Clinical and Pathological Correlation in Concomitant Celiac Disease and Eosinophilic Esophagitis Suggests Separate Etiologies.

Introduction. Recently, an increased risk of celiac disease or eosinophilic esophagitis has been postulated among patients with either of these disorders, prompting some to suggest a common underlying mechanism, whereas others maintain that their co-existence is coincidental. Methods. We compared clinical and pathological features of 29 patients meeting criteria for both celiac disease and eosinophilic esophagitis to 26 celiac disease and 26 eosinophilic esophagitis controls to determine whether any distinguished study patients from controls. Results. Eight (28%) study patients presented with symptoms of both celiac disease and eosinophilic esophagitis, whereas 14 (48%) had celiac disease symptoms only and 5 had (17%) esophageal symptoms only. Study patients had similar autoimmune and atopic conditions seen in both control groups. Histological severity of disease, including Marsh II-III duodenal histology (study specimens: 87%; controls: 89%), mean peak esophageal eosinophil counts (study specimens: 55/400x field; controls: 80/400X field, P = .1), and presence of eosinophil microabscesses, scale crust, and subepithelial fibrosis were also similar to controls. Gluten-free diet resolved celiac disease-related symptoms (19 of 20, 95%) and histology (10 of 12, 83%), but not esophageal symptoms or eosinophilia in most study patients. Conclusion. Patients with concomitant celiac disease and eosinophilic esophagitis lack distinguishing features compared to controls with celiac disease or eosinophilic esophagitis alone. The occurrence of both disorders is likely coincidental in most cases.

Facing the Unknown: Idiopathic Giant Cell Hepatitis.

Giant cell hepatitis is a rare infiltrative disease associated with several viruses, drugs, malignancies, and autoimmune conditions. To date, treatment aims at controlling the underlying etiology, and there are limited data on the clinical course and treatment of idiopathic cases. We present a case of idiopathic giant cell hepatitis in an otherwise healthy adult man and review the literature regarding treatment and outcomes in this population.

Educational Case: Staphylococcus aureus Bacteremia: Utilization of Rapid Diagnostics for Bloodstream Pathogen Identification and Prediction of Antimicrobial Susceptibility.

The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040. 1.

Clinical significance of pathologic abnormalities in biopsy samples from the appendiceal orifice.

AIMS: Appendiceal orifice mucosa often appears inflamed endoscopically, even when other colonic segments appear normal. Histological findings in biopsy samples taken from endoscopically abnormal mucosa may simulate a variety of inflammatory colitides. We performed this study to evaluate the clinical implications of inflammatory changes isolated to the appendiceal orifice. METHODS AND RESULTS: In this double cohort study, biopsy samples from 26 histologically abnormal appendiceal orifices were reviewed. Twenty-five control cases were culled from endoscopically normal (n = 11) and abnormal (n = 14) appendiceal orifices that were histologically normal. Histological findings were correlated with presentation, medication history, findings at other colonic sites and clinical outcomes. Study cases displayed active inflammation (n = 12), chronic active inflammation (n = 13) or features simulating collagenous colitis (n = 1). Eighteen patients had biopsies taken from other colonic sites; these revealed benign polyps (n = 10) or displayed active (n = 4) or chronic active (n = 4) inflammation. All patients with findings isolated to the appendiceal orifice were asymptomatic at most recent clinical follow-up. Four of eight (50%) of the patients with inflammation in other biopsy samples were ultimately diagnosed with ulcerative colitis, in keeping with the well-established role of the appendix as a 'skip lesion' in that disorder. Control patients presented for screening colonoscopy (n = 19), iron deficiency anaemia (n = 3) or change in bowel habits (n = 3) and none reported gastrointestinal symptoms upon follow-up, regardless of the endoscopic appearance of the appendiceal orifice. CONCLUSION: Isolated inflammation of the appendiceal orifice mucosa should not be regarded as a feature of evolving inflammatory bowel disease or other types of chronic colitis.

Features That Aid Identification of Autoimmune Gastritis in a Background of Active Helicobacter pylori Infection.

