Skip to main content

Advertisement

SpringerLink
Log in

Retro-orbital granuloma associated with granulomatosis with polyangiitis: a series of nine cases

  • Original Article - Cases with a Message
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Retro-orbital granuloma is a rare and devastating component of granulomatosis with polyangiitis (GPA). Current medical treatment protocols are falling short, and outcomes are poor. The aim of the study was to investigate the frequency, clinical features, and treatment outcomes of retro-orbital granuloma in patients with GPA. This is a retrospective, multi-centre study, which involves GPA cohorts from five different clinics. Data were extracted from patient charts including history, physical examination, radiological–laboratory–histological findings, and treatment protocols. Major clinical outcome measures were changes in the volume of the granuloma on comparative MRI, and visual acuity on repeated ophthalmologic examinations. Among 141 GPA patients, nine (five females and four males) were diagnosed with a retro-orbital granuloma. Median duration of disease was 8 years. Proptosis and diplopia were the dominant presenting symptoms (77 %), followed by orbital pain (55 %). Three out of nine patients had isolated retro-orbital granulomas, without other organ involvement of GPA. Five patients received conventional pulse steroid and pulse cyclophosphamide (CYC) as the first-line remission induction therapy. Four of these patients had progressive disease, and a regression in granuloma size was observed in one patient using this regimen. Two patients were already receiving immunosuppressants when they were diagnosed with retro-orbital granuloma. Six patients had been treated with RTX as the second-line remission induction therapy. None of these patients had progression following RTX therapy. Three patients underwent orbital decompression surgery. The indication for the decision for surgery was either progressive loss of vision or intractable pain. Standard first-line chemotherapy (CYC and steroids) was ineffective against retro-orbital granuloma associated with GPA. RTX could be an alternative in these cases. Surgical intervention may help to decrease the morbidity. Further prospective studies with greater patient numbers are needed to test the clinical efficiency of RTX as a first-line remission induction chemotherapy.

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. Pagnoux C et al (2011) Wegener’s granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database. J Rheumatol 38(3):475–478

    Article  PubMed  Google Scholar 

  2. Stone JH, Wegener’s G (2003) Granulomatosis Etanercept Trial Research, Limited versus severe Wegener’s granulomatosis: baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum 48(8):2299–2309

    Article  PubMed  Google Scholar 

  3. Jayne DR, Rasmussen N (1997) Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group. Mayo Clin Proc 72(8):737–747

    Article  CAS  PubMed  Google Scholar 

  4. Holle JU et al (2010) Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis 69(11):1934–1939

    Article  PubMed  Google Scholar 

  5. Stone JH et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363(3):221–232

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  6. Leavitt RY et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33(8):1101–1107

    Article  CAS  PubMed  Google Scholar 

  7. Holle JU et al (2013) Orbital masses in granulomatosis with polyangiitis are associated with a refractory course and a high burden of local damage. Rheumatology (Oxford) 52(5):875–882

    Article  Google Scholar 

  8. Haynes BF et al (1977) The ocular manifestations of Wegener’s granulomatosis. Fifteen years experience and review of the literature. Am J Med 63(1):131–141

    Article  CAS  PubMed  Google Scholar 

  9. Talar-Williams C et al (2005) Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegener’s granulomatosis. Br J Ophthalmol 89(4):493–497

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  10. Rothschild PR et al (2013) Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature. Semin Arthritis Rheum 42(5):507–514

    Article  PubMed  Google Scholar 

  11. Mukhtyar C et al (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68(3):310–317

    Article  CAS  PubMed  Google Scholar 

  12. Specks U et al (2013) Efficacy of remission–induction regimens for ANCA-associated vasculitis. N Engl J Med 369(5):417–427

    Article  CAS  PubMed  Google Scholar 

  13. Joshi L et al (2011) Rituximab in refractory ophthalmic Wegener’s granulomatosis: pR3 titers may predict relapse, but repeat treatment can be effective. Ophthalmology 118(12):2498–2503

    Article  PubMed  Google Scholar 

  14. Woo TL et al (2001) Australasian orbital and adnexal Wegener’s granulomatosis. Ophthalmology 108(9):1535–1543

    Article  CAS  PubMed  Google Scholar 

  15. Bijlsma WR et al (2011) Orbital mass as manifestation of Wegener’s granulomatosis: an ophthalmologic diagnostic approach. Clin Exp Rheumatol 29(1 Suppl 64):S35–S39

