Review Article SMJ
Retinal Vasculitis: Fundamentals, Diagnostics, and
Management
Sutasinee Boonsopon, M.D.*, Stephen D. Anesi, M.D., FACS*,***
*Department of Ophthalmology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Tailand, **Massachusetts Eye Research and
Surgery Institution, Waltham, MA, USA and ***Ocular Immunology and Uveitis Foundation, USA.
ABSTRACT
Retinal vasculitis is inflammation of retinal blood vessels typically resulting from infection or immune-mediated
inflammatory processes. It may present as isolated ocular inflammation or as a part of severe or potentially fatal
systemic disease. Ocular complications of retinal vasculitis include cystoid macular edema, neovascularization,
tractional retinal detachment, and vitreous hemorrhage, which all greatly threaten vision. Multimodal imaging
and thorough systemic investigations are the main tools for making a precise diagnosis, which aids in predicting
disease prognosis and visual outcome as well as preserving a patient’s vision and possibly their life. Tis review
aims to discuss the current understanding of retinal vasculitis as well as current diagnostic tools and treatments.
Keywords: Retinal vasculitis; retinal vascular inflammation; posterior uveitis (Siriraj Med J 2021; 73: 493-500)
INTRODUCTION
thorough systemic work up are important when trying
Retinal vasculitis (RV) is a sight-threatening condition
to uncover the etiology of the inflammation as it may
that is characterized by inflammation of the retinal blood
help to improve prognosis and visual outcome, as well
vessels. Tis condition is defined by ophthalmologists
as reduce secondary complications such as retinal and
as impairment of the blood-retina barrier resulting
macular ischemia, cystoid macular edema (CME), and
from inflammatory processes. Diagnosis of RV can be
secondary glaucoma. Tese are associated with poor visual
primarily made by finding sheathed retinal vessels or
outcome.1-3 Tis review focuses first on the fundamentals
perivascular exudate during dilated fundus examination.
of retinal vascular inflammation, and then aims to further
However, this condition should be evaluated by and can
discuss newer diagnostic tools and treatments.
be confirmed with fluorescein fundus angiography. RV
may be idiopathic or may be a manifestation of systemic
Epidemiology
inflammatory diseases, such as Adamantiades-Behcet’s
Studies have shown RV to be found in 6-15% of
disease (ABD), granulomatosis with polyangiitis (GPA),
patients with uveitis.4,5 Ethnicity may be responsible
and systemic lupus erythematosus (SLE). RV can also
for differences in the incidence and prevalence of RV
occur as a result of infection, such as viral acute retinal
worldwide. Each specific disease related to RV was also
necrosis, ocular toxoplasmosis, and ocular tuberculosis.
found to have a different incidence and prevalence,
It may seldomly occur in the setting of a malignant
such as in ABD, which was found in 20-420/100,000
masquerade syndrome. Multimodal imaging and a population in Turkey, in 80/100,000 population in Iran,
Corresponding author: Sutasinee Boonsopon
E-mail: sutasinee.boo@mahidol.edu
Received 28 April 2021 Revised 12 July 2021 Accepted 12 July 2021
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Boonsopon et al.
and in 0.64/100,000 population in the United Kingdom,
when there is a significant area of retinal ischemia, and
and among these ABD patients, uveitis presented with
NV may eventually lead to fibrovascular traction and/
or without RV in 45-90%.6 Te incidence of SLE also
or vitreous hemorrhage. RV can also be categorized by
differs among regions, ranging from 0.3 to 8.7/100,000
the affected retinal blood vessels into periarteriolitis,
population/year, and the prevalence ranged from 1.1
periphlebitis, or combined arteriolitis and phlebitis. Also,
to 534.9/100,000 population. Te highest incidence of
a pattern of small vessel leakage affecting retina where
SLE was found in the USA, the Caribbean, Brazil, and
the vasculature is not clearly visible may be considered
Sweden.7 Te incidence of retinal involvement in SLE was
capillaritis. Te usefulness of identifying the involved
7-26%.8 Regarding infectious etiology, ocular tuberculosis
retinal blood vessels is to narrow the differential diagnosis
(TB) is the predominant culprit in Asia. Te incidence
in hopes of unveiling the etiology of inflammation.
