Eosinophilic Colitis Research Articles

Eosinophilic gastroenteritis, a rare case report and literature review

Abstract Introduction/Objective Eosinophilic gastroenteritis (EGE) is a rare inflammatory condition affecting the gastrointestinal tract, initially described by Kaijser et al. in 1937. It manifests as eosinophil-rich infiltration throughout the GI tract, often accompanied by peripheral eosinophilia. EGE is categorized under eosinophilic gastrointestinal disorders (EGIDs), alongside eosinophilic esophagitis (EOE) and eosinophilic colitis (EC). Symptoms vary based on the affected GI segment. This report aims to present a case of extensive EGE in a young male patient and review of similar cases to enhance clinical and histopathologic understanding. Methods/Case Report A 29-year-old male presented with abdominal pain, nausea, vomiting, and recent weight loss. He had a history of similar abdominal symptoms for a few years. Previous extensive investigations were inconclusive. Current imaging revealed soft tissue nodules, mild splenomegaly, and biliary dilation. Laboratory tests showed no significant abnormalities. Endoscopy revealed a gastric antral ulcer with marked eosinophilic infiltration on microscopy. Results (if a Case Study enter NA) NA Conclusion Eosinophilic gastroenteritis is an uncommon disease that presents with variable non-specific symptoms which require a high level of suspicion for diagnosis. EGE diagnosis requires excluding other causes of eosinophil accumulation. There is no clear distinction between the upper normal and abnormally increased eosinophils in GIT biopsies. Peripheral eosinophilia is common, along with other abnormal laboratory findings. Diagnosis relies on histopathology due to nonspecific symptoms. Clinicians and pathologists face challenges in diagnosing EGE, emphasizing the importance of considering it in the differential diagnoses. Efforts to establish diagnostic criteria for EGE are limited, despite its long-known existence. The condition’s vague symptoms necessitate a high level of clinical suspicion, with diagnosis primarily based on histopathologic features.

S3065 CART-Induced Eosinophilic Colitis With Good Response to Corticosteroids and Infliximab

S3065 CART-Induced Eosinophilic Colitis With Good Response to Corticosteroids and Infliximab

S2993 Mycophenolate-Induced Eosinophilic Colitis

S2993 Mycophenolate-Induced Eosinophilic Colitis

Eosinophilic gastrointestinal disorders and the role for the epithelium in pathogenesis and treatment.

This review aims to provide an overview of the current understanding of eosinophilic gastrointestinal disorders (EGIDs) and the role of the epithelium in influencing disease pathogenesis to inform and devise future therapeutic strategies. Changes in epithelial cell structure, functions, and integrity are observed in EGIDs. In eosinophilic esophagitis (EoE), the esophageal epithelium has been shown to play key roles in perpetuating the inflammatory response in EoE through the expression of pro-inflammatory cytokines and immunological cell-surface proteins. Similar mechanisms appear to exist in the other EGIDs, including eosinophilic gastritis (EoG), eosinophilic enteritis (EoN), and eosinophilic colitis (EoC). Because of the increasing rarity of each non-EoE EGID, research focusing on how the epithelium is modulating disease in each lower gastrointestinal compartment is still in its rudimentary stages. While there has been significant progress in understanding the role of the epithelium in EoE, further research is needed to obtain a better understanding of the mechanisms mediating epithelial-immune crosstalk in non-EoE EGIDs. Using EoE-epithelial cell research to inform future EGID investigations could lead to the development of new therapeutic interventions, such as targeted therapies to restore epithelial barrier function and reduce inflammation, to improve rare disease-patient quality of life.

Clinical significance of direct fast scarlet staining on the diagnosis of eosinophilic colitis: A comparative study focusing on the eosinophil degranulation in colonic mucosal tissue.

