INTRODUCTION: Sarcoidosis is a systemic disease that is characterized by presence of non-caseating granulomas which can affect multiple organ systems which include lungs, eyes and skin. Involvement of the liver is common in sarcoidosis but typically patients are asymptomatic and as a result underdiagnosed. Abnormalities of liver enzymes is seen only in thirty five percent of patients. Only one percent of these patients will progress to end stage liver disease requiring orthotopic liver transplant (OLT). This is a unique case of recurrent sarcoidosis in a patient who was compliant with anti-rejection immunosuppressive medication and had a recurrent flare of sarcoidosis in the liver fou years after transplant. CASE DESCRIPTION/METHODS: We present a case of a 59-year-old African American lady with medical history remarkable for DM2, psoriasis, and systemic sarcoidosis s/p OLT 4 years ago for hepatic sarcoidosis presented to the emergency department for increasing abdominal pain. She is complaint with her immunosuppressive medications, which included prednisone, tacrolimus and mycophenolate. Work-up showed hypercalcemia, which prompted imaging. Imaging showed typical changes for systemic sarcoidosis consistent with retroperitoneal and pelvic adenopathy. A liver biopsy was prompted which showed both cirrhosis and granuloma formation consistent with sarcoidosis. She was discharged with a significant increase in immunosuppressive medications especially her prednisone after an appropriate response in her liver chemistries. She follows with hepatology clinic for long-term care of her liver sarcoidosis. DISCUSSION: This is a unique case of recurrent sarcoidosis in a patient who was compliant with medication including steroids and had a recurrent flare of sarcoidosis in the liver 4 years after OLT. According to our review of literature there has only been a handful of cases of hepatic sarcoidosis following OLT. Most of these reports involved the recurrence of sarcoidosis within 8 months and up to 5 years ,but most of them had early cessation of steroids. Recurrent hepatic sarcoidosis is a rare occurrence which likely under diagnosed. Future studies implementing protocol liver biopsies in this population could lead to the discovery of the true prevalence along with further insights into the prevention of recurrent disease long-term.

No abstract

INTRODUCTION: Ulcerative colitis (UC) is an inflammatory disease that predominantly involves the colon and rectum often requiring treatment with corticosteroids and immunomodulators. However chronic use of immunomodulators is usually associated with increased risk for lymphoma. Extranodal involvement of GI tract is typically seen however primary GI lymphoma is very rare. Here we present a unique case of B-cell lymphoma diagnosed from stomal biopsies. CASE DESCRIPTION/METHODS: A 76-year-old caucasian male with history of Ulcerative Colitis for almost 28 years, status post multiple abdominal surgeries including ventral hernia repair, ileostomy and colostomy presented with ongoing stoma complications for about 6 months. These complications included new fistula formation around the colostomy and ileostomy sites. Despite aggressive wound care, lesions did not regress, and continued to grow producing sharp, burning type of pain. There was ongoing purulent discharge from new fistula formation. Peri-ostomal biopsy from both ileostomy and colostomy was positive for B-cell lymphoma. On further history, patient endorsed fever, night sweats, and 30-lb weight loss over 5 months. PET scan showed increased uptake in both the stomas as well as liver. Chemotherapy options were discussed with Oncology, however patient opted for hospice as the prognosis was deemed poor. DISCUSSION: Primary GI lymphoma is common in stomach. Small intestine and colonic lymphoma account for 9% and 1% respectively. There have been multiple reports published citing association of lymphoma in IBD patients treated with immunomodulators. Our patient had two stomas (ileostomy and colostomy). Both periostomal biopsies were positive for B cell lymphoma. Periostomal lymphoma has been rarely reported. Parnesh et al (Case 1) reported a similar case of stomal lymphoma in a patient with history of Ulcerative Colitis. Levecq et al (Case 2) reported a stromal lymphoma in a patient with transfusion related AIDS who underwent total colectomy and ileostomy for indeterminate colitis. The lymphoma was diagnosed at 20 years (Case 1) and 2 years (Case 2) post stoma formation. Lymphoma should be in the differential of stoma being complicated by peristomal tissue proliferation, fistula and infection.

INTRODUCTION: Cystic fibrosis (CF) poses considerable morbidity and mortality. Largely identified in children with “typical” pulmonary problems, some present past adolescence and instead display “atypical” complaints. CF liver disease (CFLD) has a significant disease burden as a leading cause of death in CF. Symptom variation makes diagnosing CF in adults difficult. Our case report describes a diagnosis of atypical CF and CFLD in adulthood after nearly two decades of inconclusive findings. The case highlights hepatic sequelae of CF, disease diversity, and the impact of delayed diagnosis. CASE DESCRIPTION/METHODS: We present a 32-year-old African American female first noted to have elevated liver function tests (LFTs) on routine labs at age 16, yet was asymptomatic. Despite numerous clinical encounters over 17 years - including chemistry panels, imaging, and biopsies - the etiology remained unclear. In adulthood, she began experiencing chronic abdominal pain, and was later found to have critically low magnesium levels resulting in a seizure. The differential was expanded, and sweat chloride testing was positive, though genetic testing was negative. Given clinical history of high LFTs with abdominal pain and hypomagnesemia, appearance of symptoms past age 18, and unfixed results, she qualified for the diagnosis of atypical CF with CFLD. Her delayed diagnosis was further masked by unconventional misnomers not typically seen in CF. The patient was enlisted by our CF team for treatment and surveillance, and her LFTs improved. DISCUSSION: Our patient's clinical course made the diagnosis of CF less obvious, including: age, race, lack of family history, absence of pulmonary complications, presence of fertility, and vagueness of complaints. While sweat chloride testing was positive, genetic testing was negative. Retrospectively, each result lends itself to the diagnosis of atypical CF, which may show negative genetics despite sweat testing. The presence of a positive sweat test and clinical history is consistent with non-classic CF, and the chronically elevated LFTs and abdominal complaints were indicative of CFLD. Formal declaration of CFLD is important, as it leads to biliary cirrhosis and portal HTN, imposing high mortality. No gold standard modality exists for CFLD, further making diagnosis difficult in lieu of typical CF features. Our case emphasizes the importance of identifying both atypical CF and CFDL, how limited findings confound diagnosis, and the role of a multi-disciplinary team approach for CF patients.