of ocular TB in Australia was 0.77/100,000, and 11% of
Causes of RV associated with different types of retinal
those patients presented with RV.7 In India, the reported
blood vessel involvement are shown in Table 1,2 which
incidence of ocular TB was as high as 9.86% among all
can be categorized into systemic autoimmune-related
types of uveitis.9
RV, infectious-related RV, isolated ocular inflammation
with known etiology, and idiopathic RV.1 Te etiologies
Pathogenesis
of RV categorized by systemic disease association are
Te mechanisms of the development of RV are
shown in Table 2.
not fully understood. Type III (immune complex-
mediated) hypersensitivity is generally believed to be
Clinical findings
responsible for the development of RV,3 but humoral
Te most common ocular symptom of patients with
and cell-mediated immunity may also play an important
RV is blurred vision. Additional symptoms depend on
role. Immunohistochemical analysis of enucleated eyes
several factors, including extension of involved retinal blood
from ABD and sarcoidosis patients revealed T-helper
vessels, associated findings, and existing complications, such
lymphocytes comprise most of the involved cells. Major
as vitritis, vitreous hemorrhage, CME, retinal or macular
histocompatibility complex (MHC) I and II antigens
ischemia, and papillitis. Patients may also experience
and the cell adhesion molecules intercellular adhesion
photopsia, floaters, metamorphopsia, and either focal or
molecule-1 (ICAM-1), E-Selectin, vascular cell adhesion
diffuse scotomas. Te location of inflammation may be
protein-1 (VCAM-1), lymphocyte function-associated
limited to the posterior segment of the eye as posterior
antigen (LFA)-1a, and LFA-1b were found on vascular
uveitis, or as panocular inflammation in more severe cases.
endothelial cells.1 In animal models and following systemic
On dilated fundus examination, one may find perivascular
inoculation and induction of experimental autoimmune
infiltration, vascular cuffing, or vascular sheathing. In
uveitis, retinal S antigen was found to play an important
SLE (with or without antiphospholipid thrombosis),
role in immune-mediated uveitis and RV.10-12 Many studies
presentation with classic RV with perivascular infiltration
also reported a genetic predisposition to be related to
was less common than presentation with features of
retinal vascular inflammation. Previous reports described
occlusive vasculopathy.7 Exudates that result from vascular
a condition called autosomal dominant neovascular
leakage are occasionally observed. Cotton wool spots
inflammatory vitreoretinopathy (ADNIV) as being related
representing retinal nerve fiber layer infarction can be
to the development of intraocular inflammation that
seen in occlusive retinal arteriolitis. Late presentations of
results from mutations in calcium-dependent cysteine
retinal vascular occlusion include retinal telangiectasia,
protease (Calpain 5).13 Some mutations may associate
neovascularization, tractional retinal detachment, rubeosis
with predisposition to specific diseases, such as three
iridis, neovascular glaucoma, and optic disc atrophy.16
prime repair exonuclease 1 (TREX1) in SLE14, and tumor
necrosis factor alpha-induced protein 3 (TNFAIP3) in
Diseases mimicking retinal vasculitis
ABD-like disease.15
Non-inflammatory conditions, such as diabetic
retinopathy and Coats’ disease, can sometimes be confused
Classification
with RV because of extensive vascular leakage. Significant
Retinal vascular inflammation can be classified as either
amounts of perivascular exudates along retinal blood vessels
occlusive or non-occlusive depending on the mechanisms
may mislead the diagnosis. Sheathing of retinal blood
of inflammation. When vascular occlusion develops, the
vessels, which can be found in RV, can also be found in
affected area of the retina and/or macula may become
other conditions, such as retinal vein or artery occlusion
ischemic. Neovascularization (NV) generally develops
due to other causes.17-19 Te presence of inflammatory signs
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TABLE 1. Retinal vasculitis categorized by type of retinal blood vessel involvement.
Arteriolitis
Periphlebitis
Combined arteriolitis and periphlebitis
Acute retinal necrosis
Ocular tuberculosis
Crohn’s disease
Cat-scratch
Sarcoidosis
Granulomatosis with polyangiitis
Ocular syphilis
Birdshot chorioretinopathy
Relapsing polychondritis
Ocular toxoplasmosis
Pars planitis
Multiple sclerosis
Idiopathic retinal vasculitis,
Adamantiades-Behcet’s disease
aneurysm and neuroretinitis
Polyarteritis nodosa
Systemic lupus erythematosus
± antiphospholipid syndrome
TABLE 2. Differential diagnosis of retinal vasculitis according to etiology of inflammation.
