mycophenolic-acid and Cholangitis

Fascioliasis is caused by the trematode liver fluke Fasciola hepatica. Humans are accidental hosts getting infected after ingesting contaminated plants or water. 90 million people in 75 nations are at risk of infection with F hepatica. Immunosuppressed patients are higher risk of acquiring infection and may present with atypical manifestations. Patients can present with hepatic involvement, biliary features or a combination of both. Confirmation of the diagnosis is by demonstration of live parasites or eggs in bile or feces, serology (immunoelectrophoresis, indirect immunofluorescence, indirect hemagglutination), ELISA, typical imaging findings or a combination of any of the above. The drug of choice for treatment is triclabendazole. Fascioliasis should always be considered as a possibility in post-LT patients with findings of hepatobiliary disorder from endemic areas. Unfamiliarity with this infection in non-endemic areas often eludes prompt diagnosis thereby increasing the morbidity. We report the first case of fascioliasis in a pediatric liver transplant recipient leading to graft loss and mortality.

Topics: Animals; Child; Cholangitis; Contrast Media; End Stage Liver Disease; Fasciola hepatica; Fascioliasis; Female; Graft Rejection; Humans; Immunosuppression Therapy; Immunosuppressive Agents; India; Liver Transplantation; Morocco; Mycophenolic Acid; Stem Cell Transplantation; Tacrolimus; Tomography, X-Ray Computed

Topics: Adult; Cholangitis; Drug Interactions; Female; Humans; Hysterectomy; Immunosuppressive Agents; Leiomyomatosis; Liver Transplantation; Mycophenolic Acid; Norpregnadienes; Tacrolimus; Treatment Outcome; Uterine Neoplasms

Systemic corticosteroids represent the standard treatment for autoimmune pancreatitis with IgG4-associated cholangitis. For steroid-dependent disease, azathioprine has been used for maintenance of remission. Mycophenolate mofetil has been used for transplant immunosuppression and more recently for autoimmune hepatitis; however, there are no case reports to date on the use of mycophenolate mofetil in adult patients with autoimmune pancreatitis. A patient with IgG4-mediated autoimmune pancreatitis and IgG4-associated cholangitis refractory to steroids and intolerant of azathioprine was treated with mycophenolate mofetil, which inhibits de novo guanosine synthesis and blockade of both B and T lymphocyte production. Introduction of mycophenolate mofetil and uptitration to 1000 mg by mouth twice daily over a treatment period of 4 mo was associated with improvement in the patient's energy level and blood glucose control and was not associated with any adverse events. The patient was managed without a biliary stent. However, there was a return of symptoms, jaundice, increase in transaminases, and hyperbilirubinemia when the prednisone dose reached 11 mg per day. In the first report of mycophenolate mofetil use in an adult patient with IgG4-associated autoimmune pancreatitis and IgG4-associated cholangitis, the introduction of mycophenolate mofetil was safe and well-tolerated without adverse events, but it did not enable discontinuation of the steroids. Mycophenolate mofetil and other immunomodulatory therapies should continue to be studied for maintenance of remission in the large subset of patients with refractory or recurrent autoimmune pancreatitis.

Topics: Adrenal Cortex Hormones; Aged; Autoimmune Diseases; Biopsy; Cholangiopancreatography, Endoscopic Retrograde; Cholangitis; Drug Therapy, Combination; Humans; Immunoglobulin G; Immunosuppressive Agents; Male; Mycophenolic Acid; Pancreatitis; Recurrence; Treatment Outcome