Skip to main content

Advertisement

SpringerLink
Log in

Radiological features of IgG4-related disease in the head, neck, and brain

  • Head and Neck Radiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Introduction

Immunoglobulin (Ig) G4-related disease is a recently designated benign clinical entity histopathologically characterized by sclerosing inflammation and infiltration of numerous IgG4+ plasma cells that affects multiple organs. The purpose of this study is to characterize the imaging findings of patients with histopathologically proven IgG4-related disease in the head, neck, and brain.

Methods

A total of 17 patients (15 males, 2 females; mean age, 66.1 ± 7.4 years) with histopathologically proven IgG4-related disease in the head, neck, and brain were identified in two hospitals between January 2004 and December 2010. Imaging findings were retrospectively reviewed, with particular attention to the location and number of lesions, internal architecture, enhancement patterns, presence of vascular occlusion or compression, and changes in adjacent bones.

Results

The lesions, presented as either enlarged gland(s), or focal, localized nodules/masses, were distributed in the lacrimal gland (n = 7), the parotid gland (n = 14), the submandibular gland (n = 10), the pituitary gland (n = 2), skull base dura mater (n = 2), and the pterygopalatine fossa (n = 3). All lesions were well-defined and iso- to hypointense on T2-weighted magnetic resonance images and showed homogeneous enhancement. No lesion showed vascular occlusion or compression. Bones adjacent to the lesions showed remodeling (erosion or sclerosis) without signs of destruction (n = 6). Four patients had lesions involving multiple areas which extended along the trigeminal nerve, accompanied by expansion of neural foramina along their courses, with no signs of bone destruction.

Conclusion

Sites of predilection for IgG4-related disease in the head, neck, and brain include the lacrimal, salivary, and pituitary glands. Recognition of the typical radiological features of IgG4-related disease, such as well-defined lesion borders, T2 hypointensity, homogeneous and gradual enhancement pattern, absence of vascular occlusion or compression, and presence of bone remodeling without destruction, may be of help in the diagnosis of this benign clinical entity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Jones CC, Hamilton RG, Jordon RE (1988) Subclass distribution of human IgG autoantibodies in pemphigus. J Clin Immunol 8:43–49

    Article  PubMed  CAS  Google Scholar 

  2. Lorenzo JA (1991) The role of cytokines in the regulation of local bone resorption. Crit Rev Immunol 11:195–213

    PubMed  CAS  Google Scholar 

  3. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, Fukushima M, Nikaido T, Nakayama K, Usuda N, Kiyosawa K (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344:732–738

    Article  PubMed  CAS  Google Scholar 

  4. Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, Nakazawa K, Shimojo H, Kiyosawa K (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359:1403–1404

    Article  PubMed  Google Scholar 

  5. Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y (2004) IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol 28:1193–1203

    Article  PubMed  Google Scholar 

  6. Zen Y, Onodera M, Inoue D, Kitao A, Matsui O, Nohara T, Namiki M, Kasashima S, Kawashima A, Matsumoto Y, Katayanagi K, Murata T, Ishizawa S, Hosaka N, Kuriki K, Nakanuma Y (2009) Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 33:1833–1839

    Article  PubMed  Google Scholar 

  7. Zen Y, Inoue D, Kitao A, Onodera M, Abo H, Miyayama S, Gabata T, Matsui O, Nakanuma Y (2009) IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol 33:1886–1893

    Article  PubMed  Google Scholar 

  8. Cheuk W, Yuen HK, Chan JK (2007) Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related sclerosing disease? Am J Surg Pathol 31:643–645

    Article  PubMed  Google Scholar 

  9. Kitagawa S, Zen Y, Harada K, Sasaki M, Sato Y, Minato H, Watanabe K, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Tsuneyama K, Saito K, Haratake J, Takagawa K, Nakanuma Y (2005) Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner's tumor). Am J Surg Pathol 29:783–791

    Article  PubMed  Google Scholar 

  10. Wong S, Lam WY, Wong WK, Lee KC (2007) Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol 38:1720–1723

    Article  PubMed  Google Scholar 

  11. Ishida M, Hotta M, Kushima R, Shibayama M, Shimizu T, Okabe H (2009) Multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum. Pathol Int 59:670–675

    Article  PubMed  Google Scholar 

  12. Maruya S, Miura K, Tada Y, Masubuchi T, Nakamura N, Fushimi C, Sakashita T, Monma T, Kamata SE (2010) Inflammatory pseudotumor of the parapharyngeal space: a case report. Auris Nasus Larynx 37:397–400

    Article  PubMed  Google Scholar 

  13. Kamisawa T, Okamoto A (2006) Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol 41:613–625

    Article  PubMed  CAS  Google Scholar 

  14. Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, Okumura F, Nishikawa T, Kobayashi K, Ichiya T, Takatori H, Yamakita K, Kubota K, Hamano H, Okamura K, Hirano K, Ito T, Ko SB, Omata M (2009) Standard steroid treatment for autoimmune pancreatitis. Gut 58:1504–1507

    Article  PubMed  CAS  Google Scholar 

  15. Zen Y, Nakanuma Y (2010) IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol 34:1812–1819

    Article  PubMed  Google Scholar 

  16. Bodily KD, Takahashi N, Fletcher JG, Fidler JL, Hough DM, Kawashima A, Chari ST (2009) Autoimmune pancreatitis: pancreatic and extrapancreatic imaging findings. Am J Roentgenol 192:431–437

    Article  Google Scholar 

  17. Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242:791–801

    Article  PubMed  Google Scholar 

  18. Inoue D, Zen Y, Abo H, Gabata T, Demachi H, Kobayashi T, Yoshikawa J, Miyayama S, Yasui M, Nakanuma Y, Matsui O (2009) Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology 251:260–270

