Orbital Manifestations of Granulomatosis with Polyangiitis

1 Symptoms and Signs

GPA can present with a “limited” form of disease affecting largely the upper airways, often with orbital involvement, or more widespread disease affecting the lower airways and kidneys. Sclerokeratitis and uveitis may also occur. Chronic nasal and sinus inflammation with tissue loss and middle ear disease are common presentations. Orbital disease often occurs adjacent to involved sinuses, although dacryoadenitis may also occur [2]. Orbital inflammation is often sclerosing. Involvement of the lacrimal drainage apparatus is common [3].

2 Differential Diagnosis

Infiltrative orbital lesions such as idiopathic and specific inflammatory processes, as well as sclerosing orbital inflammation and dacryoadenitis.

3 Diagnosis

ANCA is often negative with orbital disease and “limited” disease, but a positive ANCA is very helpful. Biopsy is essential but the full-blown features of the disease with vasculitis (usually medium and small vessel arteritis) and necrotizing...