treated using bougienage. Eight patients had one single stricture, one patient had two, and another had three strictures; mean stricture length was 2.1 cm (range 1 - 6 cm). Bougienage led to prompt symptom relief. Apart from transient postprocedural odynophagia, no severe complications occurred. During the follow-up (mean 6 months; range 2 - 11 months), all patients enjoyed sustained treatment response.
    Eosinophilic oesophagitis (EO) is a chronic inflammatory disorder of the oesophagus that is rapidly increasing in prevalence. Although systemic and topical corticosteroids are effective in treating EO, some patients develop corticosteroid... more
    Eosinophilic oesophagitis (EO) is a chronic inflammatory disorder of the oesophagus that is rapidly increasing in prevalence. Although systemic and topical corticosteroids are effective in treating EO, some patients develop corticosteroid dependency. Alternative therapeutic approaches that avoid corticosteroids are scarce. To analyse our experience at inducing and maintaining remission with an immunomodulatory therapy in steroid-dependent EO patients. We analysed the clinical and histological response to azathioprine (AZA) and 6-mercaptopurine in three patients with EO (one also with eosinophilic gastroenteritis) and corticosteroid dependency. In all three patients, AZA or 6-mercaptopurine-induced clinical and histological remission that was maintained during the follow-up period (range 3-8 years). Two patients experienced relapses after ceasing AZA therapy. Remission, however, resumed when short-term corticosteroid treatment was followed by AZA. In all the patients, blood eosinophilia disappeared under AZA treatment. Only jumbo biopsies confirmed suspected EO with predominant muscle-layer involvement in one patient. In adult patients with corticosteroid-dependent EO, immunomodulatory treatment with purine analogues is a promising therapeutic approach for inducing and maintaining long-term remission without the need for further corticosteroids. Jumbo forcep biopsies might be needed to confirm a diagnosis of muscle-layer predominant EO.
    Eosinophilic esophagitis is characterized by symptoms of esophageal dysfunction and eosinophil-predominant esophageal inflammation. Eosinophilic inflammation in other parts of the gastrointestinal tract is absent and several differential... more
    Eosinophilic esophagitis is characterized by symptoms of esophageal dysfunction and eosinophil-predominant esophageal inflammation. Eosinophilic inflammation in other parts of the gastrointestinal tract is absent and several differential diagnoses for esophageal eosinophilia have to be excluded before diagnosing eosinophilic esophagitis. Most patients are male and have concomitant atopic disorders. Therapeutic options are based on drugs, diet and dilation.
    Eosinophilic esophagitis (EoE) is a relatively new chronic inflammatory disease of the esophagus. Novel aspects in the diagnosis and treatment of EoE are discussed in this review. Development of diagnostic guidelines consisting of... more
    Eosinophilic esophagitis (EoE) is a relatively new chronic inflammatory disease of the esophagus. Novel aspects in the diagnosis and treatment of EoE are discussed in this review. Development of diagnostic guidelines consisting of symptoms as well as endoscopic and histopathologic criteria has improved the awareness of clinicians and the diagnosing of EoE. The clinically challenging discrimination of EoE from gastroesophageal reflux disease has become more obvious and has evoked the need for more individualized therapy options. Today, most patients with EoE can be treated successfully with topical corticosteroids. However, no convincing alternative treatment strategies exist for corticosteroid-refractory or corticosteroid-dependent patients. Interestingly, a single-nucleotide polymorphism in the eotaxin-3 gene has been linked to increased disease susceptibility for EoE. EoE is an emerging esophageal disease. A better understanding of the pathogenesis of this disorder would improve the clinical recognition and diagnostic ability and ultimately provide patients with more optimal treatment options.