This study aimed to validate the DFS (direct fast scarlet) staining in the diagnosis of EC (eosinophilic colitis). The study included 50 patients with EC and 60 with control colons. Among the 60 control samples, 39 and 21 were collected from the ascending and descending colons, respectively. We compared the median number of eosinophils and frequency of eosinophil degranulation by HE (hematoxylin and eosin) and DFS staining between the EC and control groups. In the right hemi-colon, eosinophil count by HE was useful in distinguishing between EC and control (41.5 vs. 26.0 cells/HPF, p < 0.001), but the ideal cutoff value is 27.5 cells/HPF (high-power field). However, this method is not useful in the left hemi-colon (12.5 vs. 13.0 cells/HPF, p = 0.990). The presence of degranulation by DFS allows us to distinguish between the groups even in the left hemi-colon (58% vs. 5%, p < 0.001). DFS staining also enabled a more accurate determination of degranulation than HE. According to the current standard to diagnose EC (count by HE staining ≥20 cells/HPF), mucosal sampling from left hemi-colon is problematic since the number of eosinophils could not be increased even in EC. Determination of degranulated eosinophils by DFS may potentiate the diagnostic performance even in such conditions.

The Dual Lens of Endoscopy and Histology in the Diagnosis and Management of Eosinophilic Gastrointestinal Disorders-A Comprehensive Review.

Eosinophilic Gastrointestinal Disorders (EGIDs) are a group of conditions characterized by abnormal eosinophil accumulation in the gastrointestinal tract. Among these EGIDs, Eosinophilic Esophagitis (EoE) is the most well documented, while less is known about Eosinophilic Gastritis (EoG), Eosinophilic Enteritis (EoN), and Eosinophilic Colitis (EoC). The role of endoscopy in EGIDs is pivotal, with applications in diagnosis, disease monitoring, and therapeutic intervention. In EoE, the endoscopic reference score (EREFS) has been shown to be accurate in raising diagnostic suspicion and effective in monitoring therapeutic responses. Additionally, endoscopic dilation is the first-line treatment for esophageal strictures. For EoG and EoN, while the literature is more limited, common endoscopic findings include erythema, nodules, and ulcerations. Histology remains the gold standard for diagnosing EGIDs, as it quantifies eosinophilic infiltration. In recent years, there have been significant advancements in the histological understanding of EoE, leading to the development of diagnostic scores and the identification of specific microscopic features associated with the disease. However, for EoG, EoN, and EoC, precise eosinophil count thresholds for diagnosis have not yet been established. This review aims to elucidate the role of endoscopy and histology in the diagnosis and management of the three main EGIDs and to analyze their strengths and limitations, their interconnection, and future research directions.

Clinical and atopic features of patients with primary eosinophilic colitis: an Italian multicentre study

Eosinophilic colitis (EC) is the rarest among primary eosinophilic gastrointestinal disorders (EGID). EC is underdiagnosed due to its blurred and proteiform clinical manifestations. To explore the clinical and atopic characteristic of EC adult patients, the diagnostic delay, and relapse-associated factors, by comparison with patients with eosinophilic esophagitis (EoE) and irritable bowel syndrome (IBS). EC patients followed-up at four clinics were included, and clinical, histopathological, and laboratory data were retrieved. As control groups, age-matched patients with EoE and IBS were recruited. Allergy tests included skin prick test and serum specific IgE. Diagnostic delay was assessed. Overall, data from 73 patients were retrieved, including 40 with EC (median age 39 years IQR 22.5–59, F:M 2.1:1), 12 with EoE (F:M ratio: 1:5), and 21 with IBS (F:M ratio: 1:0.9). The most common features in EC patients were female sex (67.5%), atopy (77.5%), abdominal pain/distention (70%), diarrhoea (77.5%), and faecal calprotectin elevation (22.5%). Blood eosinophils were elevated in EoE, but not in EC (p < 0.001), while ECP did not differ across the three groups (p = 0.4). The frequency of allergen sensitization reached 25% of patients. Several frequent pan-allergens for this region were present. The overall diagnostic delay was 10 months (IQR 4–15). Factors contributing to a greater diagnostic delay were atopy, weight loss, and a previous misdiagnosis. EC is mostly a diagnosis of exclusion, burdened by a substantial diagnostic delay. In female patients the presence of allergen sensitization, abdominal symptoms and faecal calprotectin elevation should raise the suspicion of EC.

