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IgG4-Related Disease pp 45–50Cite as

Pharmacotherapy of IgG4-Related Disease

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Abstract

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.

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Author information

Authors and Affiliations

  1. Division of Rheumatology, Kanazawa University Hospital, Takara-machi, Kanazawa, Ishikawa, 920-8641, Japan

    Mitsuhiro Kawano & Kazunori Yamada

  2. Rheumatic Disease Center, Kurashiki Medical Center, Kurashiki, 710-8522, Japan

    Susumu Nishiyama

  3. Center for Health, Safety, and Environmental Management, Shinshu University, 3-1-1, Asahi, Matsumoto City, 390-8621, Japan

    Shigeyuki Kawa

Authors
  1. Mitsuhiro Kawano
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  2. Kazunori Yamada
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  3. Susumu Nishiyama
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  4. Shigeyuki Kawa
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Corresponding author

Correspondence to Mitsuhiro Kawano .

Editor information

Editors and Affiliations

  1. Department of Hematology and Immunology, Kanazawa Medical University, Ishikawa, Japan

    Hisanori Umehara

  2. The 3rd Department of Internal Medicine Div of Gastroenterology and Hepatology, Kansai Medical Univ Hirakata Hospital, Osaka, Osaka, Japan

    Kazuichi Okazaki

  3. Massachusetts General Hospital, Boston, Massachusetts, USA

    John H. Stone

  4. Shinshu University Center for Health Safety and Environment, Matsumoto, Nagano, Japan

    Shigeyuki Kawa

  5. Kanazawa University, Kanazawa, Ishikawa, Japan

    Mitsuhiro Kawano

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Kawano, M., Yamada, K., Nishiyama, S., Kawa, S. (2014). Pharmacotherapy of IgG4-Related Disease. In: Umehara, H., Okazaki, K., Stone, J., Kawa, S., Kawano, M. (eds) IgG4-Related Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54228-5_8

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  • DOI: https://doi.org/10.1007/978-4-431-54228-5_8

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54227-8

  • Online ISBN: 978-4-431-54228-5

  • eBook Packages: MedicineMedicine (R0)

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