Immune-related
Infectious
Masquerade
Systemic
Ocular
Adamantiades-Behcet’s
Birdshot chorioretinopathy
Acute retinal necrosis
Leukemia
disease
HLA*-B27 related/ seronegative
Pars planitis
Cytomegalovirus retinitis
Lymphoma
spondyloarthropathy
Granulomatosis with polyangiitis
Idiopathic retinal vasculitis,
Syphilis
aneurysm and neuroretinitis
Multiple sclerosis
Toxoplasmosis
Polyarteritis nodosa
Tuberculosis
Sarcoidosis
Systemic lupus erythematosus
*HLA = human leukocyte antigen
and associated findings of uveitis, such as inflammatory
Fig 1 shows an ultrawide field image of cytomegalovirus
cells and keratic precipitate, support an inflammatory
retinitis with frosted branch angiitis.
origin.
Fundus fluorescein angiography (FFA)
Multimodal imaging
FFA is the gold standard method for diagnosis of
Fundus photography
RV. It can also be used as a tool for treatment monitoring,
Location and extension of retinal and choroidal
for complication detection, and (in some instances)
inflammation can sometimes be documented using
for evaluating disease prognosis. FFA can reveal non-
fundus photography, and images from each follow-up
perfusion areas of ischemic retina in patients with occlusive
visit can be used for treatment monitoring except for
RV, and showcase damage induced in vital sections
the patients with subclinical RV. Ultrawide field images
of retina crucial for fine vision (i.e. macula). Patients
facilitate visualization of a wider field of peripheral retina.
with a large area of retinal ischemia and/or macular
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Boonsopon et al.
Indocyanine green angiography (ICG)
ICG is generally used to detect abnormalities in
choroidal vasculature. In RV, combined FFA and ICG
would be beneficial when inflammation involves both the
retina and choroid, such as in Vogt-Koyanagi-Harada
disease and birdshot chorioretinopathy (BSCR). Examples
of choroidal abnormalities related to RV include patchy
hypo-cyanescence, fuzzy choroidal vasculature, and late
hyper-cyanescence of choroidal vasculature.21
Adaptive optics (AO) imaging
AO, which was previously developed for astronomical
Fig 1. Ultrawide field image of cytomegalovirus retinitis.
application, is a non-invasive and feasible imaging system.
AO imaging in RV may reveal blood vessel wall thickening
A 27-year-old polymyositis patient presented with blurred vision.
from inflammatory cell deposition. AO can also be
Fundus photo of the right eye shows a classic presentation of
cytomegalovirus retinitis. Whitish fluffy retinal infiltrates, optic disc
used for follow-up imaging, with more defined vessel
infiltrates, intraretinal hemorrhages, peripheral granular retinal
lumens indicating less inflammatory cell deposition.
infiltrates and perivascular infiltrates are presented.
Other advantages of AO over FFA in addition to its non-
invasive nature include its ability to detect subclinical
RV earlier than detected by FFA.25 Current challenges
ischemia generally have poor visual outcome.20 FFA
associated with the use of AO imaging in this setting
can also detect subclinical RV in patients thought to be
include media opacity, peripheral RV, and pseudophakia.
otherwise clinically inactive. FFA findings in patients
Also, this is not readily available in a clinical setting.
with RV include retinal vascular leakage/staining, optic
disc leakage/staining, macular leakage, capillary drop out,
Optical coherence tomography (OCT) and optical
vascular occlusion, and blockage.21 Ultrawide field FFA
coherence tomography angiography (OCTA)
images are preferred to conventional FFA method due
Structural abnormalities related to RV can be identified
to improved detection of peripheral retinal lesions.22-24
by OCT. Focal retinal thickening with loss of normal
Figs 2 & 3 show fundus photos and fundus fluorescein
retinal lamination that suggested retinal damage has
angiography of RV patients.
been reported.26 Kyrieleis plaques in ocular toxoplasmosis
Fig 2. Fundus photo and fundus fluorescein angiography
of bilateral retinal vasculitis with Behcet’s disease.
Fundus photo of a 36-year-old male, Behcet’s disease
patient shows retinal vascular sheathing in both eyes
(a). Fundus fluorescein angiogram demonstrates more
obvious vascular leakage, optic disc leakage in both
eyes and macular leakage in the right eye (b) compare
to fundus photo.