SOCIOECONOMIC DISPARITIES IN HOSPITALIZATION AND OUTCOMES OF EOSINOPHILIC COLITIS: A FIVE-YEAR NATIONAL ANALYSIS

Abstract BACKGROUND Eosinophilic colitis (EoC) is a rare gastrointestinal inflammation of the colon due to an altered hypersensitivity reaction characterized by eosinophilic infiltration into the colonic wall. Patients usually present with abdominal pain, diarrhea, and weight loss. METHODS We used the National Inpatient Sample (NIS) database to conduct a retrospective study of hospitalized adult patients with eosinophilic colitis using the validated ICD-10 diagnosis codes between January 1, 2016, and December 31, 2020. RESULTS We identified 4353 cases of eosinophilic colitis using the NIS database. There were 748, 776, 889, 934, and 1,006 eosinophilic colitis cases diagnosed in 2016, 2017, 2018, 2019, and 2020 respectively. Males were more likely to be hospitalized due to eosinophilic colitis than females (51.8% vs. 48.2%). Moreover, there was a significantly higher prevalence of eosinophilic colitis among the white population, with 81.7% in Whites, 8.1% in Blacks, 6.3% in Hispanics, and 1.3% in Asians. 26.5% of those patients had Medicare, 15.7% had Medicaid, and 50.6% had private/HMO insurance. About 20.7% of patients had a median household income of $45,999 or less, 23.6% had $46,000-58,999, and 55.6% had $59,000 or more. Based on hospital location, 30.3% of the cases were in the South, 27.5% in the Midwest, 23.4% in the West, and 18.8% in the Northeast. The majority of patients admitted for eosinophilic colitis were recorded in urban teaching hospitals (77.8%) compared to 17.6% in urban nonteaching hospitals and 4.6% in rural areas. Only 16.5% of the patients with eosinophilic colitis had a Charlson Comorbidity Index of 3 or higher. CONCLUSION Based on the national inpatient sample over 5 years, males and whites are more likely to be hospitalized with eosinophilic. The report suggests that eosinophilic colitis is common among patients with high median household income and has a better prognosis.

A case of exuberant endoscopic presentation of asymptomatic eosinophilic colitis.

Eosinophilic colitis is a rare entity characterized by a broad range of gastrointestinal symptoms and idiopathic infiltration of eosinophils in the colon. This condition is most likely underreported due to the absence of standardized diagnostic criteria. We present the case of a 72-year-old man who underwent an outpatient colorectal cancer surveillance colonoscopy without gastrointestinal complaints. Colonoscopy revealed a diffuse micronodular pattern of the colonic mucosa, with histopathological analysis showing a polymorphic infiltrate rich in eosinophils in the lamina propria. The patient was kept under surveillance, with a repeat colonoscopy showing persistent endoscopic and histological findings consistent with eosinophilic colitis. The patient remains asymptomatic. This case is unique from both clinical and endoscopic perspectives due to the asymptomatic course of this rare condition and the illustrative iconography.

Review article: Emerging insights into the epidemiology, pathophysiology, diagnostic and therapeutic aspects of eosinophilic oesophagitis and other eosinophilic gastrointestinal diseases.

