Diagnosis of limited ophthalmic wegener granulomatosis: distinctive pathologic features with ANCA test confirmation

Abstract

The described histologic characteristics are highly suggestive of WG. These findings along with clinical or laboratory findings, allow the diagnosis of very limited ophthalmic WG in the absence of systemic involvement.

Granulomatosis with polyangiitis (Wegener's disease): An updated review of ocular disease manifestations

“…The respiratory system is the most common organ to be involved in limited GPA, although any other organ system can be involved. A very limited form of the disease, with clinical involvement of a single organ such as the eye, has also been described with any ocular structure being affected (9). GPA is a complex and potentially lethal disease with high mortality rate if left untreated.…”

Cyclophosphamide or Rituximab Treatment of Scleritis and Uveitis for Patients with Granulomatosis with Polyangiitis

“…Widespread systemic involvement can lead to critical organ failure and ultimately death. In the limited form of the disease, which involves only one or two organs, the eye can be the only clinically significant presentation of GPA [3]. Severe, vision-threatening ocular manifestations can commonly include scleritis, peripheral ulcerative keratitis (PUK), uveitis, and orbital pseudotumor.…”

Scleritis in patients with granulomatosis with polyangiitis (Wegener)

“…Systemic vasculitides must be considered as a possible underlying aetiology in patients with scleritis, especially in those with severely painful and necrotising scleritis 2 6. Eye involvement is common in patients with GPA, and it may be the sole presenting sign or the feature allowing diagnosis 9. However, it is to be remembered that 30% of patients with GPA are anti-neutrophil cytoplasmic antibody (ANCA) negative until late in the course of the disease, emphasising the importance of the ophthalmologist as positioned remembering his or her clinical medicine diagnostic skills 10…”