    The activity of a particular esophageal disease, such as eosinophilic esophagitis (EoE) or achalasia, can be evaluated using clinician-reported outcome (ClinRO) measures assessing various endoscopic, histologic, functional, and laboratory... more
    The activity of a particular esophageal disease, such as eosinophilic esophagitis (EoE) or achalasia, can be evaluated using clinician-reported outcome (ClinRO) measures assessing various endoscopic, histologic, functional, and laboratory findings, and patient-reported outcome (PRO) measures. The patient-reported outcome (PRO) measures are those that are designed to be self-reported by patients. Commonly used PRO instruments include those that assess symptom severity, health-related quality of life, "general" quality of life, or health status. Regulatory authorities increasingly rely on PRO measures to support labeling claims for drug development. Validated PRO measures for various esophageal diseases are needed in order to unify and standardize the way disease activity is assessed, define clinically meaningful endpoints for use in interventional and observational studies, compare the efficacy/effectiveness of various therapies, and optimize therapeutic algorithms for management of these diseases. This article reviews commonly used PRO instruments designed to assess symptom severity and quality of life in adult patients with EoE and achalasia.
    We evaluated the diagnostic delay (time from first symptoms to diagnosis) in 100 pediatric patients with Crohn's disease (CD) and 75 patients with ulcerative coltiis (UC). Median [interquartile range] diagnostic delay in CD patients... more
    We evaluated the diagnostic delay (time from first symptoms to diagnosis) in 100 pediatric patients with Crohn's disease (CD) and 75 patients with ulcerative coltiis (UC). Median [interquartile range] diagnostic delay in CD patients was 4 [2-8] (range 0-82) months compared with 2 [1-7] (range 0-52) months in UC patients (p = 0.003). The time interval from first physician visit to IBD diagnosis was longer in CD patients and UC patients when compared to the time interval from symptom onset to first physician visit (CD: median 3 vs. 1 months, p < 0.001; UC: median 2 vs. 0 months, p < 0.001). No specific risk factors were identified for the length of diagnostic delay. Measures should be taken to reduce diagnostic delay.
    Eosinophilic Esophagitis (EoE), first described in the early 1990's, has rapidly evolved as distinctive chronic inflammatory esophageal disease. The diagnosis is based clinically by the presence of symptoms related... more
    Eosinophilic Esophagitis (EoE), first described in the early 1990's, has rapidly evolved as distinctive chronic inflammatory esophageal disease. The diagnosis is based clinically by the presence of symptoms related to an esophageal dysfunction and histologically by an eosinophil-predominant inflammation once other conditions leading to esophageal eosinophilia are excluded. This striking male-prevalent disease has an increasing incidence and prevalence in the westernised countries. Currently, EoE represents the main cause of dysphagia and bolus impaction in adult patients. Despite the fact that EoE often occurs in atopic patients, the value of allergic testing is still under discussion. Topical corticosteroids lead to a rapid improvement of active EoE clinically and histologically; they are therefore regarded as first-line drug therapy. Elimination diets have similar efficacy as topical corticosteroids, but their long-term use is limited by practical issues. Esophageal dilation of EoE-induced strictures can also be effective in improving symptoms, but this therapy has no effect on the underlying inflammation. Neither the diagnostic nor the long-term therapeutic strategies are yet fully defined.
    Patients with M. Crohn or colitis ulcerosa live with a chronic disease. Pharmaceuticals used in convention-al medicine have been proven to be effective but can have strong side-effects. Thus, it is no surprise that affected patients are... more
    Patients with M. Crohn or colitis ulcerosa live with a chronic disease. Pharmaceuticals used in convention-al medicine have been proven to be effective but can have strong side-effects. Thus, it is no surprise that affected patients are very interested in complementary and alternative medicine (CAM). The aim of our survey was to investigate the use and the application of CAM by patients with M. Crohn and colitis ulcerosa in Switzerland. Out-patients of the gastroenterological centre at the University Hospital Bern and of two gastroenterological private practices in a medium-sized city completed a self-administered questionnaire on CAM. Demographic variables, disease-related data, use of different approaches of complementary medicine, attitudes towards and the use of CAM were asked for. Out of 204 mailed questionnaires 71% (144) could be evaluated, 44% from patients of the University Hospital, 56% from patients of the gastroenterological private practices. CAM was used by 47% of the patients. The most commonly used methods of CAM were: homeopathy, traditional Chinese medicine and acupuncture. 67% of the patients benefitted by CAM in the long run, whereas 10.5% suffered a relapse of their disease during CAM therapies. The main reason for the patients to apply to CAM was to complement conventional treatment. Our survey points out that the studied out-patients have a strong interest in CAM. It is therefore important that clinical research in the various unconventional therapies will be further promoted.
