Mycophenolate mofetil for maintenance of remission in steroid-dependent autoimmune pancreatitis

Abstract

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.

Mycophenolate therapy in Autoimmune Pancreatitis

“…Small case series suggest steroid-sparing effects of several agents in AIP [2,6,7]. Though AZA and RTX remain commonly used, our data add to previous case reports that MMF also may be effective as a steroid-sparing agent in AIP [9][10][11][12]. MMF may be an alternative option for patients who cannot receive or are intolerant of RTX or AZA.…”

“…However data regarding efficacy and safety of MMF in AIP are lacking. As of 4-2016, less than 15 such cases have been reported in Englishlanguage literature [9][10][11][12]. AIP patients were identified by Rheumatology and Gastroenterology providers at the University of Minnesota (UMN), a tertiary referral center for patients with pancreatic diseases.…”

Immunoglobulin G4-Related Disease and the Lung

“…3,4,8,26,68 These agents have included azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide, 6-mercaptopurine, and tacrolimus. 2,3,8,9,26,68,76,77 The use of such agents should be considered in the initial treatment of IgG4-RD in patients at increased risk of serious adverse effects from corticosteroid administration. In addition, steroid-sparing immunosuppressive agents are used when adverse effects from corticosteroid therapy have occurred or the dose of corticosteroids cannot be reduced owing to persistent disease activity.…”

Traitement de la maladie associée aux IgG4 en 2014 : mise au point et revue de la littérature

“…Les résultats concernant l'efficacité des traitements immunosuppresseurs en deuxième ligne sont satisfaisants (Tableau 3) [3,31,34,39,40,[42][43][44][45][46]. Dans l'étude de Ghazale et al [3], l'azathioprine (utilisé aux posologies de 2 à 2,5 mg/kg/j) et le mycophénolate mofetil (750 mg × 2/j) permettent l'obtention d'une rémission chez tous les patients traités avec un suivi moyen de 6 mois.…”