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Immunoglobulin G4 (IgG4) Related Disease

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Challenging Cases in Rheumatology and Diseases of the Immune System

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Abstract

IgG4-related disease (IgG4-RD) is a recently recognized syndrome characterized by frequent elevation of serum Ig4 levels and typical pathology of the affected organs. Biopsy shows lymphoplasmacytic inflammation, frequent eosinophils, storiform fibrosis, obliterative phlebitis and abundant IgG4-positive staining plasma cells. Disease manifestations often respond dramatically to steroid therapy.

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References

  1. Umehara H, Okazaki K, Masaki Y, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol. 2012;22(1):1–14.

    Article  PubMed  CAS  Google Scholar 

  2. Masaki Y, Kurose N, Umehara H. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century. J Clin Exp Hematop. 2011;51(1):13–20.

    Article  PubMed  Google Scholar 

  3. Masaki Y, Dong L, Kurose N, et al. Proposal for a new clinical entity, IgG4-positive ­multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis. 2009;68(8):1310–5.

    Article  PubMed  CAS  Google Scholar 

  4. Stone JH, Khosroshahi A, Deshpande V, Stone JR. IgG4-related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases. Arthritis Care Res (Hoboken). 2010;62(3):316–22.

    Article  Google Scholar 

  5. Aalberse RC, Stapel SO, Schuurman J, Rispens T. Immunoglobulin G4: an odd antibody. Clin Exp Allergy. 2009;39(4):469–77.

    Article  PubMed  CAS  Google Scholar 

  6. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30.

    Article  PubMed  CAS  Google Scholar 

  7. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92. doi: 10.1038/modpathol.2012.72.

    Google Scholar 

  8. Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504–7.

    Article  PubMed  CAS  Google Scholar 

  9. Kamisawa T, Okazaki K, Kawa S, et al. Japanese consensus guidelines for management of autoimmune pancreatitis: III. Treatment and prognosis of AIP. J Gastroenterol. 2010;45(5):471–7.

    Article  PubMed  CAS  Google Scholar 

  10. Pannala R, Chari ST. Corticosteroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1438–9.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

We gratefully acknowledge assistance from Dr. James E. Huprich, Dr. Thomas C. Smyrk and Dr. Suresh T. Chari. VRC is funded by National Institute of Arthritis and Musculoskeletal Diseases—K23 AR057815-01A1, Ronald F. Kinney Executive Dean for Research Career Development Award, Mayo Foundation.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Krati Chauhan M.D. & Vaidehi R. Chowdhary M.D.

  2. Division of Gastroenterology, Department of Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA

    Phil A. Hart M.D.

Authors
  1. Krati Chauhan M.D.
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  2. Phil A. Hart M.D.
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  3. Vaidehi R. Chowdhary M.D.
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Corresponding author

Correspondence to Krati Chauhan M.D. .

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Editors and Affiliations

  1. Department of Medicine, University of California, San Francisco, 812 Pollard Rd. Suite #5, Los Gatos, 95032, California, USA

    Massoud Mahmoudi

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Chauhan, K., Hart, P.A., Chowdhary, V.R. (2013). Immunoglobulin G4 (IgG4) Related Disease. In: Mahmoudi, M. (eds) Challenging Cases in Rheumatology and Diseases of the Immune System. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5088-7_5

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  • DOI: https://doi.org/10.1007/978-1-4614-5088-7_5

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-5087-0

  • Online ISBN: 978-1-4614-5088-7

  • eBook Packages: MedicineMedicine (R0)

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