    At present, it is still debated whether the focus of treatment in eosinophilic esophagitis (EoE) should be directed toward a symptomatic or histological response, or even toward a combination of both. This question cannot be answered as... more
    At present, it is still debated whether the focus of treatment in eosinophilic esophagitis (EoE) should be directed toward a symptomatic or histological response, or even toward a combination of both. This question cannot be answered as long as we have no solid data evaluating the long-term consequences of untreated symptoms and untreated inflammation. Nevertheless, today there are at least three established reasons to treat patients with clinically and histologically active EoE. First, dysphagia has a substantial negative impact on the patient's daily life. Despite the fact that patients often cope with this symptom and accept even marked restrictions in their nutritional habits, they usually experience a marked enhancement in quality of life under treatment. Second, untreated EoE harbors the risk of long-lasting food impactions. There is evidence that this unpleasant, unforeseeable and even risky incident can be prevented by efficient treatment of the eosinophilic inflammation. Third, it has been demonstrated in several clinical studies as well as in animal models that unbridled eosinophilic inflammation leads to a so-called remodeling of the esophagus with wall thickening, stiffness of the organ and stricture. Prevention of esophageal damage caused by tissue remodeling is therefore another reason to strongly advocate a consequential treatment. Finally, there is increasing evidence that untreated EoE might be a risk factor for acute infection of the esophagus with herpes simplex virus (HSV), leading to a severe ulcerative and extremely painful esophagitis. Prevention of HSV esophagitis might therefore be a fourth indication for treating EoE.
    Eosinophilic esophagitis (EoE), which was first described in the early 1990s, has rapidly evolved as a distinctive chronic inflammatory esophageal disease. The diagnosis is based clinically on the presence of symptoms related to... more
    Eosinophilic esophagitis (EoE), which was first described in the early 1990s, has rapidly evolved as a distinctive chronic inflammatory esophageal disease. The diagnosis is based clinically on the presence of symptoms related to esophageal dysfunction and histologically by an eosinophil-predominant inflammation once other conditions leading to esophageal eosinophilia are excluded. This striking male-prevalent disease has an increasing incidence and prevalence in the Westernized countries. Currently, EoE represents the main cause of dysphagia and bolus impaction in adult patients. Despite the fact that EoE often occurs in atopic patients, the value of allergic testing is still under discussion. Topical corticosteroids lead to a rapid improvement of active EoE clinically and histologically; they are therefore regarded as first-line drug therapy. Elimination diets have similar efficacy as topical corticosteroids, but their long-term use is limited by practical issues. Esophageal dilation of EoE-induced strictures can also be effective in improving symptoms, but this therapy has no effect on the underlying inflammation. Neither the diagnostic nor the long-term therapeutic strategies have been fully defined. Currently, the list of unsolved issues--or mysteries--is still long and a concerted effort on behalf of clinicians and scientists is required to improve the understanding and the therapeutic management of this mysterious disease.
    Consensus diagnostic recommendations to distinguish GORD from eosinophilic oesophagitis (EoE) by response to a trial of proton pump inhibitors (PPIs) unexpectedly uncovered an entity called 'PPI-responsive oesophageal... more
    Consensus diagnostic recommendations to distinguish GORD from eosinophilic oesophagitis (EoE) by response to a trial of proton pump inhibitors (PPIs) unexpectedly uncovered an entity called 'PPI-responsive oesophageal eosinophilia' (PPI-REE). PPI-REE refers to patients with clinical and histological features of EoE that remit with PPI treatment. Recent and evolving evidence, mostly from adults, shows that patients with PPI-REE and patients with EoE at baseline are clinically, endoscopically and histologically indistinguishable and have a significant overlap in terms of features of Th2 immune-mediated inflammation and gene expression. Furthermore, PPI therapy restores oesophageal mucosal integrity, reduces Th2 inflammation and reverses the abnormal gene expression signature in patients with PPI-REE, similar to the effects of topical steroids in patients with EoE. Additionally, recent series have reported that patients with EoE responsive to diet/topical steroids may also achi...
