Skip to main content

Advertisement

SpringerLink
Log in

Consideration concerning similarities and differences between ANCA-associated vasculitis and IgG-4-related diseases: case series and review of literature

  • ORIGINAL ARTICLE
  • Published:
Immunologic Research Aims and scope Submit manuscript

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related diseases (IgG4-RD) are regarded as entirely different disease types with different etiological mechanisms. However, we experienced two cases that had clinical features of both AAV and IgG4-RD. The first case is an 81-year-old woman who showed periaortitis and retroperitoneal fibrosis and periarteritis with elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody and IgG4 levels. The second case is a 63-year-old woman who had dura mater, ear, nose, lung, and kidney involvement with serum negative for ANCA and elevated IgG4. Renal biopsy revealed tubulointerstitial nephritis involving IgG4+ plasma cells (IgG4+/IgG+ cell ratio of ≥ 40%). On the other hand, lung biopsy showed features of granulomatosis with polyangiitis (GPA). These two cases suggested that AAV and IgG4-RD might overlap. To investigate the similarities and differences between AAV and IgG4-RD, we retrospectively analyzed 13 cases of typical GPA, a subtype of AAV, and 13 cases of typical IgG4-RD at our hospital for comparison of clinical features and found some differences that can be useful in the differential diagnosis between the two diseases. Although AAV and IgG4-RD are distinguishable based on characteristic findings in many cases, the diagnosis can be unclear in rare cases, in which clinicians should consider possible coexistence of AAV and IgG4-RD when performing further workup. Here, we discuss the similarities and differences between AAV and IgG4-RD on the basis of our results and past literature.

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

Similar content being viewed by others

References

  1. Lin W, Lu S, Chen H, Wu Q, Fei Y, Li M, et al. Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford). 2015;54(11):1982–90. https://doi.org/10.1093/rheumatology/kev203.

    Article  CAS  Google Scholar 

  2. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51. https://doi.org/10.1056/NEJMra1104650.

    Article  CAS  PubMed  Google Scholar 

  3. Jennette JC, Falk RJ, Bacon PA, Cid MC, Ferrario F, Flores-Suarez LF, et al. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11. https://doi.org/10.1002/art.37715.

    Article  CAS  PubMed  Google Scholar 

  4. Umehara H, Okazaki K, Kawano M, Mimori T, Chiba T. How to diagnose IgG4-related disease. Ann Rheum Dis. 2017;76(11):e46. https://doi.org/10.1136/annrheumdis-2017-211330.

    Article  PubMed  Google Scholar 

  5. Su Y, Sun W, Wang C, Wu X, Miao Y, Xiong H, et al. Detection of serum IgG4 levels in patients with IgG4-related disease and other disorders. PLoS One. 2015;10(4):e0124233. https://doi.org/10.1371/journal.pone.0124233.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Yu KH, Chan TM, Tsai PH, Chen CH, Chang PY. Diagnostic performance of serum IgG4 levels in patients with IgG4-related disease. Medicine (Baltimore). 2015;94(41):e1707. https://doi.org/10.1097/MD.0000000000001707.

    Article  CAS  Google Scholar 

  7. Chang SY, Keogh KA, Lewis JE, Ryu JH, Cornell LD, Garrity JA, et al. IgG4-positive plasma cells in granulomatosis with polyangitis (Wegener’s): a clinicopathologic and immunohistochemical study on 43 granulomatosis with polyangitis and 20 control cases. Hum Pathol. 2013;44(11):2432–7. https://doi.org/10.1016/j.humpath.2013.05.023.

    Article  CAS  PubMed  Google Scholar 

  8. Della-Tolle E, Lanzillotta M, Campochiaro C, Bozzalla E, Bozzolo E, Bazzigaluppi E, et al. Antineutrophil cytoplasmic antibody positivity in IgG4-related disease: a case report and review of literature. Medicine (Baltimore). 2016;95(34):e4633. https://doi.org/10.1097/MD.0000000000004633.