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Fig 3. Fundus photo and fundus fluorescein
angiography of ocular tuberculosis.
A 31-year-old male patient was diagnosed with
ocular tuberculosis. Interferon gamma releasing
assay was 2.1, Mantoux test was 8 mm, chest
radiograph showed no pulmonary infiltration.
Dilated fundus examination showed retinal
periphlebitis with secondary branch vein occlusion
(a). Fundus fluorescein angiogram demonstrates
optic disc leakage, vascular leakage, and capillary
drop-out (b).
and cytomegalovirus retinitis can be observed on OCT
autoimmune diseases, immunomodulatory therapy
as hyper-reflectivity of blood vessel walls.21 CME is
(IMT) or biologics can be considered to reduce the side
clearly identified on OCT. Enhanced-depth imaging OCT
effects of corticosteroids and to achieve better control
yields more detail about choroidal thickness, including
of inflammation.
suprachoroidal fluid.3 Microvascular flow of the retina
and choroid can be detected by OCTA. Moreover, OCTA
Regional corticosteroids
can identify microvascular occlusion without injection
Regional steroids, such as trans-septal/sub-Tenon’s/
of dye. Unfortunately, the stage of inflammation (active
intravitreal triamcinolone acetonide (TA) injection, can
vs. inactive) cannot be determined. Another limitation
be considered as an adjunctive therapy for RV with or
of OCT and OCTA is its limited field of view.21
without CME. However, since the duration of TA injection
treatment is short, the use of TA injection alone is not
Diagnostic testing
recommended. Intravitreal dexamethasone implant is
Te causes of RV vary. Laboratory investigations
thought by many to produce a slightly more sustained
and imaging should be based on clinical presentations.
effect than TA27,28 but was comparable to intravitreal
As such, the pattern of investigation should be based
triamcinolone injection at 8 weeks in terms of decreasing
on each patient’s clinical presentation and provisional
central subfield thickness of uveitic macular edema.29,30
diagnosis. A summary of common diagnostic studies in
Te available fluocinolone acetonide implants convey
RV is shown in Table 3.
an even longer treatment duration making them more
reasonable for non-infectious RV. In these therapies,
Treatment modalities
systemic side effects are ofen avoided, while local side
Treatment for RV is generally dependent on the
effects, including complicated cataract and steroid-
etiology of underlying disease, if any. In infectious RV,
induced glaucoma, are ofen still seen.27,28,31-33
specific antimicrobial therapy is indicated. In non-
infectious RV, treatment aims to control inflammation
Immunomodulatory therapy
to regain or at least stabilize vision and to prevent further
IMT is generally required when patients become
complications.
steroid-dependent, and they require long-term systemic
steroids to control inflammation. Prednisolone of any
Systemic corticosteroids
duration, but particularly more than 7.5 mg/day is considered
Te initial mainstay therapy for non-infectious RV,
risky by many if used chronically. Studies have shown
and particularly in bilateral cases, is systemic corticosteroids
that the use of prednisone 5 mg daily for only 8 weeks in
due to their effectiveness and rapid response. High-dose
post-menopausal women was associated with the potential
prednisolone is generally prescribed at the initial stage.
development of steroid-related complication, specifically
Both short term and long term unwanted and widely
decreased indices of bone formation.34 Guidelines created
recognized side effects of high-dose corticosteroids are
by Jabs et al. recommend prescription of steroid-sparing
at times unavoidable, however an absolute certainty
agents when patients require chronic use of prednisolone
when used chronically. In patients who require long-
greater than 10 mg/day.35 Te Ocular Immunology and
term treatment or who suffer from serious systemic
Uveitis Foundation recommended to administer IMT
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TABLE 3. Investigations related to retinal vasculitis.