    Article  PubMed  Google Scholar 

  19. Takahira M, Kawano M, Zen Y, Minato H, Yamada K, Sugiyama K (2007) IgG4-related chronic sclerosing dacryoadenitis. Arch Ophthalmol 125:1575–1578

    Article  PubMed  Google Scholar 

  20. Lee FJ, Varikatt W, Kairaitis K, D'Souza F, Lin MW (2010) IgG4-related dacryoadenitis. Ophthalmology 117:398.e3–398.e4

    Article  Google Scholar 

  21. Abe T, Sato T, Tomaru Y, Sakata Y, Kokabu S, Hori N, Kobayashi A, Yoda T (2009) Immunoglobulin G4-related sclerosing sialadenitis: report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 108:544–550

    Article  PubMed  Google Scholar 

  22. Schaffer AJ, Jacobsen AW (1927) Mikulicz's syndrome: a report of ten cases. Am J Dis Child 34:327–346

    Google Scholar 

  23. Morgan WS, Castleman B (1953) A clinicopathologic study of Mikulicz's disease. Am J Pathol 29:471–503

    PubMed  CAS  Google Scholar 

  24. Yamamoto M, Takahashi H, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Shinomura Y, Imai K (2006) A new conceptualization for Mikulicz's disease as an IgG4-related plasmacytic disease. Mod Rheumatol 16:335–340

    Article  PubMed  CAS  Google Scholar 

  25. Yamamoto M, Harada S, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, Takahashi H, Imai K (2005) Clinical and pathological differences between Mikulicz's disease and Sjögren's syndrome. Rheumatology 44:227–234

    Article  PubMed  CAS  Google Scholar 

  26. Masaki Y, Dong L, Kurose N, Kitagawa K, Morikawa Y, Yamamoto M, Takahashi H, Shinomura Y, Imai K, Saeki T, Azumi A, Nakada S, Sugiyama E, Matsui S, Origuchi T, Nishiyama S, Nishimori I, Nojima T, Yamada K, Kawano M (2009) Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 68:1310–1315

    Article  PubMed  CAS  Google Scholar 

  27. Chan SK, Cheuk W, Chan KT, Chan JK (2009) IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol 33:1249–1252

    Article  PubMed  Google Scholar 

  28. Lui PC, Fan YS, Wong SS, Chan AN, Wong G, Chau TK, Tse GM, Cheng Y, Poon WS, Ng HK (2009) Inflammatory pseudotumors of the central nervous system. Hum Pathol 40:1611–1617

    Article  PubMed  CAS  Google Scholar 

  29. Katsura M, Morita A, Horiuchi H, Ohtomo K, Machida T (2010) IgG4-related inflammatory pseudotumor of the trigeminal nerve: another component of IgG4-related sclerosing disease? Am J Neuroradiol 32:E150–E152

    Article  PubMed  Google Scholar 

  30. Mehta M, Jakobiec F, Fay A (2009) Idiopathic fibroinflammatory disease of the face, eyelids, and periorbital membrane with immunoglobulin G4-positive plasma cells. Arch Pathol Lab Med 133:1251–1255

    PubMed  Google Scholar 

  31. Cheuk W, Tam FK, Chan AN, Luk IS, Yuen AP, Chan WK, Hung TC, Chan JK (2010) Idiopathic cervical fibrosis—a new member of IgG4-related sclerosing diseases: report of 4 cases, 1 complicated by composite lymphoma. Am J Surg Pathol 34:1678–1685

    PubMed  Google Scholar 

  32. Cheuk W, Yuen HK, Chu SY, Chiu EK, Lam LK, Chan JK (2008) Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol 32:671–681

    Article  PubMed  Google Scholar 

  33. Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, Nakai Y, Sasahira N, Tsujino T, Yoshida H, Kawabe T, Omata M (2007) Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut 56:1719–1724

    Article  PubMed  CAS  Google Scholar 

  34. Cheuk W, Yuen HK, Chan AC, Shih LY, Kuo TT, Ma MW, Lo YF, Chan WK, Chan JK (2008) Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 32:1159–1167

    Article  PubMed  Google Scholar 

  35. Takahashi N, Ghazale AH, Smyrk TC, Mandrekar JN, Chari ST (2009) Possible association between IgG4-associated systemic disease with or without autoimmune pancreatitis and non-Hodgkin lymphoma. Pancreas 38:523–526

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

We declare that we have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Radiology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Masaki Katsura, Harushi Mori, Akira Kunimatsu, Hiroki Sasaki & Kuni Ohtomo

  2. Department of Radiology, School of Medicine, Nihon University, 30-1 Oyaguchi-kamicho, Itabashi-ku, Tokyo, 173-8610, Japan

    Osamu Abe

  3. Department of Radiology, NTT Medical Center Tokyo, 5-9-22 Higashi-Gotanda, Shinagawa-ku, Tokyo, 141-8625, Japan

    Toru Machida

Authors
  1. Masaki Katsura
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Harushi Mori
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Akira Kunimatsu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Hiroki Sasaki
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Osamu Abe
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Toru Machida
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kuni Ohtomo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Masaki Katsura.

Additional information

This article was presented in part and received the Cum Laude Award at Radiological Society of North America (RSNA) 2010: the 96th Scientific Assembly and Annual Meeting, 28 November to 03 December 2010, Chicago.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Katsura, M., Mori, H., Kunimatsu, A. et al. Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology 54, 873–882 (2012). https://doi.org/10.1007/s00234-012-1012-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-012-1012-1

Keyword

Search

Navigation