, INDEX
Classification 1
Anterior uveitis 1
Uveitis in spondyloarthropathies 17
Fuchs uveitis syndrome 21
Uveitis in juvenile idiopathic arthritis 23
Uveitis in bowel disease 29
Uveitis in renal disease 31
Intermediate uveitis 32
Vogt–Koyanagi–Harada (VKH) syndrome 37
Sympathetic ophthalmitis 40
Lens-induced uveitis 42
Sarcoidosis 44
Behçet disease 49
Parasitic uveitis 53
Viral uveitis 66
Fungal uveitis 77
Bacterial uveitis 81
Miscellaneous idiopathic chorioretinopathies 91
, CLASSIFICATION
The Standardization of Uveitis Nomenclature (SUN) Working Group guidance on
uveitis terminology, endorsed by the International Uveitis Study Group (IUSG),
categorizes uveitis anatomically (Fig. 11.1):
Anterior: the anterior chamber is the primary site of inflammation.
Intermediate: primarily vitreous inflammation; includes pars planitis.
Posterior: retina and/or choroid.
Panuveitis: all uveal structures are involved.
An IUSG clinical classification based on aetiology is also in use:
Infectious: bacterial, viral, fungal, parasitic, others.
Non-infectious: with and without a known systemic association.
Masquerade: neoplastic and non-neoplastic.
The SUN Working Group guidance includes the following descriptions relating to the
timing of inflammatory activity:
Onset: sudden or insidious.
Duration: limited (3 months or less) or persistent.
Clinical course: acute (of sudden onset and limited duration), recurrent (repeated
episodes separated by untreated inactive periods), or chronic (persistent duration,
with relapse less than 3 months after discontinuation of treatment). Remission is
defined as inactivity (no visible cells) for 3 months or longer.
ANTERIOR UVEITIS
Introduction
- Anterior uveitis is inflammation involving the anterior uveal tract – the iris and the
anterior part (pars plicata) of the ciliary body –; the commonest form of uveitis
Iritis refers to inflammation primarily involving the iris, and iridocyclitis to
involvement of both the iris and anterior ciliary body; in practice these are
interchangeable as they cannot be distinguished clinically.
1
, - Acute anterior uveitis (AAU) is the most common presentation, of which
HLA-B27-related and idiopathic forms make up the largest proportion. Aetiology
in these cases is uncertain, but may involve cross-reactivity with particular
microbial antigens in genetically predisposed individuals.
AAU can be a feature of a wide variety of ocular conditions such as trauma
(including surgery), lens-related inflammation and herpes simplex infection,
or can be secondary to inflammation elsewhere in the eye, such as bacterial
keratitis and scleritis.
AAU can also be the presenting clinical scenario, without accompanying
intermediate or posterior uveitis, in a range of systemic conditions including
chronic inflammatory disorders such as sarcoidosis.
- Chronic anterior uveitis (CAU) is less common than AAU.
It is more commonly bilateral, and associated systemic disease is more
likely. Granulomatous inflammatory signs (see below) are often present.
- Surveys of systemic associations of anterior uveitis vary in their findings; The
following table lists important possibilities, but is not exhaustive.
Idiopathic No detectable systemic association ــaround 50 %
Infectious • Varicella Zoster ــusually current or past ophthalmic shingles
• Tuberculosis
• Syphilis
• Lyme disease
• Miscellaneous systemic viral infections
Non- • HLA-B27 positivity ــaround 20% of AAU ــwith or without
infectious manifestations of HLA-B27 related systemic disease (see text)
• Juvenile idiopathic arthritis
• Sarcoidosis
• Behcet disease
• Tubulointerstitial nephritis and uveitis syndrome
• Systemic lupus erythematosus
• Multiple sclerosis
• Drug-induced (See ch.20)
Masquerade • Neoplastic, e.g. lymphoma, anterior segment melanoma
• Non-Neoplastic, e.g. juvenile xanthogranuloma
- The prognosis is usually good in most idiopathic and HLA-B27- related AAU
provided management is adequate. Outcomes are more variable in CAU and in
cases where there is an underlying ocular or systemic disorder.