Basic investigations
Specific investigations
Imaging
Infection
Inflammation
Complete blood count
Anti-HIV*
Antibody blood tests
Chest radiograph/
Antinuclear antibody
computerized
ANCA†
tomography
Blood chemistries
Interferon-gamma
Anti-cardiolipin
Brain computerized
releasing assay for
Anti-b2 glycoprotein
tomography/ magnetic
tuberculosis/ T-Spot
Lupus anticoagulant
resonance imaging
TB
Anti-Smith antibody
Blood sugar
Toxoplasma serology
Anti-SSA‡/anti-SSB§
Ultrasound
Urinalysis
Treponemal/ non-treponemal
Complement/immune
Gallium scan
testing
complex studies
Mantoux test
C-reactive protein
Polymerase chain reaction
Erythrocyte
for Herpesviridae
sedimentation rate
(ocular specimen)
HLAII testing
CSF¶ analysis
Vitreous biopsy
*HIV = human immunodeficiency virus, †ANCA = antineutrophilic cytoplasmic antibody, ‡SSA = Sjogren’s syndrome A antibody, §SSB =
Sjogren’s syndrome B antibody, IIHLA = human leukocyte antigen, ¶CSF = cerebrospinal fluid
when corticosteroid therapy is required for longer than
medications (i.e., infliximab and adalimumab) are
3 months or a dose of more than 5 mg/day is required to
recommended as a first-line therapy for active ABD
control inflammation.36 Treatment with corticosteroids
with ocular inflammation by the American Uveitis
alone is not recommended in certain diseases, such as
Society.39 Te VISUAL I and VISUAL II clinical trials
ABD, GPA, and BSCR, since inflammation is generally
reported favorable results of adalimumab treatment
severe and requires long-term therapy.
in non-infectious uveitis patients.40 Other biologics in
Favored types of IMT include antimetabolite therapy
addition to anti-TNF-α agents have been used to treat
using methotrexate, azathioprine, and mycophenolate
RV, including rituximab (anti-CD20), daclizumab (anti-
mofetil, or the interleukin-2 inhibitor cyclosporine,
CD25), and tocilizumab (IL-6R inhibitor).3,20,41
though the latter is seldom used as monotherapy. Tese
are typically well tolerated and have a relatively low risk
Anti-vascular endothelial growth factor (anti-VEGF)
profile. Alkylating agents, such as cyclophosphamide
NV and CME are two main complications found in
and chlorambucil, have use, especially in more severe
RV that ofen require intravitreal anti-VEGF injection,
disease or inflammation that is recalcitrant to approaches
and this treatment can be used in both infectious and
associated with less risk, but are usually relied upon later
non-infectious RV. When anti-VEGF is considered,
due to their potential complications.1,37,38
it is sometimes used in combination with panretinal
photocoagulation (PRP) for prevention or treatment of
Biologics
ischemic RV.37 A systematic review of 4 studies comparing
ABD is a good example of RV that responds well
intravitreal triamcinolone and intravitreal bevacizumab
to biologics. Anti-tumor necrosis factor alpha (TNF-α) revealed no significant difference in visual acuity at
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any time point afer the treatment of uveitic macular
vitreoretinopathy
edema. One study showed significant difference in central
Anti-TNFa, anti-tumor necrosis factor alpha
macular thickness with potential favorable benefit with
Anti-VEGF, anti-vascular endothelial growth factor
intravitreal triamcinolone.42
AO, adaptive optics
BSCR, birdshot chorioretinopathy
Laser photocoagulation
Calpain 5, calcium-dependent cysteine protease
NV, tractional retinal detachment, and vitreous
CME, cystoid macular edema
hemorrhage are more severe complications that are
FFA, fundus fluorescein angiography
frequently found in occlusive RV. Te mainstay therapy for
GPA, granulomatosis with polyangiitis
managing these complications associated with widespread
ICG, indocyanine green angiography
and irreversible retinal ischemia is PRP. Unlike ischemic
IMT, immunomodulatory therapy
central retinal vein occlusion, there is no consensus
MHC, major histocompatibility complex
regarding the appropriate timing of PRP in occlusive
NV, neovascularization
RV. Rouvas et al. proposed PRP when there is retinal
OCT, optical coherence tomography
ischemia in more than 2 quadrants in idiopathic RV,
OCTA, optical coherence tomography angiography
aneurysm, and neuroretinitis (IRVAN).43 Aggressive PRP
RV, retinal vasculitis
may be beneficial in preventing these complications.1,37
SLE, systemic lupus erythematosus
TA, triamcinolone acetonide
Complications and prognosis
TB, tuberculosis
Chronic CME, macular ischemia, secondary
TNFAIP3, tumor necrosis factor alpha-induced protein 3
glaucoma, and optic disc atrophy are leading causes
TREX1, three prime repair exonuclease 1
of permanent visual damage.2,4 Occlusive RV tends to
develop complications, such as epiretinal membrane,
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