J Rheum Dis.  2012 Oct;19(5):270-273. 10.4078/jrd.2012.19.5.270.

A Case of Wegener's Granulomatosis Presenting as a Diplopia

Affiliations
  • 1Department of Internal Medicine, College of Medicine, Chosun University, Gwangju, Korea. healthyra@chosun.ac.kr

Abstract

Wegener's granulomatosis (WG) is a multisystemic granulomatous inflammatory disorder which is presumably caused by an autoimmune response. It typically targets the upper and lower respiratory tract and the kidney. Ocular involvement occurs in 50 to 60% of WG patients. However, orbital myositis and diplopia as a presenting symptom is a rare ocular manifestation. We present the case of a 49-year-old man who was admitted with bilateral diplopia due to orbital myositis under a diagnosis of WG. He was successfully treated with high dose corticosteroid therapy.

Keyword

Wegener's granulomatosis; Diplopia; Myositis

MeSH Terms

Autoimmunity
Diplopia
Humans
Kidney
Middle Aged
Myositis
Orbital Myositis
Respiratory System
Wegener Granulomatosis

Figure

  • Figure 1 Previous X-ray examination shows wall thickening of both maxillary sinuses.

  • Figure 2 (A) Fat suppressed contrast enhanced T1WI shows illdefined enhancement and thickening of the sclera in right orbit (arrows). (B) Ill-defined enhancements are also seen in the right retrobulbar fat and lateral rectus muscle (arrowheads).


Reference

1. Duna GF, Galperin C, Hoffman GS. Wegener's granulomatosis. Rheum Dis Clin North Am. 1995. 21:949–986.
2. Harper SL, Letko E, Samson CM, Zafirakis P, Sangwan V, Nguyen Q, et al. Wegener's granulomatosis: the relationship between ocular and systemic disease. J Rheumatol. 2001. 28:1025–1032.
3. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. 1990. 33:1101–1107.
4. Ozaki S. ANCA-associated vasculitis: diagnostic and therapeutic strategy. Allergol Int. 2007. 56:87–96.
5. Lee SJ, Kim SS, Kim WS. A case of Wegener's Granulomatosis with retinal pigment epitherial detachment and subretinal hemorrhage. J Korean Ophthalmol Soc. 2005. 46:176–185.
6. Lim HJ, Park W, Park BH, Kim HJ, Kim SS, Lee YH, et al. A case of Wegener's Granulomatosis with transient myocardial ischemia and monocular blindness. J Korean Rheum Assoc. 2003. 10:89–95.
7. Choi JS, Choi KY. Five cases of Wegener's Granulomatosis with ocular manifestation. J Korean Ophthalmol Soc. 1986. 27:903–910.
8. Fechner FP, Faquin WC, Pilch BZ. Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope. 2002. 112:1945–1950.
9. Woo TL, Francis IC, Wilcsek GA, Coroneo MT, McNab AA, Sullivan TJ. Australasian Orbital and Adnexal Wagener's Study Group. Australasian orbital and adnexal Wegener's granulomatosis. Ophthalmology. 2001. 108:1535–1543.
10. Salam A, Meligonis G, Malhotra R. Superior oblique myositis as an early feature of orbital Wegener's granulomatosis. Orbit. 2008. 27:203–206.
11. Sadiq SA, Jennings CR, Jones NS, Downes RN. Wegener's granulomatosis: The ocular manifestations revisited. Orbit. 2000. 19:253–261.
12. Koldingsnes W, Nossent H. Predictors of survival and organ damage in Wegeners granulomatosis. Rheumatology (Oxford). 2002. 41:572–581.
13. WGET Research Group. Design of the Wegener's Granulomatosis Etanercept Trial (WGET). Control Clin Trials. 2002. 23:450–468.
14. Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission maintenance in the treatment of Wegener's granulomatosis. Arthritis Rheum. 2004. 51:278–283.
15. Pakrou N, Selva D, Leibovitch I. Wegener's granulomatosis: ophthalmic manifestations and management. Semin Arthritis Rheum. 2006. 35:284–292.
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