Pharmacotherapy of IgG4-Related Disease

  • Mitsuhiro Kawano
  • Kazunori Yamada
  • Susumu Nishiyama
  • Shigeyuki Kawa


We outline the pharmacotherapy for IgG4-related disease (IgG4-RD), with a particular focus on type 1 autoimmune pancreatitis (AIP). The basis of therapy for type 1 AIP is oral prednisolone administration. Prednisolone at a dose of 30–40 mg/day is typically employed for remission induction. Assuming improvement, the dose is then tapered to a maintenance dose over a period of 2–4 months. Maintenance therapy, which is typically continued for 6–12 months, usually ranges from 5 to 10 mg/day. Patients must be followed carefully for the possibility of recurrence.

When oral glucocorticoid therapy is felt to provide adequate protection against conditions like lower bile duct stenosis, the combination of oral glucocorticoids and minipulse therapy may be employed when long-term oral glucocorticoid administration is difficult—for example, when surgery is planned or the patient refuses long-term oral glucocorticoid use. For glucocorticoid-refractory cases, a variety of steroid-sparing agents have been employed. However, all of these require further investigation.


Maintenance Therapy Obstructive Jaundice Glucocorticoid Therapy Retroperitoneal Fibrosis Oral Glucocorticoid 


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Copyright information

© Springer Japan 2014

Authors and Affiliations

  • Mitsuhiro Kawano
    • 1
  • Kazunori Yamada
    • 1
  • Susumu Nishiyama
    • 2
  • Shigeyuki Kawa
    • 3
  1. 1.Division of RheumatologyKanazawa University HospitalKanazawaJapan
  2. 2.Rheumatic Disease Center, Kurashiki Medical CenterKurashikiJapan
  3. 3.Center for Health, Safety, and Environmental ManagementShinshu UniversityMatsumoto CityJapan

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