Diana L. Franco

An update on cancer risk and surveillance in primary sclerosing cholangitis

Malignancy represents substantial morbidity and mortality in patients with primary sclerosing cholangitis (PSC). This subset of patients has been proven to be at increased risk for developing cholangiocarcinoma, gallbladder carcinoma and colorectal cancer in those with overlapping inflammatory bowel disease. Herein, we review the prevalence of these malignancies and recommend screening tools and current knowledge to reduce the disease burden in this population. Cholangiocarcinoma is the most dominant malignancy affecting PSC patients, with a lifetime risk ranging from 5% to 20%. We advocate for serial US or MRI/MRCP and CA 19‐9 to screen for cholangiocarcinoma. Gallbladder cancer has a lifetime risk around 2% in this population and we agree with annual imaging for lesions as recommended by national guidelines. Patients with PSC and concomitant IBD are at increased risk of colorectal carcinoma from time of diagnosis and therefore should likely undergo annual surveillance. The low rates of hepatocellular cancer and pancreatic cancer indicate surveillance for these malignancies is less advantageous.

Presentation, Diagnosis, and Management of Esophageal Lichen Planus: A Series of Six Cases

Lichen planus (LP) is an idiopathic disorder that presents with cutaneous and genital manifestations. Esophageal LP (ELP) was first described by Al-Shihabi and Jackson [J Laryngol Otol 1982;96:567-571] in 1982. Only approximately 80 cases have been documented in the literature since. It is a rare and underrecognized disorder, leading to a delay in diagnosis and a lack of standardized management. We describe the presentation, diagnosis, and management of 6 cases of ELP, at a tertiary institution, because we believe that an increasing awareness of this condition can help identify more cases and increase our understanding of this interesting condition.

Eosinophilic Granulomatosis with Polyangiitis and Diffuse Gastrointestinal Involvement

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic small- and medium-sized-vessel vasculitis, characterized by the presence of severe asthma as well as blood and tissue eosinophilia. Gastrointestinal (GI) symptoms, like diarrhea and abdominal pain, are common; however, there are few reports of histologic evidence of GI involvement. We report the case of a patient on treatment for EGPA who presented with recurrent small bowel obstruction and choledocholithiasis. Biopsies of the esophagus, small bowel and common bile duct showed diffuse eosinophilia, with clear EGPA in the GI tract. Improved awareness of GI EGPA may allow for timely management of this disorder.

Black Cohosh Hepatotoxicity with Autoimmune Hepatitis Presentation

Herbal medicines have been used for the treatment of various ailments since time immemorial. Black cohosh (BC) is well known for the treatment of postmenopausal symptoms, with conflicting evidence supporting its safety and benefits. We present a rare case of BC-induced autoimmune hepatitis (AIH) with hepatotoxicity in a 69-year-old female. To our knowledge, this represents the third case of BC-induced AIH.

Duodenal Aspirates for Small Intestine Bacterial Overgrowth: Yield, PPIs, and Outcomes after Treatment at a Tertiary Academic Medical Center

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.

Small cell lung cancer associated with multiple paraneoplastic syndromes

We report the case of a patient presenting with multiple severe electrolyte disturbances who was subsequently found to have small cell lung cancer. Upon further evaluation, she demonstrated three distinct paraneoplastic processes, including the syndrome of inappropriate antidiuretic hormone, Fanconi syndrome, and an inappropriate elevation in fibroblast growth factor-23 (FGF23). The patient underwent one round of chemotherapy, but she was found to have progressive disease. After 36 days of hospitalization, the patient made the decision to enter hospice care and later she expired.

28-Year-Old Man With Crohn Disease and Hematuria

Resident in Internal Medicine, Mayo School of Graduate Medical Education, Mayo Clinic, Scottsdale, AZ (D.L.F.); Advisor to resident and Consultant in Nephrology, Mayo Clinic, Scottsdale, AZ (M.T.K.). A 28-year-old man presented with a 3month history of mid abdominal pain. His medical history was notable for Crohn disease diagnosed in 1994, complicated by ileocolonic resection (20 cm of ileum and 15 cm of the right colon removed) in 2001. He had been taking mesalamine for 2 years, and although the prednisone dose was increased over the previous 3 months because of recurrent abdominal pain, it produced minimal relief. Computed tomography (CT) revealed fistulizing distal ileal Crohn disease with abscess formation. The patient was hospitalized; ertapenem and vancomycin were initiated, and 2 trials of CTguided abscess drainage were attempted but failed. Twelve days after admission, the patient underwent laparotomy for abscess drainage without any surgical complications. On postoperative day 1, his vital signs deteriorated, with a temperature of 39.3 C, respiratory rate of 30 breaths/min, heart rate of 108 beats/min, and blood pressure of 130/80 mm Hg. Pertinent findings on examination included mild respiratory distress and rapid heart rate but regular rhythm and no murmurs. His skin was warm to touch, and no rashes or edema were noted on examination. A right lower quadrant drain was in place with minimal blood-tinged drainage. Evaluation included chest CT with contrast medium, which ruled out pulmonary embolism, and blood cultures were obtained. Intravenous ketorolac, 30 mg every 6 hours, was initiated for fever and postoperative pain management. Blood culture results remained negative after 48 hours, and the vancomycin and ertapenem regimen was continued. On postoperative day 3, laboratory testing yielded the following notable findings (reference ranges provided parenthetically): hemoglobin, 7.1 g/dL (13.5-17.5 g/dL); white blood cells (WBCs), 14.7 10/L (3.5-10.5 10/L) with 13.8% neutrophils; sodium, 136 mmol/L (135-145 mmol/L); potassium, 3.9 mmol/L (3.6-5.2 mmol/L); serum urea nitrogen, 12 mg/dL