Eosinophilic gastrointestinal diseases (EGIDs) are chronic, immune-mediated disorders characterised clinically by gastrointestinal symptoms and histologically by a pathologic increase in eosinophil-predominant inflammation in the gastrointestinal tract, in the absence of secondary causes of eosinophilia. To highlight emerging insights and research efforts into the epidemiology, pathophysiology, diagnostic and therapeutic aspects of eosinophilic oesophagitis (EoE) and non-EoE EGIDs, and discuss key remaining knowledge gaps. We selected and reviewed original research, retrospective studies, case series, randomised controlled trials, and meta-analyses. Standardised nomenclature classifies EGIDs as EoE, eosinophilic gastritis (EoG), eosinophilic enteritis (EoN), and eosinophilic colitis (EoC). Incidence and prevalence of EoE are rising, emphasising the need to better understand how environmental risk factors and genetic features interact. Advances in understanding EoE pathophysiology have led to clinical trials of targeted therapy and the approval (in the United States) of dupilumab for EoE. Several therapies that are under investigation hope to satisfy both histologic and clinical targets. For non-EoE EGIDs, efforts are focused on better defining clinical and histopathologic disease determinants and natural history, as well as establishing new therapies. Unmet needs for research are dramatically different for EoE and non-EoE EGIDs. In EoE, non-invasive diagnostic tests, clinicopathologic models that determine the risk of disease progression and therapeutic failure, and novel biologic therapies are emerging. In contrast, in non-EoE EGIDs, epidemiologic trends, diagnostic histopathologic thresholds, and natural history models are still developing for these more rare disorders.

Current and Novel Therapies for Eosinophilic Gastrointestinal Diseases.

Eosinophilic gastrointestinal diseases (EGIDs) are an emerging group of pathological entities characterized by an eosinophil-predominant infiltration of different tracts of the gut in the absence of secondary causes of eosinophilia. According to the specific tract of the gut involved, EGIDs can be classified into eosinophilic esophagitis (EoE), eosinophilic gastritis (EoG), eosinophilic enteritis (EoN), and eosinophilic colitis (EoC). The epidemiology of EGIDs is evolving rapidly. EoE, once considered a rare disease, now has an incidence and prevalence of 7.7 new cases per 100,000 inhabitants per years and 34.4 cases per 100,000 inhabitants per year, respectively. Fewer data are available regarding non-EoE EGIDs, whose prevalence are estimated to range between 2.1 and 17.6 in 100,000 individuals, depending on age, sex, and ethnicity. Diagnosis requires the presence of suggestive symptoms, endoscopic biopsies showing abnormal values of eosinophils infiltrating the gut, and exclusion of secondary causes of eosinophilia. EoE typically presents with dysphagia and episodes of food bolus impactions, while EoG, EoN, and EoC may all present with abdominal pain and diarrhea, with or without other non-specific symptoms. In addition, although different EGIDs are currently classified as different entities, there may be overlap between different diseases in the same patient. Despite EGIDs being relatively novel pathological entities, the research on possible treatments is rapidly growing. In this regard, several randomized controlled trials are currently ongoing to investigate novel molecules, including ad-hoc steroid formulations, immunosuppressants, and mostly monoclonal antibodies that target the specific molecular mediators of EGIDs. This narrative review provides an up-to-date overview of available and investigational drugs for different EGIDs.

Eosinophilic gastroenteritis and colitis related to cow’s milk allergy: A case report

Objective: To provide evidence on clinical, laboratory, histopathological features, diagnostic and treatment of primary eosinophilic gastroenteritis in children. Case presentation: A 7-year-old boy admitted to the 108 Military Central Hospital with symptoms of abdominal pain, nausea and vomiting, recurrent diarrhea related to cow's milk intakes. The child was diagnosed with eosinophilic gastroenteritis, combined with increased serum IgE. The disease was stabilized with corticosteroids, oral proton pump inhibitors (PPIs) combined with the elimination of cow's milk from the daily diet. Conclusion: Primary eosinophilic gastroenteritis in children was definitively diagnosed based on exclusion of other causes of eosinophilia and 3 features: Gastrointestinal symptoms, endoscopy showed gastrointestinal eosinophilic infiltration, peripheral eosinophilia. Corticosteroids are the most effective and useful symptom control drugs in most cases in combination with a diet, eliminating the allergic component.

