Unilateral ocular proptosis as the initial manifestation of Wegener’s granulomatosis

Authors

  • Dália Estevão Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.
  • Ana Sofia Teixeira Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.
  • Emília Louro Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.
  • Jorge Crespo Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.
  • Rui Proença Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.
  • Armando Carvalho Serviço de Medicina Interna, Centro Hospitalar e Universitário de Coimbra, E.P.E.

DOI:

https://doi.org/10.24950/rspmi.1019

Keywords:

Wegener’s granulomatosis, ocular proptosis, orbital mass

Abstract

Wegener’s granulomatosis (WG), a rare systemic vasculitis of
small and medium caliber vessels, mainly affects the upper and
lower respiratory tract, the ear and the kidney. The occurrence
of orbital and upper airways masses and nasal deformities
caused by destruction of cartilage and bone are suggestive of
WG. Approximately 90% of patients are ANCA positive and the
diagnosis is confirmed through lesions biopsy.
A 77-year-old woman presented left eye proptosis and decreased visual acuity evolving for one month. The MRI revealed
a mass filling the left paranasal sinuses that extended into the
ipsilateral orbital cavity, causing the ocular proptosis. The biopsy revealed a granulomatous inflammatory process that, associated with a positive ANCA, suggested a diagnosis of WG.
Particular emphasis is placed on the unusual form of presentation of WG.

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References

Falk RJ, Gross WL, Guillevin L et al. American College of Rheumatology, American Society of Nephrology, European League Against Rheumatism. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011;63(4):863.

Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117(1):39.

Jayne D. The diagnosis of vasculitis. Best Practice & Research Clinical Immunology 2009;23:445.

Gómez-Puerta JA, Hernández-Rodríguez J, López-Soto A, Bosch X. Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory disease. Chest. 2009;136(4):1101-1111.

Hoffman GS, Kerr GS, Leavitt RY et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488-498.

Taylor SR, Salama AD, Joshi L, Pusey CD, Lightman SL. Rituximab is effective in the treatment of refractory ophthalmic Wegener’s granulomatosis. Arthritis Rheum. 2009 May;60(5):1540-1547.

Sadiq SA, Jenning CR, Jones NS, Downes RN. Wegner’s granulomatosis: the ocular manifestations revisited. Orbit 2000;19:253-261.

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

Pagnoux C, Mahr A, Hamidou MA et al. Vasculitis Study Group. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359(26):2790-2803.

Gordon LK. Orbital inflammatory disease: a diagnostic and therapeutic challenge. Eye (Lond). 2006 Oct;20(10):1196-1206.

Guntinas-Lichius O, Kreppel MP, Stuetzer H, Semrau R, Eckel HE, Mueller RP. Single modality and multimodality treatment of nasal and paranasal sinuses cancer: a single institution experience of 229 patients. Eur J Surg Oncol. 2007;33(2):222-228.

Rao JK, Weinberger M, Oddone EZ, Allen NB, Landsman P, Feussner JR. The role of anti-neutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis. A literature review and meta-analysis. Ann Intern Med 1995;123:925-932

Additional Files

Published

2014-12-31

How to Cite

1.
Estevão D, Teixeira AS, Louro E, Crespo J, Proença R, Carvalho A. Unilateral ocular proptosis as the initial manifestation of Wegener’s granulomatosis. RPMI [Internet]. 2014 Dec. 31 [cited 2024 Dec. 18];21(4):160-3. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/1019

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