Eye disease
Per disease
RA: necrotizing keratitis is emergency → CYC or antiTNF (IFX, ADA).
SLE: sicca (and secondary SjS), retinal vasculitis (± intraocular inflammation) by vasculopathy
± posterior uveitis. Also, ischemic optic neuropathy and optic neuritis.
DM: heliotrope rash, periorbital edema, sicca, orbital myositis.
GCA: ΑΙΟΝ, PION, CRAO (20%), retinal vasculitis, ophthalmic artery ischemia, occipital cortex
ischemia. Retinal involvement due to GCA is manifested as peripapillary cotton-wool spots,
which represent nerve fiber layer infarcts.
PAN: scleritis, PUK, ischemic neuropathy.
GCA: orbital involvement (15-50%). Scleritis, (νεκρωτική), ischemic neuropathy.
Behçet’s: panuveitis (+ hypopyon), retinal vasculitis (+ischemia + neovascularization), veno-
occlusion, CNS disease.
Sarcoidosis (26-38% ocular): AAU / panuveitis, sicca, optic neuritis, lid inflammation or
orbital disease.
Mixed Cryoglobulinemia: bilateral chronic uveitis.
Urticarial vasculitis: anterior uveitis, bilateral chronic uveitis, conjunctivitis, episcleriti, and
diffuse anterior scleritis.
Cogan: usually interstitial keratitis.
RP (50-80%): scleritis (35-41%), uveitis.
Drugs: anti-TNF: uveitis (paradoxal), herpetic keratitis, ETN/UST: scleritis, uveitis,
bisphosphonates: uveitis, scleritis.
Uveitis
Uveitis is characterized by inflammation of the uvea, which is the middle portion of the eye;
the anterior portion of the uvea includes the iris and ciliary body and the posterior portion of
the uvea is known as the choroid. Inflammation of the anterior uveal tract, characterized by
the presence of leukocytes in the anterior chamber of the eye, is called anterior uveitis and
is synonymous with iritis. When the adjacent ciliary body is also inflamed, it’s iridocyclitis.
The presence of leukocytes in the vitreous humor is intermediate uveitis and evidence of
active chorioretinal inflammation is posterior uveitis, respectively. Terms used to describe
forms of uveitis posterior to the lens include vitritis, intermediate uveitis, pars planitis,
choroiditis, retinitis, chorioretinitis.
The term retinal vasculitis often causes confusion. In part, this results from
diagnosing retinal vasculitis on the basis of vascular leakage as seen by a fluorescein
angiogram or on the basis of perivascular infiltrate as seen on examination. Similar criteria
, are not used to diagnose systemic vasculitis. Retinal vasculitis can be secondary to a disease
such as sarcoidosis or birdshot chorioretinopathy, which are not generally categorized as
vasculitides.
Tubulointerstitial nephritis and uveitis syndrome (TINU) presents as an acute
bilateral anterior uveitis but may evolve into a chronic anterior or, rarely, a chronic
panuveitis.
Acute (anterior) uveitis has a sudden onset with a limited duration: HLA-B27[+]
axSpA, Behçet (also panuveitis), sarcoidosis, HSV.
Recurrent uveitis describes a course of multiple attacks of sudden onset and
limited duration alternating with periods of remission when the uveitis is inactive and the
patient is not receiving therapy for the disease.
Chronic uveitis typically has an insidious onset and a persistent duration, with
disease relapse promptly on discontinuing therapy: PsA/IBD, sarcoidosis (also panuveitis),
JIA, CMV.
Causes
-High-income countries, 50-80% are non-infectious (but mostly eye-restricted) (axSpA = 35-
50%).
-Low- and middle-income countries: infectious (50%): TB and toxoplasmosis are common
-Worldwide: 25-50% of all cases worldwide, idiopathic uveitis.
Acute (anterior) uveitis: HLA-B27[+] axSpA, HSV, idiopathic (B27-associated or not)
Intermediate: don’t forget MS and Lyme
Retinal vasculitis: Behçet, sarcoidosis, SLE.
Panuveitis: Behçet, sarcoidosis, Voght-Koyanagi-Harada, TB, HSV, Toxoplasma
- Infectious
•Viral: HSV, CMV (posterior uveitis in adults is found almost exclusively in the
immunocompromised), HIV, West-Nile, COVID etc.
•Bacterial: syphilis, TB (esp. panuveitis), Lyme (esp. intermediate uveitis), Whipple,
Bartonella, Rickettsia, Leptospira, Brucella.
•Parasitic: toxoplasma (surprisingly common)
-Systemic immune-mediated (40%)
•Spondyloarthritis
SpA: 50% of AAU are B27(+) and of them 50-80% have axSpA. In axSpA, 25-40% have
uveitis. axSpA is typically unilateral and tends to resolve within 3 months of its onset.
Recurrences are common and can occur in the contralateral eye. The prognosis for this form
of uveitis is generally excellent.
PsA (7% have uveitis)/ IBD (2-9% have uveitis): frequently bilateral, posterior to the
lens, insidious in onset, chronic in duration and more common in females than males.
ReA: 5-20% develop uveitis and up to 50% at long-term follow-up. Usually unilateral.
•Other
Behçet: up to 80% develop uveitis and often presents as a panuveitis (+ hypopyon)
and retinal vasculitis.
Sarcoidosis: uveitis frequently associated with retinal vasculitis, which may be
either perivascular or involve retinal vascular changes. Nodules/ granulomas can be found in
many parts of the eye.
JIA: 15%, particularly in the subset with oligoarticular disease and ANA (+). The
onset of uveitis is usually between 2-8yrs and is asymptomatic in the majority of patients.