S2502 Novel Use of an Interleukin-4 and Interleukin-13 Monoclonal Antibody to Treat Refractory Eosinophilic Colitis

S2502 Novel Use of an Interleukin-4 and Interleukin-13 Monoclonal Antibody to Treat Refractory Eosinophilic Colitis

S2282 A Case of Suspected Adult Eosinophilic Colitis

S2282 A Case of Suspected Adult Eosinophilic Colitis

Non-esophageal eosinophilic gastrointestinal diseases: a narrative review.

Eosinophilic gastrointestinal disorders are a group of rare diseases characterized by the infiltration of eosinophils in the gastrointestinal wall in a greater amount than in homeostatic conditions. 'Non-esophageal eosinophilic gastrointestinal disorders' is the umbrella term for all eosinophilic gastrointestinal disorders outside of the well known eosinophilic esophagitis. This includes eosinophilic gastritis, eosinophilic enteritis and eosinophilic colitis. The clinical presentation is atypical and not very different for the three disorders. The depth of infiltration has a bigger influence on the presenting symptoms than the disease location. Although the frequency of diagnosis and research in this subject is increasing over time, non-esophageal eosinophilic disorders are rare and high quality evidence is limited to date. In this narrative review, we provide an overview of the latest insights in the pathophysiology, diagnostic approach and available treatment options. Transcriptome studies have found the pathogenesis to be T helper type 2 driven. Various laboratory findings can be used to trigger raised suspicion and investigation with endoscopy. As the endoscopic appearance of the mucosa is normal in most cases, multiple biopsies in each segment are needed to quantify the amount of eosinophils in the tissue. Eosinophilic cut-offs for diagnosis are a controversial topic and a consensus is still lacking. A recently developed tissue based diagnostic platform which measures differentially expressed genes might be available in the future to classify patients with intermediate eosinophilic tissue levels under the cut-off. For the treatment, corticosteroids are still the cornerstone of treatment but promising research suggests a role of biologicals, such as Lirentelimab (anti-siglec 8) in particular.

Hypereosinophilia Syndrome: Myriad Presentation—A Case Report

AbstractHypereosinophilic syndrome is a myeloproliferative disorder that is distinguished with the presence of eosinophilia, which is an absolute eosinophil level of more than 1500/µL for no less than 6 months, no recognized cause for eosinophilia, and clear organ involvement characteristics. But, in some cases where patients require therapy to decrease eosinophil counts due to severe symptoms, a lower time period of hypereosinophilic is also admissible. In this case series, we have included three cases in which first case presented with the features of diarrhea, nausea, and decreased appetite who underwent colonoscopy and was diagnosed as a case of eosinophilic colitis. The second patient was a female came with abdominal pain and burning micturition, who was having eosinophilia on blood count examination and was diagnosed as a case of eosinophilic cystitis. Third case was young female who was diagnosed as eosinophilic endocarditis on two-dimensional echocardiography having dyspnea and rash and high eosinophil count on blood smear. All the three patients were started on steroids, who recovered well with medication and were discharged for follow-up after 7 days with complete blood count.

Early life exposures as risk factors for non-esophageal eosinophilic gastrointestinal diseases