    Article  Google Scholar 

  9. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21–30. https://doi.org/10.1007/s10165-011-0571-z.

    Article  CAS  PubMed  Google Scholar 

  10. ter Maaten JC, Franssen CF, Daenekindt AA, Hoorntje SJ. Triple Wegener’s granulomatosis in the urogenital tract. Nephron. 1993;63(3):358–9.

    Article  PubMed  Google Scholar 

  11. Kaipiainen-Seppänen O, Jantunen E, Kuusisto J, Marin S. Retroperitoneal fibrosis with antineutrophil cytoplasmic antibodies. J Rheumatol. 1996;23(4):779–81.

    PubMed  Google Scholar 

  12. Sakemi T, Tomiyoshi Y, Yano H, Ikeda Y, Matsuo Y, Kudo S. Retroperitoneal fibrosis with perinuclear antineutrophil cytoplasmic antibodies and a longitudinally extended periaortic soft-tissue structure on CT. Nephron. 1998;78(2):218–20.

    Article  CAS  PubMed  Google Scholar 

  13. Vaglio A, Manenti L, Allegri L, Ferrozzi F, Corradi D, Buzio C. ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis? J Intern Med. 2002;251(3):268–71.

    Article  CAS  PubMed  Google Scholar 

  14. van Bommel E, Brouwers A, Makkus A, van Vilet A. Retroperitoneal fibrosis and p-ANCA-associated polyarteritis nodosa: coincidental or common etiology? Eur J Intern Med. 2002;13(6):392–5.

    Article  PubMed  Google Scholar 

  15. de RS, Serratrice J, Granel B, Disdier P, Bartoli JM, Pache X, et al. Periaortitis heralding Wegener’s granulomatosis. J Rheumatol. 2002;29(2):392–4.

    PubMed  Google Scholar 

  16. Aslangul E, Ranque B, Pago T. Pseudotumoral retroperitoneal fibrosis and localized vasculitis with very high serum levels of anti-PR3 ANCA. Am J Med. 2003;115(3):250–2.

    Article  PubMed  Google Scholar 

  17. Carels T, Verbeken E, Blockmans D. P-ANCA-associated periaortitis with histological proof of Wegeners’s granulomatosis: case report. Clin Rheumatol. 2005;24(1):83–6.

    Article  CAS  PubMed  Google Scholar 

  18. Mavragani CP, Voulgarelis M. Retroperitoneal fibrosis and c-ANCA positivity. Clin Rheumatol. 2007;26(1):115–6.

    Article  PubMed  Google Scholar 

  19. Martínez-Odriozola P, Gutiérrez-Macías A, Moina Eguren I, Arrieta LJ. Association of idiopathic retroperitoneal fibrosis, rapidly progressive glomerulonephritis and antiproteinase 3 antineutrophil cytoplasmic antibodies (anti PR3-ANCA). Clin Nephrol. 2008;70(3):251–4.

    Article  PubMed  Google Scholar 

  20. Kasagi S, Saegusa J, Tsuji G, Sendo S, Miura N, Hayashi H, et al. Epidural spinal tumor and periaortitis as rare complications of Wegeners’ granulomatosis. Mod Rheumatol. 2011;21(6):678–83. https://doi.org/10.1007/s10165-011-0456-1.

    Article  PubMed  Google Scholar 

  21. Kotani S, Wakamatsu R, Itoh A, Miyamoto K, Yoshino M, Takami K, et al. Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) positive IgG4-related retroperitoneal fibrosis: utility of PET-CT with 18F-fluorodeoxy glucose (FDG). Intern Med. 2012;51(7):755–8.

    Article  CAS  PubMed  Google Scholar 

  22. Alexandraki KI, Kaltsatou M, Chatzellis E, Goules AV, Boutzios G, Kolomodi D, et al. Hypophysitis in IgG4-related disease associated with p-ANCA vasculitis. Am J Med. 2016;129(6):e25–7. https://doi.org/10.1016/j.amjmed.2015.

