Long-Term Management of IRVAN: A Clinical Challenge
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Yes
Purpose
To characterize long-term management of IRVAN (Retinal Vasculitis, Aneurysms, and Neuroretinitis Idiopathic Retinal Vasculitis).
Methods
Retrospective chart review of clinical/laboratorial data/multimodal imaging.
Results
Three females, aged12-33 years, presented with bilateral disease, two with acute/recent changes consisting of multiple arteriolar aneurysms(AA), optic disc swelling(ODS), lipid exsudation(LE) and extensive peripheral vascular sheathing/oclussion(PVSO), consistent with IRVAN. The other younger one had chronic/longstanding changes, inclusing bilateral foveal scarring, diffuse lipid exudation and PSVO, with a history of “Coat’s disease”, treated elsewhere with laser/anti-VEGF. BCVA at presentation ranged from 20/20 to 20/160. All cases were aggressively treated with corticosteroid therapy, immunomodulation, laser photocoagulation, and the two older ones also with anti-VEGF. Intraocular inflammation was subsequently controlled, with stabilization or improvement of VA in all cases to a range of 20/20 to 20/70, with a follow-up of 38-148 months. Curiously, the younger patient initially presenting with chronic changes developed recrudescence (onset of AA, increased LE and ODS), after 7 years of my immunomodulation with methotrexate. Adalimumab was added, with a course of PO steroids, further leading to control of the condition over the following 5 years of follow-up.
Conclusion
IRVAN represents a rare and chronic potentially vision-threatening condition requiring early diagnosis and treatment. Multimodal imaging is critical for accurate diagnosis/monitoring. Aggressive long-term management with steroids, immunomodulation, laser and anti-VEGF can effectively stabilize disease and prevent vision loss, improving long-term outcomes.
Conflict of interest
No
1
Last name
MACHADO
Initials of first name(s)
D.O
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
2
Last name
Andrade
Initials of first name(s)
A.C
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG),
City
Belo Horizonte
Country
Brazil
3
Last name
Rocha
Initials of first name(s)
AC.H
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
4
Last name
Beneti
Initials of first name(s)
G.C
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG),
City
Belo Horizonte
Country
Brazil
5
Last name
Lara
Initials of first name(s)
C.M
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG)
City
Belo Horizonte
Country
Brazil
6
Last name
Vasconcelos-Santos
Initials of first name(s)
D.V
Department
Hospital São Geraldo/HC da Universidade Federal de Minas Gerais (UFMG
City
Belo Horizonte
Country
Brazil
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