ObjectivesEarly life exposures increase risk of eosinophilic esophagitis (EoE), but it is unknown whether they contribute to increased risk for non-EoE eosinophilic gastrointestinal diseases (EGIDs). We aimed to assess the association between prenatal, antenatal, and early life factors and non-EoE EGIDs. MethodsWe conducted a case-control study based in EGID Partners, an online patient-centered research network. Adults (≥18 years) with non-EoE EGIDs, caregivers of children <18 years of age with an EGID, and non-EGID adult controls were eligible. Subjects completed our Early Life Exposure Questionnaire, detailing maternal and early childhood exposures. We assessed for associations between non-EoE EGIDs and early life exposures, focusing on exposures previously evaluated in association with EoE. ResultsWe analyzed 61 non-EoE EGID cases and 20 controls. Of the EGID cases, 14 had eosinophilic gastritis, 19 had eosinophilic enteritis, 6 had eosinophilic colitis, and 22 had multiple areas affected; additionally, 30 had esophageal involvement. Relative to controls, EGID cases were more likely to have had antenatal/perinatal pregnancy-related complications (43% vs 13%; p = 0.02), NICU admission (20% vs 0%; p = 0.03), and antibiotics in infancy (43% vs 10%; p = 0.01). With adjustment for age at diagnosis, we observed increased odds of an EGID for pregnancy complications (aOR 3.83; 95% CI: 0.99–14.9) and antibiotic use in infancy (aOR 7.65; 95% CI: 1.28–45.7). ConclusionsEarly life factors, including pregnancy complications, NICU admission, and antibiotics in infancy, were associated with development of non-EoE EGIDs. The impact of early life exposures on non-EoE EGID pathogenic mechanisms should be investigated.

Gastrointestinal Eosinophilic Disorder in a Patient with Acute Pancreatitis. Case Report

Eosinophilic colitis is a rare disease. It is part of the eosinophilic gastroenteritis, characterized by eosinophilic infiltration of the tissues that affects any segment of the digestive tract, but more frequently the stomach and small intestine and rarely the colon in isolation. The clinical case of a 29- year-old patient is described, who initially presented data of acute pancreatitis and later acute diarrhea with sluggishness and scant mucus. The analysis showed a complete blood count with eosinophilia 9.9% Immunoglobulin E with a positive result + 2500 IU/mL (&lt;100IU/mL). Endoscopy and colonoscopies show diffuse inflammatory changes, confirming with the histopathological study infiltration of eosinophils throughout the gastrointestinal tract, conservative treatment was started with a good response. Eosinophilic gastroenteritis has a benign course, with significant infiltration of eosinophils in the wall of the digestive tract, which rarely involves only the colon. Its cause and pathogenic mechanisms are unknown; It classically presents in patients between the third and fifth decades of life, although it can affect any age group. The diagnostic criteria are: 1) gastrointestinal symptoms; 2) eosinophilic infiltration of one or more areas of the digestive tract demonstrated by biopsy; 3) absence of eosinophilic infiltration in organs outside the digestive tract; and 4) absence of parasitic infection. Eosinophilic gastroenteritis are pathologies that require a high index of suspicion for their diagnosis.

Diagnostic yield of random colon biopsy sampling in patients with chronic diarrhea and normal colonoscopy: a systematic review and meta-analysis