    It is not clear whether symptoms alone can be used to estimate the biologic activity of eosinophilic esophagitis (EoE). We aimed to evaluate whether symptoms can be used to identify patients with endoscopic and histologic features of... more
    It is not clear whether symptoms alone can be used to estimate the biologic activity of eosinophilic esophagitis (EoE). We aimed to evaluate whether symptoms can be used to identify patients with endoscopic and histologic features of remission. Between April, 2011 and June, 2014, we performed a prospective, observational study and recruited 269 consecutive adults with EoE (67% male; median age, 39 years old) in Switzerland and the United States. Patients first completed the validated symptom-based EoE activity index patient-reported outcome instrument and then underwent esophagogastroduodenoscopy with esophageal biopsy collection. Endoscopic and histologic findings were evaluated with a validated grading system and standardized instrument, respectively. Clinical remission was defined as symptom score <20 (range 0-100); histologic remission was defined as a peak count of <20 eosinophils/mm(2) in a high-power field (corresponds to approximately <5 eosinophils/median high-powe...
    Eosinophililc esophagitis is a clinicopathologic disease characterized clinically by dysphagia and food impaction in adults and nonspecific symptoms of gastroesophageal reflux disease in children, and histologically by large numbers of... more
    Eosinophililc esophagitis is a clinicopathologic disease characterized clinically by dysphagia and food impaction in adults and nonspecific symptoms of gastroesophageal reflux disease in children, and histologically by large numbers of eosinophils in the proximal and distal esophageal epithelium. Importantly, these symptoms and histologic abnormalities appear to be unresponsive to proton pump inhibition. Recent clinical and basic studies suggest an allergic
    Background  Eosinophilic esophagitis (EoE) represents a relatively new, rapidly increasing, chronic inflammatory disease of the esophagus. Methods  To present a review of the recent literature, we performed a PubMed search for... more
    Background  Eosinophilic esophagitis (EoE) represents a relatively new, rapidly increasing, chronic inflammatory disease of the esophagus. Methods  To present a review of the recent literature, we performed a PubMed search for eosinophilic esophagitis, pathogenesis, and treatments. Results  EoE is defined clinicopathologically by esophagus- related symptoms in combination with a dense esophageal eosinophilia, both of which are refractory to treatment with proton pump inhibitors. EoE can affect patients at any age. In adult patients the leading symptom is dysphagia for solids, often leading to food impaction. Children with EoE present mainly with vomiting, thoracic and abdominal pain, and failure to grow. EoE can mimic symptoms and findings associated with gastroesophageal reflux disease. Studies to date suggest an allergy-triggered etiology with an overriding Th2 phenotype. Current treatments include topical and systemic corticosteroids, immunosuppressants, immunomodulators, dilation, and hypoallergic diets. Conclusions  EoE represents a immune-mediated disease of undetermined pathogenesis. Natural history studies as well as prospective therapeutic trials will be critical to defining future treatment strategies.
    Data evaluating the chronological order of appearance of extraintestinal manifestations (EIMs) relative to the time of inflammatory bowel disease (IBD) diagnosis is currently lacking. We aimed to assess the type, frequency, and... more
    Data evaluating the chronological order of appearance of extraintestinal manifestations (EIMs) relative to the time of inflammatory bowel disease (IBD) diagnosis is currently lacking. We aimed to assess the type, frequency, and chronological order of appearance of EIMs in patients with IBD. Data from the Swiss Inflammatory Bowel Disease Cohort Study were analyzed. The data on 1249 patients were analyzed (49.8% female, median age: 40 [interquartile range, 30-51 yr], 735 [58.8%] with Crohn's disease, 483 [38.7%] with ulcerative colitis, and 31 [2.5%] with indeterminate colitis). A total of 366 patients presented with EIMs (29.3%). Of those, 63.4% presented with 1, 26.5% with 2, 4.9% with 3, 2.5% with 4, and 2.7% with 5 EIMs during their lifetime. Patients presented with the following diseases as first EIMs: peripheral arthritis 70.0%, aphthous stomatitis 21.6%, axial arthropathy/ankylosing spondylitis 16.4%, uveitis 13.7%, erythema nodosum 12.6%, primary sclerosing cholangitis 6.6...
