Superior Oblique Myositis as an Early Feature of Orbital Wegener's Granulomatosis

Abstract

A 66-year-old female presented with a 6-month history of increasing right-sided facial pain and diplopia. Right ocular movements were restricted in adduction and downgaze. Superior oblique (SO) enlargement was seen on an initial MRI scan, and subsequent diffuse infiltration was seen on a repeat scan. An elevated cANCA and biopsy of SO confirmed Wegener's granulomatosis (WG). We report a case of SO myositis as a predominant early feature of WG granulomatosis and review the literature regarding extraocular muscle involvement in WG. To our knowledge, early SO involvement has not been reported.

Atypical Clinical Presentation of Isolated Extraocular Muscle Sarcoid

“…Extraocular involvement has been reported as a first presentation of Wegener's granulomatosis, but this can be excluded by a normal c-ANCA level and lack of vascular inflammation in tissue biopsy [13]. In addition, our patient had no sinus symptoms, which commonly precede orbital involvement in Wegener's granulomatosis.…”

Ocular manifestations of Wegener’s granulomatosis

“…Rituximab has been reported to be effective in treating various ocular manifestations of WG/GPA, such as PUK [121], relapsing necrotizing scleritis [43], optic nerve infiltration [76] and optic neuritis [122], although remission may take 7 months to occur [123]. RTX has also been found to be effective in inducing remission in refractory ophthalmic WG/GPA, and is capable of inducing extended remission compared with other biologics and conventional treatments [124].…”

“…Patients with proptosis and lid destruction are prone to develop exposure keratopathy, which can lead to corneal ulceration, ocular perforation and blindness [4]. Orbital myositis in WG/GPA may occur as a result of inflammation of the extraocular muscles or vasculitis of the vasa nervorum, and patients mainly present with symptoms of diplopia and pain on ocular movement and signs of restriction of ocular motility [76].…”

A patient with diplopia and upper eyelid edema: a case report

“…Od tega je šlo pri enem bolniku za potrjeno Wegenerjevo granulomatozo (Salam, 2008), pri dveh pa so bili podatki nepopolni, ker smo izgubili stik med spremljanjem (Sekhar 1993 in Muralidhar 2015) (1,2,4,11).…”