Background and AimsMost colonoscopy examinations in patients with chronic unexplained diarrhea do not reveal endoscopic abnormalities, and routine random colon biopsy specimens are taken for further diagnostic assessment. In this study, we aimed to perform a systematic review and meta-analysis to study the pooled diagnostic value of random colon biopsy sampling in this patient population.MethodsMultiple databases (MEDLINE, Scopus, Embase) were searched in May 2022 for studies evaluating the diagnostic yield of random biopsy sampling in patients with chronic diarrhea with normal colonoscopy examinations. Standard meta-analysis methods used the random-effects model, and heterogeneity was assessed using the I2 statistics.ResultsTwenty-one studies were included in the study (3322 patients). The pooled rate of normal colon biopsy sampling was 48% (95% confidence interval [CI], 31-65.2; I2 = 98%), and normal ileal biopsy sampling was 88.5% (95% CI, 51.3-98.3; I2 = 93%). The pooled rate of significant histopathologic change was 30% (95% CI, 20-41; I2 = 96%). The pooled rates of specific diagnoses based on random colon biopsy sampling were as follows: microscopic colitis, 15% (95% CI, 10-21; I2 = 92%); lymphocytic colitis, 9% (95% CI, 6 -13; I2 = 85%); collagenous colitis, 3.5% (95% CI, 2.4-5.7; I2 = 80%); nonspecific colitis, 20% (95% CI, 11-32.8; I2 = 97%); inflammatory bowel disease, 1.7% (95% CI, .8-3.5; I2 = 54%); and eosinophilic colitis, 2.3% (95% CI, 1.1-4.5; I2 = 69%).ConclusionsThis meta-analysis of 21 studies demonstrated significant histopathologic change in 30% and microscopic colitis in 15%. Normal colon biopsy sampling was noted in 48% and normal ileum biopsy sampling in 88.5%. Ileal biopsy data were based on limited data and studies (only 3 included). In studies with a mean patient age <49 years, 40% demonstrated significant histopathologic changes. Most colonoscopy examinations in patients with chronic unexplained diarrhea do not reveal endoscopic abnormalities, and routine random colon biopsy specimens are taken for further diagnostic assessment. In this study, we aimed to perform a systematic review and meta-analysis to study the pooled diagnostic value of random colon biopsy sampling in this patient population. Multiple databases (MEDLINE, Scopus, Embase) were searched in May 2022 for studies evaluating the diagnostic yield of random biopsy sampling in patients with chronic diarrhea with normal colonoscopy examinations. Standard meta-analysis methods used the random-effects model, and heterogeneity was assessed using the I2 statistics. Twenty-one studies were included in the study (3322 patients). The pooled rate of normal colon biopsy sampling was 48% (95% confidence interval [CI], 31-65.2; I2 = 98%), and normal ileal biopsy sampling was 88.5% (95% CI, 51.3-98.3; I2 = 93%). The pooled rate of significant histopathologic change was 30% (95% CI, 20-41; I2 = 96%). The pooled rates of specific diagnoses based on random colon biopsy sampling were as follows: microscopic colitis, 15% (95% CI, 10-21; I2 = 92%); lymphocytic colitis, 9% (95% CI, 6 -13; I2 = 85%); collagenous colitis, 3.5% (95% CI, 2.4-5.7; I2 = 80%); nonspecific colitis, 20% (95% CI, 11-32.8; I2 = 97%); inflammatory bowel disease, 1.7% (95% CI, .8-3.5; I2 = 54%); and eosinophilic colitis, 2.3% (95% CI, 1.1-4.5; I2 = 69%). This meta-analysis of 21 studies demonstrated significant histopathologic change in 30% and microscopic colitis in 15%. Normal colon biopsy sampling was noted in 48% and normal ileum biopsy sampling in 88.5%. Ileal biopsy data were based on limited data and studies (only 3 included). In studies with a mean patient age <49 years, 40% demonstrated significant histopathologic changes.

Primary sclerosing cholangitis with partial steroid responsiveness: a case report.

A 69-year-old woman suspected to have IgG4-related sclerosing cholangitis causing bile duct stenosis was transferred from another hospital after diarrhea, eosinophilia, and eosinophilic infiltration were detected and prednisolone was prescribed. Additional biliary imaging suggested primary sclerosing cholangitis, but the IgG4 level and inferior bile duct stenosis were alleviated by steroid therapy, suggesting IgG4-related sclerosing cholangitis. Therefore, prednisolone was continued. Bile duct biopsy findings suggesting adenocarcinoma led to a diagnosis of pancreatoduodenectomy. The latter specimen only displayed evidence of primary sclerosing cholangitis, and prednisolone was discontinued. Intractable cholangitis necessitated left hepatectomy, after which serum alkaline phosphatase levels increased and eosinophilic colitis recurred. The reintroduction of prednisolone effectively managed the diarrhea but only temporarily reversed the alkaline phosphatase elevation. When histologic sections from resection specimens were compared, the hepatectomy specimen exhibited greater eosinophil infiltration than the earlier pancreatoduodenectomy specimen, suggesting eosinophilic cholangiopathy superimposed on primary sclerosing cholangitis.