    Article  PubMed  Google Scholar 

  23. Tosovský M, Bradna P, Laco J, Podhola M, Soukup T, Brozík J. Case 1-2012: ANCA associated glomerulonephritis in combination with IgG4-positive mediastinal mass in a patient with ankylosing spondylitis treated with TNF alpha inhibitors. Acta Med (Hradec Kralove). 2012;55(1):42–6.

    Article  Google Scholar 

  24. Ohno K, Matsuda Y, Arai T, Sugihara T, Iga S, Kimura Y. Myeloperoxidase-Antineutrophil cytoplasmic antibody-positive otitis media and rhinosinusitis with pathological features of immunoglobulin G4-related disease: a case report. Ann Otol Rhinol Laryngol. 2016;125(6):516–21. https://doi.org/10.1177/0003489415625072.

    Article  PubMed  Google Scholar 

  25. Hanioka Y, Yamagami K, Yoshioka K, Nakamura T, Kishida M, Nakamura T, et al. Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease. Intern Med. 2012;51(17):2457–61.

    Article  PubMed  Google Scholar 

  26. Paulus YM, Cockerham KP, Cockerham GC, Gratzinger D. IgG4-positive sclerosing orbital inflammation involving the conjunctiva: a case report. Ocul Immunol Inflamm. 2012;20(5):375–7. https://doi.org/10.3109/09273948.2012.709574.

    Article  PubMed  Google Scholar 

  27. Brouwer E, Tervaert JW, Horst G, Huitema MG, van der Giessen M, Limburg PC, et al. Predominance of IgG1 and IgG4 subclasses of anti-neutrophil cytoplasmic autoantibodies (ANCA) in patients with Wegener’s granulomatosis and clinically related disorders. Clin Exp Immunol. 1991;83(3):379–86.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Holland M, Hewins P, Goodall M, Aud D, Jefferis R, Savage CO. Anti-neutrophil cytoplasm antibody IgG subclasses in Wegener’s granulomatosis: a possible pathogenic role for the IgG4 subclass. Clin Exp Immunol. 2004;138(1):183–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Liu LJ, Chen M, Yu F, Zhao MH, Wang HY. IgG subclass distribution, affinity of anti-myeloperoxidase antibodies in sera from patients with Wegener’s granulomatosis and microscopic polyangiitis. Nephrology. 2008;13(7):629–35. https://doi.org/10.1111/j.1440-1797.2008.00976.x.

    Article  CAS  PubMed  Google Scholar 

Download references

Funding

This research was funded entirely by the authors themselves, with no funding from outside sources.

Author information

Authors and Affiliations

  1. Department of Rheumatology and Allergy, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan

    Hirotoshi Kawashima, Atsunari Utsugi, Asuka Shibamiya, Kazuma Iida, Norihiro Mimura, Takao Yanagisawa & Masaki Hiraguri

  2. Department of Nephrology, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan

    Hirohumi Ohashi

  3. Department of Infectious Diseases, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan

    Ryota Hase

  4. Department of Pathology, Japanese Red Cross Society Narita Hospital, 90-1, Iida-cho, Narita-shi, Chiba, 286-8523, Japan

    Makio Kawakami

Authors
  1. Hirotoshi Kawashima
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Atsunari Utsugi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Asuka Shibamiya
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kazuma Iida
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Norihiro Mimura
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Hirohumi Ohashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ryota Hase
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Makio Kawakami
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Takao Yanagisawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Masaki Hiraguri
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hirotoshi Kawashima.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the ethical committee of Narita Red Cross Hospital and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Data obtained in this study did not interfere with course of treatment for patients included.

Human and animal rights

This article does not contain any studies with animals performed by any of the authors.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Kawashima, H., Utsugi, A., Shibamiya, A. et al. Consideration concerning similarities and differences between ANCA-associated vasculitis and IgG-4-related diseases: case series and review of literature. Immunol Res 67, 99–107 (2019). https://doi.org/10.1007/s12026-019-9070-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12026-019-9070-7

Keywords

Search

Navigation