Kahn, Allon

IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

Duodenal aspirates are not commonly collected, but they can be easily used in detection of small intestinal bacterial overgrowth (SIBO). Proton pump inhibitor (PPI) use has been proposed to contribute to the development of SIBO. We aimed to determine the yield of SIBO-positive cultures detected in duodenal aspirates, the relationship between SIBO and PPI use, and the clinical outcomes of patients identified by this method. In a retrospective study, we analyzed electronic medical records from 1263 consecutive patients undergoing upper endoscopy at a tertiary medical center. Aspirates were collected thought out the third and fourth portions of the duodenum, and cultures were considered to be positive for SIBO if they produced more than 100,000 cfu/mL. Culture analysis of duodenal aspirates identified SIBO in one-third of patients. A significantly higher percentage of patients with SIBO use PPIs than patients without SIBO, indicating a possible association. Similar proportions of patients with SIBO improved whether or not they received antibiotic treatment, calling into question the use of this expensive therapy for this disorder.

Recipients of liver transplantation (LT) are at increased risk for symptomatic coccidioidomycosis, primarily because of chronic immunosuppression and impaired cellular immunity. Unfortunately, no consensus exists regarding optimal posttransplant prophylaxis. In a prior study at our institution, we observed both de novo and recurrent coccidioidomycosis despite targeted antifungal prophylaxis. In response, in February 2011, we instituted a universal prophylaxis program consisting of fluconazole (200 mg daily) for the first posttransplant year. In the current study, we retrospectively reviewed the medical records of all patients who underwent LT between the initiation of universal prophylaxis and July 11, 2013. Patients receiving a second transplant or dual‐organ transplant and those who died or did not have follow‐up in the 12‐month post‐LT period were excluded. Data from the universal prophylaxis cohort were compared with previously published data from the targeted prophylaxis era. Of the 160 patients undergoing LT during the study period, 143 met criteria for data analysis. When compared with the 349 patients in the targeted prophylaxis cohort, patients in the universal prophylaxis group were older and had higher rates of pre‐LT coccidioidomycosis, asymptomatic coccidioidal seropositivity, posttransplant diabetes mellitus, and renal insufficiency. Fluconazole‐related toxicity occurred in 13 of the universal prophylaxis patients, 7 of whom were required to discontinue use of the medication. Coccidioidomycosis developed in 10 of the 391 patients (2.6%) in the targeted prophylaxis cohort and in none of the patients in the universal prophylaxis group (P = 0.04). These data strongly support the use of a 1‐year antifungal prophylaxis regimen for LT recipients in endemic regions.Liver Transpl 21:353–361, 2015. © 2015 AASLD.