    Physicians traditionally treat ulcerative colitis (UC) using a step-up approach. Given the paucity of data, we aimed to assess the cumulative probability of UC-related need for step-up therapy and to identify escalation-associated risk... more
    Physicians traditionally treat ulcerative colitis (UC) using a step-up approach. Given the paucity of data, we aimed to assess the cumulative probability of UC-related need for step-up therapy and to identify escalation-associated risk factors. Patients with UC enrolled into the Swiss IBD Cohort Study were analyzed. The following steps from the bottom to the top of the therapeutic pyramid were examined: (1) 5-aminosalicylic acid and/or rectal corticosteroids, (2) systemic corticosteroids, (3) immunomodulators (IM) (azathioprine, 6-mercaptopurine, methotrexate), (4) TNF antagonists, (5) calcineurin inhibitors, and (6) colectomy. Data on 996 patients with UC with a median disease duration of 9 years were examined. The point estimates of cumulative use of different treatments at years 1, 5, 10, and 20 after UC diagnosis were 91%, 96%, 96%, and 97%, respectively, for 5-ASA and/or rectal corticosteroids, 63%, 69%, 72%, and 79%, respectively, for systemic corticosteroids, 43%, 57%, 59%, a...
    Long-lasting food impactions requiring endoscopic bolus removal occur frequently in patients with eosinophilic esophagitis (EoE) and harbor a risk for severe esophageal injuries. We evaluated whether treatment with swallowed topical... more
    Long-lasting food impactions requiring endoscopic bolus removal occur frequently in patients with eosinophilic esophagitis (EoE) and harbor a risk for severe esophageal injuries. We evaluated whether treatment with swallowed topical corticosteroids is able to reduce the risk of occurrence of this complication. We analyzed data from the Swiss EoE Cohort Study. Patients with yearly clinic visits, during which standardized assessment of symptoms, endoscopic, histologic, and laboratory findings was carried out, were included. A total of 206 patients (157 males) were analyzed. The median follow-up time was 5 years with a total of 703 visits (mean 3.41 visits/patient). During the follow-up period, 33 patients (16 % of the cohort) experienced 42 impactions requiring endoscopic bolus removal. We evaluated the following factors regarding the outcome 'bolus impaction' by univariate logistic regression modeling: swallowed topical corticosteroid therapy (OR 0.503, 95%-CI 0.255-0.993, P ...
    Eosinophilic esophagitis is a chronic disease limited to the esophagus and has a persistent or spontaneously fluctuating course. So far it does not seem to limit life expectancy, but it often substantially impairs the quality of life. To... more
    Eosinophilic esophagitis is a chronic disease limited to the esophagus and has a persistent or spontaneously fluctuating course. So far it does not seem to limit life expectancy, but it often substantially impairs the quality of life. To date, there has been no association with malignant conditions, but there is concern that the chronic, uncontrolled inflammation will evoke irreversible structural alterations of the esophagus, leading to tissue fibrosis, stricture formation, and impaired function. This esophageal remodeling may result in several disease-inherent and procedure-related complications.
    Esophageal dilation often leads to long-lasting relief of dysphagia in eosinophilic esophagitis (EoE). The aim of this study was to define the effectiveness, safety, and patient acceptance of esophageal dilation in EoE. In addition, we... more
    Esophageal dilation often leads to long-lasting relief of dysphagia in eosinophilic esophagitis (EoE). The aim of this study was to define the effectiveness, safety, and patient acceptance of esophageal dilation in EoE. In addition, we examined the influence of dilation on the underlying esophageal inflammation. Two databases including 681 EoE patients were reviewed. Cohort 1 consisted of patients treated with dilation alone, whereas cohort 2 included patients treated with a combination of dilation and antieosinophilic medication. Patients from cohort 1 underwent a prospective histological reexamination and an evaluation using a questionnaire. In total, 207 EoE patients were treated with esophageal dilation, 63 in cohort 1 and 144 in cohort 2. Dilation led to a significant increase in esophageal diameter and to an improvement in dysphagia in both the cohorts (P<0.001). After dilation, dysphagia recurred after 23+/-22 months in cohort 1 and 20+/-14 months in cohort 2. No esophagea...

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