1 Ann Rheum Dis Month 2019 Vol 0 No 0
Is retinal microvascular damage an early
manifestation of systemic sclerosis?
We read with great interest the paper by Rothe et al, recently
published on Annals of the Rheumatic Diseases,
1
who provided
evidence that patients affected with systemic sclerosis (SSc) had
impaired retinal microvascular perfusion, despite the lack of
ocular symptoms. Angiograms of the full retina were obtained
by optical coherent tomography (OCTA) in 12 patients with SSc
with established disease and 12 matched healthy subjects in a
case-control cross-sectional study. The retinal microvascular
damage was not confirmed by the conventional fluorescence
angiography (FA), but we do believe that FA would have detected
similar findings, as previously reported.
2 3
We were not surprised
by these results as a tissue vascular damage is expected whereby
macro-angiopathy and micro-angiopathy are the hallmark of
the disease. Indeed, in Rothe’s study, 10 out of 12 patients have
suffered with digital ulcers,
1
although a correlation between
retinal vasculitis and nailfold capillaroscopy patterns had not
been reported.
3
However, the issue to be raised is whether the
OCTA findings can be predictive of an overt ocular disease and
therefore these patients should be worthy to undergo a specific
treatment. Some years ago, we reported a series of three patients
with SSc whose disease onset was characterised with visual disor-
ders and retinal vascular abnormalities at FA.
4
All three patients
had Raynaud phenomenon (RP) and tested positive for antinu-
clear antibodies, but only one had also sclerodactyly by the time
of ocular disease and none fulfilled the 1980 ACR classification
criteria for SSc. Additional SSc-related symptoms occurred after
1–5 years. Of note, after release of ACR/EULAR 2013 classifica-
tion criteria, all three patients would have defined as early SSc:
this evidence can pave the way to several points of speculation.
Should patients with acute vision abnormalities and RP be
investigated for SSc? Might OCTA be of aid for identifying
patients with early SSc at risk to develop ocular disease? We
believe that these issues should be addressed by further studies
on longitudinal cohort of patients with SSc with early disease.
Florenzo Iannone, Cinzia Rotondo
School of Medicine, University of Bari, Bari, Italy
Correspondence to Professor Florenzo Iannone, School of Medicine, University of
Bari, Bari 70124, Italy; forenzo.iannone@uniba.it
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; internally peer reviewed.
© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and
permissions. Published by BMJ.
To cite Iannone F, Rotondo C. Ann Rheum Dis Epub ahead of print: [please include
Day Month Year]. doi:10.1136/annrheumdis-2019-215127
Received 24 January 2019
Accepted 26 January 2019
Ann Rheum Dis 2019;0:1. doi:10.1136/annrheumdis-2019-215127
REFERENCES
1 Rothe M, Rommel F, Klapa S, et al. Evaluation of retinal microvascular perfusion
in systemic sclerosis: a case–control study. Ann Rheum Dis 2019. doi:10.1136/
annrheumdis-2018-214541. [Epub ahead of print: 3 Jan 2018].
2 Waszczykowska A, Goś R, Waszczykowska E, et al. Prevalence of ocular manifestations
in systemic sclerosis patients. Arch Med Sci 2013;9:1107–13.
3 Ushiyama O, Ushiyama K, Yamada T, et al. Retinal fndings in systemic sclerosis: a
comparison with nailfold capillaroscopic patterns. Ann Rheum Dis 2003;62:204–7.
4 Rotondo C, Lopalco G, Lapadula G, et al. Retinal vessels thrombosis as onset
manifestation of systemic sclerosis: 3 clinical cases. J Rheumatol 2014;41:2495–6.
Correspondence
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