Researchers found that subclinical microvascular retinal changes may be observed in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by using optical coherence tomography angiography (OCT-A) in the early detection of vascular damage.

These findings were published in the Orphanet Journal of Rare Diseases. 

Because microvascular damages are commonly associated with AAV, the authors of the study sought to assess the extent to which the eye may be involved. At present, routine screening of retinal abnormalities for patients with AAV are not indicated. 

One of the emerging tools for diagnosing subclinical retinopathy in systemic autoimmune diseases is OCT-A. In addition to assessing eye disease, it can also be used to examine the nailfold bed via videocapillaroscopy in a number of systemic diseases. The authors examined the potential use of OCT-A and nailfold videocapillaroscopy in further quantifying disease activity in AAV. 

Read more about AAV etiology

They recruited patients from September 1, 2020, to October 31, 2021, at a single center; patients must be between 18 and 75 years of age with a diagnosis of eosinophilic granulomatosis with polyangiitis  (EGPA), granulomatosis with polyangiitis (GPA), or microscopic polyangiitis (MPA). The inclusion criteria also required selected participants to have a best-corrected visual acuity of 0.5 logMAR or more and a spherical equivalent refractive error between −6.0 and +4.0 diopters. Patients with alternative systemic disorders potentially affecting the retina were excluded. All participants underwent ophthalmological and nailfold vessel examinations. 

The research team recruited 23 patients within their inclusion criteria. A total of 46 eyes were examined; the best-corrected visual acuity values of recruited patients were within the normal range for each eye and similar to healthy cohorts. However, retinal vessel density in superficial whole and superficial parafovea vascular plexi was found to be significantly decreased among the AAV patient cohort compared with healthy individuals. 

The researchers reported an inverse correlation between vasculitis damage index and superficial parafovea vascular plexi and deep parafovea density. In addition, a direct correlation was found between Birmingham Vasculitis Activity Score and vasculitis damage index. 

Nailfold investigations revealed no statistically significant differences in terms of findings between patients with AAV and healthy individuals. 

“Our results may represent the first hypothesis-generating basis for defining the role of OCT-A in non-symptomatic AAV patients to obtain an early diagnosis of retinal involvement, an accurate detection of disease-damage, and a more tailored treatment and management of such rare and complexes patients,” the authors concluded. 


Triggianese P, D’Antonio A, Nesi C, et al. Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damageOrphanet J Rare Dis. Published online July 10, 2023. doi:10.1186/s13023-023-02782-7