CONTEXT.­: Helicobacter pylori-associated and autoimmune gastritis may coexist in a subset of patients who require treatment for both disorders. OBJECTIVE.­: To delineate findings that identify autoimmune gastritis in the background of H pylori infection. DESIGN.­: We examined cases of (1) patients with H pylori-associated gastritis who had successful eradication therapy and subsequent biopsies diagnostic of autoimmune gastritis and (2) H pylori-associated gastritis wherein pathologists noted features of autoimmune gastritis during original interpretation. Control patients underwent H pylori eradication but lacked evidence of autoimmune gastritis or H pylori infection after 10 years of follow-up. RESULTS.­: Eight subjects had H pylori-associated gastritis followed by H pylori-negative sampling that showed autoimmune gastritis. Review of original samples showed full-thickness inflammation of oxyntic mucosa in 8 of 8 and oxyntic gland loss in 7 of 8 cases. Enterochromaffin-like (ECL) cell hyperplasia, pyloric metaplasia, and intestinal metaplasia were present in 4 of 8 (80% of 5 tested cases), 4 of 8, and 3 of 8 cases, respectively. Features of autoimmune gastritis were noted at the time of their original H pylori diagnosis in 11 study subjects. Ten of 11 samples displayed full-thickness inflammation of oxyntic mucosa and/or partial loss of oxyntic glands, 8 of 11 had ECL cell hyperplasia (all tested cases), 6 of 11 showed pyloric metaplasia, and 4 of 11 harbored intestinal metaplasia. Except for full-thickness oxyntic mucosa inflammation, these features were absent in control cases. CONCLUSIONS.­: Full-thickness inflammation combined with oxyntic gland loss and ECL cell hyperplasia may help to identify autoimmune gastritis in patients with concomitant H pylori infection.

Application of the Milan System for Reporting Salivary Gland Cytopathology: Experience of an academic institution in a tertiary academic medical center.

BACKGROUND: The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC) represents a standardized reporting system for salivary gland lesions. The recent literature has demonstrated a wide range of data regarding range of malignancy (ROM) and interobserver variability. The objective of the current study was to evaluate the reproducibility and interobserver agreement of MSRSGC, and establish the ROM in a unique patient population residing within a designated Health Professional Shortage Area. METHODS: A total of 380 salivary gland fine-needle aspiration cases were obtained over a 3-year period. Corresponding cytology reports and slides were reviewed in a blinded fashion by a panel of cytopathologists and recategorized using MSRSGC. ROM was calculated by cytohistologic correlation in 176 cases. Agreement between review of reports and slides and interobserver reliability were determined using kappa statistics. RESULTS: The ROMs per MSRSGC category based on review of reports and slides were as follows: 4% and 0%, respectively, for nonneoplastic; 22% and 0%, respectively, for nondiagnostic; 42.9% and 48%, respectively, for atypia of undetermined significance; 1.6% and 1.9%, respectively, for benign-neoplastic; 17.9% and 15.6%, respectively, for salivary gland neoplasm of uncertain malignant potential; 81.8% and 71.4%, respectively, for suspicious for malignancy; and 100% and 90.5%, respectively, for malignant. There was a 59.2% overall agreement between review of reports and slides with regard to recategorizing salivary gland lesions (kappa, 0.51). The interobserver reliability demonstrated a 64.6% agreement (weighted kappa, 0.59). CONCLUSIONS: The ROMs at the study institution appeared comparable to those in the published literature. There was moderate overall agreement among cytopathologists and low interobserver agreement with regard to the indeterminate categories. Image-guided fine-needle aspiration specimens; rapid onsite adequacy; and integration of clinical, imaging, and ancillary studies can improve diagnostic accuracy among indeterminate lesions.

Features of endoscopic procedure site reaction associated with a recently approved submucosal lifting agent.

Endoscopic resection techniques, such as endoscopic mucosal resection (EMR) and endoscopic submucosal dissection (ESD), are frequently aided by injection of submucosal lifting solutions that create a plane for dissection and protect deeper mural layers. ORISE™ gel is a recently approved synthetic lifting solution that produces a localized inflammatory reaction associated with retained gel at the injection site. We describe a series of six cases of ORISE™-associated inflammatory lesions in patients who underwent endoscopic resections. Deposits comprised pale fibrillary or hyalinized eosinophilic material, depending on their age. All cases were associated with an inflammatory reaction composed of foreign-body giant cells and scattered eosinophils. ORISE™ gel extended laterally and deeply beyond residual tumors in all cases. Histochemically, the material proved to be negative for Congo Red, and mucicarmine, faint blue with Alcian blue, but positive for PAS and PAS-D. It stained blue with trichrome. Such deposits were absent in cases, wherein other widely-available lifting solutions were used. We compared ORISE™ deposits to histologically similar extracellular deposits, namely amyloid and pulse granulomata. Unlike ORISE™ material, amyloid deposits appear as waxy, more densely eosinophilic material, and stain positive with Congo Red. Amyloid demonstrated prominent intramucosal and perivascular distributions, features not seen in this series of ORISE™ deposits. Hyalinized pulse granulomata showed strong overlap with ORISE™ deposits, since they also comprise eosinophilic material associated with giant cell reaction. On the other hand, they form ribbons of glassy material in circumscribed lobules, unlike the ill-defined ORISE™ deposits. In summary, we describe the pathologic findings at injection sites in patients who underwent endoscopic procedures aided by the recently approved lifting agent, ORISE™. Pathologists should be aware of its appearance and associated reaction to avoid confusion with other common extracellular deposits seen in the gastrointestinal tract.