Journal
ORPHANET JOURNAL OF RARE DISEASES
Volume 16, Issue 1, Pages -Publisher
BMC
DOI: 10.1186/s13023-020-01649-5
Keywords
Optical coherence tomography angiography; Systemic sclerosis; Retina; Choroid; Biomarker
Funding
- Projekt DEAL
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The study aimed to investigate the correlation between retinal and choroidal microperfusion in systemic sclerosis (SSc) patients using optical coherence tomography angiography (OCTA). Results showed impaired perfusion in both tissues, supporting the hypothesis of widespread vascular injury in SSc patients. In early stages, either the retinal or the choroidal perfusion may be involved, while later on, vascular impairment affects both tissues alike.
Purpose To investigate the correlation between retinal and choroidal microperfusion in patients with systemic sclerosis (SSc) using optical coherence tomography angiography (OCTA). Methods In this cross-sectional study SSc patients without clinical evidence of ocular involvement and healthy, age- and sex-matched volunteers were recruited. Participants underwent specific rheumatological and ophthalmological examinations, including optical coherence tomography (OCT) and OCTA. Retinal and choroidal thicknesses as well as perfusion of the retina and the choroidal sublayers were evaluated. Results A total of 15 SSc patients (30 eyes) with a median disease duration of 60 months and 15 matched, healthy controls (30 eyes) were recruited. OCT data revealed a significantly lower macular volume, as well as Sattler's layer and Haller's layer thickness in SSc patients compared to controls. In OCTA analysis, the perfusion of both retinal plexus as well as Sattler's and Haller's layer were significantly reduced in the SSc group. Patients with a disease duration of more than 60 months showed a statistically significant positive correlation between retinal and choroidal malperfusion, while those with a shorter disease duration did not. Conclusion OCTA analysis confirmed impairment of retinal and choroidal microperfusion in SSc patients, supporting the hypothesis of wide spreading vascular injury. In early stages, either the retinal or the choroidal perfusion seems to be involved, while later on, vascular impairment affects both tissues alike. Both, retinal and choroidal examinations should be considered as soon as the diagnosis of SSc is made, to avoid missing out on early alterations.
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