Granulomatosis/polyarteritis nodosa associated disorders

Granulomatosis with polyangiitis (GPA) and polyarteritis nodosa (PAN) are rare autoimmune vasculitides with distinct pathophysiological pathways and systemic involvement. They may affect almost every structure of the eye and they have both been reported as very scarce underlying causes of chorioretinal vascular disease. GPA and PAN may present with general manifestations of retinal vasculitis, such as perivascular sheathing and leakage or occlusion in fluorescein angiography (FA), but have been found to be associated with a wide range of nonspecific vascular signs. Occlusive arterial vasculitis is the most sight-threatening manifestation of GPA and PAN, thus both diseases should be part of the differential diagnosis in cases of noninfectious occlusive events. Although FA, indocyanine green angiography, and optical coherence tomography angiography can all prove useful in detecting chorioretinal vasculitis, ophthalmic involvement is neither necessary nor sufficient in establishing a GPA/PAN diagnosis. Further clinical, laboratory, imaging, and histological work-up should always be performed.