Orbital Wegener’s granulomatosis: a case report and review of the literature

Abstract

Wegener's granulomatosis (WG) is a multisystem granulomatous, necrotizing vasculitis of presumed autoimmune origin that affects small- to medium-sized blood vessels. The respiratory tract and kidneys are typically involved (Gross and Reinhold-Keller, "Clinical features of primary ANCA-associated vasculitis" in Oxford textbook of rheumatology, third edition, 2004). The limited form usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease (Cassan et al., Am. J. Med. 49:366-379, 1970). Ocular involvement, which may be the initial manifestation, is often encountered and can result in significant morbidity and possibly blindness (Pakrou et al., Semin. Arthritis Rheum. 35:284-292, 2006). We report an unusual case of WG presenting as an orbital mass. The diagnostic triad of granulomatous inflammation with multinucleated giant cells, vasculitis, and necrosis was discovered on histopathology (McDonald and Edwards, JAMA 173:1205-1209, 1960).

Orbital apex syndrome secondary to granulomatosis with polyangiitis

“…The site of biopsy is a major variable in diagnostic studies, with the best results from paranasal sinus tissue 9. The classical histological triad of GPA featuring vasculitis, tissue necrosis and granulomatous inflammation is only present in 50% of biopsy samples and therefore clinical correlation is of importance 10 17 18. This case reiterates the importance of histological analysis in diagnosing the cause of OAS.…”

“…The sensitivity of c-ANCA is 91% and the specificity is 99%. Approximately 80–90% of patients with GPA are positive for ANCA, with 80% of the cohort being c-ANCA positive 10 15. Levels of c-ANCA can indicate disease activity with normal levels demonstrating remission and better prognosis 8 16…”

Bilateral dacryoadenitis as initial presentation of a locally aggressive and unresponsive limited form of orbital granulomatosis with polyangiitis

“…Moreover, the probability of a definitive diagnosis from biopsy of a tissue such as of the sinus or orbit is very low, with the diagnostic pathological triad of parenchymal necrosis, vasculitis and granulomatous inflammation being demonstrated in only 50% of patients, in part because medium-sized vessels are not typically biopsied. Mid-facial bone erosion and destruction of the nasal septum are independent of ANCA or systemic involvement, so they are good predictors of GPA in patients with orbital inflammatory symptoms and signs 10 11 13 19. Radiological findings are helpful in discriminating between GPA and other aetiologies of orbital inflammation.…”

“…MRI is more useful in identifying granulomas and delineating mucosal changes in sinuses, the nasal cavity and orbits. Orbital masses are slightly hyperdense relative to nasal mucosa on contrast-enhanced CT; they are hypointense relative to orbital fat in both T1 and T2 modalities on MRI, and enhance with iv gadolinium contrast 11 13 20 21. All of these clinical and imagiological characteristics are non-specific and may occur in other orbital inflammatory diseases.…”

Current Perspectives on Ophthalmic Manifestations of Childhood Rheumatic Diseases

“…Necrotizing scleritis portends a poor ocular and systemic prognosis [88]. Orbital involvement can include periorbital edema, paniculitis, myositis, dacryoadenitis, and dacryocystitis/canaliculitis and is highly associated with nasal sinus involvement [90]. Because orbital tissues are frequently involved, a tissue diagnosis of GPA may be achieved via an orbital biopsy.…”