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Clinical Reviews in Allergy & Immunology

, Volume 42, Issue 2, pp 135–144 | Cite as

The Etiology of Paraneoplastic Autoimmunity

  • Emanual MaverakisEmail author
  • Heidi Goodarzi
  • Lisa N. Wehrli
  • Yoko Ono
  • Miki Shirakawa Garcia
Article

Abstract

Although they may sometimes appear similar, paraneoplastic autoimmunity has a unique pathogenesis, different from the classical autoimmune diseases not associated with cancer. When distinguished clinically, paraneoplastic autoimmunity is more severe and often presents with a broader range of clinical signs and symptoms. Management of these patients is difficult and is usually centered in part on treatment of the underlying malignancy. Self-antigens recognized in the setting of paraneoplastic autoimmunity can be diverse, and the number of determinants recognized within a single antigen can be numerous. This review uses prototypic examples of paraneoplastic immune-mediated diseases and their associated malignancies to describe the mechanisms by which immune dysregulation can occur in the setting of cancer. Specific diseases covered include paraneoplastic pemphigus, Sweet’s syndrome, pyoderma gangrenosum, thymoma-associated multiorgan autoimmunity, myasthenia gravis, autoimmune hemolytic anemia, immune thrombocytopenia, and the paraneoplastic neurological syndromes. The malignancies discussed include thymoma, non-Hodgkin’s lymphoma, and chronic lymphocytic leukemia, among others. The mechanisms by which cancers induce autoimmunity are broken down into the following categories: disruption of central tolerance, peripheral immune dysregulation, and alteration of self-antigens. For each category, examples of paraneoplastic autoimmune diseases and their associated malignancies are discussed. Finally, mechanisms by which cancer treatment can lead to autoimmunity and examples of polymorphisms that are linked to both cancer and autoimmunity are discussed.

Keywords

Paraneoplastic pemphigus Myasthenia gravis Autoimmunity Self Non-Hodgkin’s lymphoma CLL Pyoderma gangrenosum Sweet’s syndrome Thymoma Pemphigus vulgaris 

Notes

Acknowledgments

EM holds career awards from the Burroughs Wellcome Fund and the Howard Hughes Medical Institute.

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Copyright information

© Springer Science+Business Media, LLC (outside the USA)  2011

Authors and Affiliations

  • Emanual Maverakis
    • 1
    • 2
    Email author
  • Heidi Goodarzi
    • 1
  • Lisa N. Wehrli
    • 1
  • Yoko Ono
    • 1
  • Miki Shirakawa Garcia
    • 1
  1. 1.Department of DermatologyUniversity of California Davis, School of MedicineSacramentoUSA
  2. 2.Department of Veteran Affairs Northern California Health Care SystemSacramentoUSA

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