Other Organs (Central Nervous System, Prostate)

  • Yasufumi Masaki
  • Nozomu Kurose
  • Hisao Tonami
  • Hisanori Umehara


IgG4-related disease (IgG4-RD) is a systemic lymphoproliferative disease characterized by elevated serum IgG4 values and increased numbers of IgG4-producing plasmacytic cells in tissues. Lesions may develop at sites throughout the body. The disease tends to target some organs frequently, e.g., the pancreas, lacrimal glands, salivary glands, kidneys, and retroperitoneum. Other organs, such as the central nervous system and prostate gland, are affected less frequently. In this chapter, we discuss the central nervous system lesions of hypertrophic pachymeningitis, hypophysitis, and intracerebral inflammatory pseudotumor, as well as the prostate lesions associated with IgG4-RD.


Giant Cell Arteritis Posterior Lobe Pituitary Stalk Central Nervous System Lesion Central Diabetes Insipidus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Chan SK, Cheuk W, Chan KT, Chan JKC. IgG4-related sclerosing pachymeningitis. A previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009;33:1249–52.PubMedCrossRefGoogle Scholar
  2. 2.
    Riku S, Hashizume Y, Yoshida M, Riku Y. Is hypertrophic pachymeningitis a Dural lesion of IgG4-related systemic disease? Rinshyo Shinkeigaku. 2009;49:594–6.CrossRefGoogle Scholar
  3. 3.
    Kosakai A, Yamada S, Ideta S, et al. A case of definite IgG4-related pachymeningitis. Neurology. 2010;75:1390–2.PubMedCrossRefGoogle Scholar
  4. 4.
    Choi SH, Lee SH, Khang SK, Jeon SR. IgG4-related sclerosing pachymeningitis causing spinal cord compression. Neurology. 2010;75:1388–90.PubMedCrossRefGoogle Scholar
  5. 5.
    Lindstrom KM, Cousar JB, Lopes MBS. IgG4-related meningeal disease: clinic-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010;120:765–76.PubMedCrossRefGoogle Scholar
  6. 6.
    Kim EH, Kim SH, Cho JM, et al. Immunoglobulin G4-related hypertrophic pachymeningitis involving cerebral parenchyma. J Neurosurg. 2011;115:1242–7.PubMedCrossRefGoogle Scholar
  7. 7.
    Norikane T, Yamamoto Y, Okada M, et al. Hypertrophic cranial pachymeningitis with IgG4-positive plasma cells detected by C-11 methionine PET. Clin Nucl Med. 2012;37:108–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Yamashita H, Takahashi Y, Ishiura H, et al. Hypertrophic pachymeningitis and tracheobronchial stenosis in IgG4-related disease: case presentation and literature review. Intern Med. 2012;51(8):935–41.PubMedCrossRefGoogle Scholar
  9. 9.
    Yamamoto M, Takahasi H, Ohara M, et al. A case of Mikulicz's disease (IgG4-related plasmacytic disease) complicated by autoimmune hypophysitis. Scand J Rheumatol. 2006;35:410–1.PubMedCrossRefGoogle Scholar
  10. 10.
    Tanabe T, Tsushima K, Yasuo M, et al. IgG4-associated multifocal systemic fibrosis complicating sclerosing sialadenitis, hypophysitis, and retroperitoneal fibrosis, but lacking pancreatic involvement. Intern Med. 2006;45:1243–7.PubMedCrossRefGoogle Scholar
  11. 11.
    Wong S, Lam WY, Wong WK, Lee KC. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol. 2007;38:1720–3.PubMedCrossRefGoogle Scholar
  12. 12.
    Shimatsu A, Oki Y, Fujisawa I, Sano T. Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity. Endocr J. 2009;56:1033–41.PubMedCrossRefGoogle Scholar
  13. 13.
    Hori M, Makita N, Andoh T, et al. Long-term clinical course of IgG4-related systemic disease accompanied by hypophysitis. Endocr J. 2010;57:485–92.PubMedCrossRefGoogle Scholar
  14. 14.
    Nishioka H, Shibuya M, Haraoka J. Immunohistochemical study for IgG4-positive plasmacytes in pituitary inflammatory lesions. Endocr Pathol. 2010;21:236–41.PubMedCrossRefGoogle Scholar
  15. 15.
    Leporati P, Landek-Salgado MA, Lupi I, et al. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum. J Clin Endocrinol Metab. 2011;96:1971–80.PubMedCrossRefGoogle Scholar
  16. 16.
    Landek-Salgado MA, Leporati P, Lupi I, et al. Growth hormone and proopiomelanocortin are targeted by autoantibodies in a patient with biopsy-proven IgG4-related hypophysitis. Pituitary. 2011 Sep;15(3):412–9.CrossRefGoogle Scholar
  17. 17.
    Moss HE, Mejico LJ, de la Roza G, et al. IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil. J Neurol Sci. 2012 Jul 15;318(1–2):31–5.PubMedCrossRefGoogle Scholar
  18. 18.
    Yoshimura Y, Takeda S, Ieki Y, et al. IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med. 2006;45:897–901.PubMedCrossRefGoogle Scholar
  19. 19.
    Nishimori I, Kohsaki T, Onishi S, et al. IgG4-related autoimmune prostatitis: two cases with or without autoimmune pancreatitis. Intern Med. 2007;46:1983–9.PubMedCrossRefGoogle Scholar
  20. 20.
    Uehara T, Hamano H, Kawakami M, et al. Autoimmune pancreatitis-associated prostatitis: distinct clinicopathological entity. Pathol Int. 2008;58:118–25.PubMedCrossRefGoogle Scholar
  21. 21.
    Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol. 2012 Feb;22(1):1–14.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Japan 2014

Authors and Affiliations

  • Yasufumi Masaki
    • 1
  • Nozomu Kurose
    • 2
  • Hisao Tonami
    • 3
  • Hisanori Umehara
    • 1
  1. 1.Department of Hematology and ImmunologyKanazawa Medical UniversityUchinada, KahokuJapan
  2. 2.Department of Pathology and Laboratory MedicineKanazawa Medical UniversityUchinada, KahokuJapan
  3. 3.Department of RadiologyKanazawa Medical UniversityUchinada, KahokuJapan

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