Background/Purpose: Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s Granulomatosis, is a systemic autoimmune condition caused by granulomatous inflammation of small and medium arteries, arterioles and venules (1). While GPA most commonly affects the upper respiratory tract, lungs and kidneys; ophthalmologic symptoms including orbital and ocular manifestations contribute to morbidity in GPA patients. Ophthalmic manifestations of GPA occur in up to 58% (2) of patients involving the optic nerve, eyelid and orbital structures (3). Diagnoses recorded in current literature include scleritis, episcleritis, keratitis, proptosis, sclerochoroidal granulomas (4), ptosis, uveitis, sclerouveitis, and retinal vein occlusion (5). We aim to systematically analyze published cases of orbital and ocular GPA to characterize symptoms, complications, and outcomes. This may increase provider awareness to prevent misdiagnoses and complications, and lessen patient disease burden.

Methods: On March 21^st, 2021, a literary search was conducted from the following electronic databases: PubMed, Web of Science, and Scopus using the MeSH terms “(Orbital OR Ocular) AND (Wegener’s OR Wegener OR polyangiitis OR granulomatosis) following PRISMA guidelines (#CRD42021244745). The search included case reports and series discussing patients diagnosed with GPA confirmed ocular or orbital symptoms. We restricted our search to articles published or translated articles in the English language. All articles were included regardless of patient age, gender, sex, ethnicity, or country of origin.

Results: The literature search returned 2,579 articles with 372 meeting inclusion criteria representing 158 (54.1%) female and 134 (45.9%) male patients. The mean age of ocular or orbital symptom presentation and GPA diagnosis were 47.99 and 45.81 years of age, respectively. Fifty percent were not previously diagnosed with GPA prior to presenting with ocular or orbital symptoms. The most common presenting symptom was vision impairment (54.5%) followed by orbital mass (43.8%), proptosis (40.4%), eye pain (39.7%), and extraocular muscle limitation (33.9%). Histopathological examination of orbital/ocular biopsy identified granulomas in 48.5% of patients. Proteinase 3 anti-neutrophil cytoplasmic antibody (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) positivity was found in 60.4% and 9.9% of patients, respectively. Systemic manifestations of GPA included pulmonary (34.6%), genitourinary (21.9%) and central nervous system (6.5%) among patients. Overall, 18.9% patients relapsed, 11.9% suffered blindness and 3.4% were treated with enucleation. Death was reported in 3.8% of cases. Patient outcomes encompassed full resolution (30.1%), partial resolution (47.3%), and unknown (21.6%).

Conclusion: Our review found that half of patients without prior GPA diagnosis presented with ocular and orbital symptoms. Clinical awareness and early diagnosis of ophthalmological GPA may prevent disease-related complications and improve patient outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ocular-and-orbital-manifestations-of-granulomatosis-with-polyangiitis-a-systematic-review-of-published-cases/