    PubMed  Google Scholar 

  16. Fauci AS et al (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98(1):76–85

    Article  CAS  PubMed  Google Scholar 

  17. Baslund B et al (2012) Treatment of orbital inflammation with rituximab in Wegener’s granulomatosis. Clin Exp Rheumatol 30(1 Suppl 70):S7–S10

    PubMed  Google Scholar 

  18. Ooka S et al (2009) Treatment of refractory retrobulbar granuloma with rituximab in a patient with ANCA-negative Wegener’s granulomatosis: a case report. Mod Rheumatol 19(1):80–83

    Article  PubMed  Google Scholar 

  19. Holle JU et al (2012) Rituximab for refractory granulomatosis with polyangiitis (Wegener’s granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations. Ann Rheum Dis 71(3):327–333

    Article  CAS  PubMed  Google Scholar 

  20. Stone JH et al (2001) Etanercept combined with conventional treatment in Wegener’s granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum 44(5):1149–1154

    Article  CAS  PubMed  Google Scholar 

  21. Bartolucci P et al (2002) Efficacy of the anti-TNF-alpha antibody infliximab against refractory systemic vasculitides: an open pilot study on 10 patients. Rheumatology (Oxford) 41(10):1126–1132

    Article  CAS  Google Scholar 

  22. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group (2005) Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med 352(4):351–61

  23. Lamprecht P et al (2002) Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 41(11):1303–1307

    Article  CAS  Google Scholar 

  24. de Menthon M et al (2011) Infliximab or rituximab for refractory Wegener’s granulomatosis: long-term follow up. A prospective randomised multicentre study on 17 patients. Clin Exp Rheumatol 29(1 Suppl 64):S63–s71

    PubMed  Google Scholar 

  25. Wygoda A et al (2013) Low dose radiotherapy as an effective treatment in a patient with solitary Wegener’s granulomatosis resistant to systemic treatment—case report. Contemp Oncol (Pozn) 17(1):107–111

    Google Scholar 

  26. Eagleton LE et al (1979) Radiation therapy and mechanical dilation of endobronchial obstruction secondary to Wegener’s granulomatosis. Chest 76(5):609–610

    Article  CAS  PubMed  Google Scholar 

  27. Fechner FP, Faquin WC, Pilch BZ (2002) Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 112(11):1945–1950

    Article  PubMed  Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Gazi University School of Medicine, 06500, Besevler, Ankara, Turkey

    Berivan Bitik, Abdurrahman Tufan & Berna Goker

  2. Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey

    Levent Kılıç, Ömer Karadağ & A. Ihsan Ertenli

  3. Division of Rheumatology, Department of Internal Medicine, Ankara University School of Medicine, Ankara, Turkey

    Orhan Küçükşahin, Aşkın Ateş & Murat Turgay

  4. Division of Rheumatology, Department of Internal Medicine, Ankara Numune Education and Research Hospital, Ankara, Turkey

    Kubilay Şahin

  5. Division of Rheumatology, Department of Internal Medicine, Gulhane Military Medical Academy, Istanbul, Turkey

    Salih Pay & Sedat Yilmaz

  6. Department of Radiology, Gazi University School of Medicine, Ankara, Turkey

    Murat Ucar

  7. Department of Otolaryngology, Gazi University School of Medicine, Ankara, Turkey

    Hakan Tutar

  8. Division of Rheumatology, Department of Internal Medicine, Ankara Numune Education and Research Hospital, Hittite University School of Medicine, Ankara, Turkey

    Yaşar Karaaslan

  9. Department of Ophthalmology, Gazi University School of Medicine, Ankara, Turkey

    Onur Konuk

Authors
  1. Berivan Bitik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Levent Kılıç
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Orhan Küçükşahin
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Kubilay Şahin
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Abdurrahman Tufan
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Ömer Karadağ
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Salih Pay
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Aşkın Ateş
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Murat Ucar
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Hakan Tutar
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Yaşar Karaaslan
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Sedat Yilmaz
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. A. Ihsan Ertenli
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Onur Konuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Murat Turgay
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Berna Goker
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Berivan Bitik.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Bitik, B., Kılıç, L., Küçükşahin, O. et al. Retro-orbital granuloma associated with granulomatosis with polyangiitis: a series of nine cases. Rheumatol Int 35, 1083–1092 (2015). https://doi.org/10.1007/s00296-014-3179-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-014-3179-8

Keywords

Search

Navigation