Per disease
RA: necrotizing keratitis is emergency → CYC or antiTNF (IFX, ADA).
SLE: sicca (and secondary SjS), retinal vasculitis (± intraocular inflammation) by vasculopathy
± posterior uveitis. Also, ischemic optic neuropathy and optic neuritis.
DM: heliotrope rash, periorbital edema, sicca, orbital myositis.
GCA: ΑΙΟΝ, PION, CRAO (20%), retinal vasculitis, ophthalmic artery ischemia, occipital cortex
ischemia. Retinal involvement due to GCA is manifested as peripapillary cotton-wool spots,
which represent nerve fiber layer infarcts.
PAN: scleritis, PUK, ischemic neuropathy.
GCA: orbital involvement (15-50%). Scleritis, (νεκρωτική), ischemic neuropathy.
Behçet’s: panuveitis (+ hypopyon), retinal vasculitis (+ischemia + neovascularization), veno-
occlusion, CNS disease.
Sarcoidosis (26-38% ocular): AAU / panuveitis, sicca, optic neuritis, lid inflammation or
orbital disease.
Mixed Cryoglobulinemia: bilateral chronic uveitis.
Urticarial vasculitis: anterior uveitis, bilateral chronic uveitis, conjunctivitis, episcleriti, and
diffuse anterior scleritis.
Cogan: usually interstitial keratitis.
RP (50-80%): scleritis (35-41%), uveitis.
Drugs: anti-TNF: uveitis (paradoxal), herpetic keratitis, ETN/UST: scleritis, uveitis,
bisphosphonates: uveitis, scleritis.
Uveitis
Uveitis is characterized by inflammation of the uvea, which is the middle portion of the eye;
the anterior portion of the uvea includes the iris and ciliary body and the posterior portion of
the uvea is known as the choroid. Inflammation of the anterior uveal tract, characterized by
the presence of leukocytes in the anterior chamber of the eye, is called anterior uveitis and
is synonymous with iritis. When the adjacent ciliary body is also inflamed, it’s iridocyclitis.
The presence of leukocytes in the vitreous humor is intermediate uveitis and evidence of
active chorioretinal inflammation is posterior uveitis, respectively. Terms used to describe
forms of uveitis posterior to the lens include vitritis, intermediate uveitis, pars planitis,
choroiditis, retinitis, chorioretinitis.
The term retinal vasculitis often causes confusion. In part, this results from
diagnosing retinal vasculitis on the basis of vascular leakage as seen by a fluorescein
angiogram or on the basis of perivascular infiltrate as seen on examination. Similar criteria
, are not used to diagnose systemic vasculitis. Retinal vasculitis can be secondary to a disease
such as sarcoidosis or birdshot chorioretinopathy, which are not generally categorized as
vasculitides.
Tubulointerstitial nephritis and uveitis syndrome (TINU) presents as an acute
bilateral anterior uveitis but may evolve into a chronic anterior or, rarely, a chronic
panuveitis.
Acute (anterior) uveitis has a sudden onset with a limited duration: HLA-B27[+]
axSpA, Behçet (also panuveitis), sarcoidosis, HSV.
Recurrent uveitis describes a course of multiple attacks of sudden onset and
limited duration alternating with periods of remission when the uveitis is inactive and the
patient is not receiving therapy for the disease.
Chronic uveitis typically has an insidious onset and a persistent duration, with
disease relapse promptly on discontinuing therapy: PsA/IBD, sarcoidosis (also panuveitis),
JIA, CMV.
Causes
-High-income countries, 50-80% are non-infectious (but mostly eye-restricted) (axSpA = 35-
50%).
-Low- and middle-income countries: infectious (50%): TB and toxoplasmosis are common
-Worldwide: 25-50% of all cases worldwide, idiopathic uveitis.
Acute (anterior) uveitis: HLA-B27[+] axSpA, HSV, idiopathic (B27-associated or not)
Intermediate: don’t forget MS and Lyme
Retinal vasculitis: Behçet, sarcoidosis, SLE.
Panuveitis: Behçet, sarcoidosis, Voght-Koyanagi-Harada, TB, HSV, Toxoplasma
- Infectious
•Viral: HSV, CMV (posterior uveitis in adults is found almost exclusively in the
immunocompromised), HIV, West-Nile, COVID etc.
•Bacterial: syphilis, TB (esp. panuveitis), Lyme (esp. intermediate uveitis), Whipple,
Bartonella, Rickettsia, Leptospira, Brucella.
•Parasitic: toxoplasma (surprisingly common)
-Systemic immune-mediated (40%)
•Spondyloarthritis
SpA: 50% of AAU are B27(+) and of them 50-80% have axSpA. In axSpA, 25-40% have
uveitis. axSpA is typically unilateral and tends to resolve within 3 months of its onset.
Recurrences are common and can occur in the contralateral eye. The prognosis for this form
of uveitis is generally excellent.
PsA (7% have uveitis)/ IBD (2-9% have uveitis): frequently bilateral, posterior to the
lens, insidious in onset, chronic in duration and more common in females than males.
ReA: 5-20% develop uveitis and up to 50% at long-term follow-up. Usually unilateral.
•Other
Behçet: up to 80% develop uveitis and often presents as a panuveitis (+ hypopyon)
and retinal vasculitis.
Sarcoidosis: uveitis frequently associated with retinal vasculitis, which may be
either perivascular or involve retinal vascular changes. Nodules/ granulomas can be found in
many parts of the eye.
JIA: 15%, particularly in the subset with oligoarticular disease and ANA (+). The
onset of uveitis is usually between 2-8yrs and is asymptomatic in the majority of patients.