2
Classification 1
Anterior uveitis 1
Uveitis in spondyloarthropathies 17
Fuchs uveitis syndrome 21
Uveitis in juvenile idiopathic arthritis 23
Uveitis in bowel disease 29
Uveitis in renal disease 31
Intermediate uveitis 32
Vogt–Koyanagi–Harada (VKH) syndrome 37
Sympathetic ophthalmitis 40
Lens-induced uveitis 42
Sarcoidosis 44
Behçet disease 49
Parasitic uveitis 53
Viral uveitis 66
Fungal uveitis 77
Bacterial uveitis 81
Miscellaneous idiopathic chorioretinopathies 91
, CLASSIFICATION
The Standardization of Uveitis Nomenclature (SUN) Working Group guidance on
uveitis terminology, endorsed by the International Uveitis Study Group (IUSG),
categorizes uveitis anatomically (Fig. 11.1):
Anterior: the anterior chamber is the primary site of inflammation.
Intermediate: primarily vitreous inflammation; includes pars planitis.
Posterior: retina and/or choroid.
Panuveitis: all uveal structures are involved.
An IUSG clinical classification based on aetiology is also in use:
Infectious: bacterial, viral, fungal, parasitic, others.
Non-infectious: with and without a known systemic association.
Masquerade: neoplastic and non-neoplastic.
The SUN Working Group guidance includes the following descriptions relating to the
timing of inflammatory activity:
Onset: sudden or insidious.
Duration: limited (3 months or less) or persistent.
Clinical course: acute (of sudden onset and limited duration), recurrent (repeated
episodes separated by untreated inactive periods), or chronic (persistent duration,
with relapse less than 3 months after discontinuation of treatment). Remission is
defined as inactivity (no visible cells) for 3 months or longer.
ANTERIOR UVEITIS
Introduction
- Anterior uveitis is inflammation involving the anterior uveal tract – the iris and the
anterior part (pars plicata) of the ciliary body –; the commonest form of uveitis
Iritis refers to inflammation primarily involving the iris, and iridocyclitis to
involvement of both the iris and anterior ciliary body; in practice these are
interchangeable as they cannot be distinguished clinically.
1
, - Acute anterior uveitis (AAU) is the most common presentation, of which
HLA-B27-related and idiopathic forms make up the largest proportion. Aetiology
in these cases is uncertain, but may involve cross-reactivity with particular
microbial antigens in genetically predisposed individuals.
AAU can be a feature of a wide variety of ocular conditions such as trauma
(including surgery), lens-related inflammation and herpes simplex infection,
or can be secondary to inflammation elsewhere in the eye, such as bacterial
keratitis and scleritis.
AAU can also be the presenting clinical scenario, without accompanying
intermediate or posterior uveitis, in a range of systemic conditions including
chronic inflammatory disorders such as sarcoidosis.
- Chronic anterior uveitis (CAU) is less common than AAU.
It is more commonly bilateral, and associated systemic disease is more
likely. Granulomatous inflammatory signs (see below) are often present.
- Surveys of systemic associations of anterior uveitis vary in their findings; The
following table lists important possibilities, but is not exhaustive.
Idiopathic No detectable systemic association ــaround 50 %
Infectious • Varicella Zoster ــusually current or past ophthalmic shingles
• Tuberculosis
• Syphilis
• Lyme disease
• Miscellaneous systemic viral infections
Non- • HLA-B27 positivity ــaround 20% of AAU ــwith or without
infectious manifestations of HLA-B27 related systemic disease (see text)
• Juvenile idiopathic arthritis
• Sarcoidosis
• Behcet disease
• Tubulointerstitial nephritis and uveitis syndrome
• Systemic lupus erythematosus
• Multiple sclerosis
• Drug-induced (See ch.20)
Masquerade • Neoplastic, e.g. lymphoma, anterior segment melanoma
• Non-Neoplastic, e.g. juvenile xanthogranuloma
- The prognosis is usually good in most idiopathic and HLA-B27- related AAU
provided management is adequate. Outcomes are more variable in CAU and in
cases where there is an underlying ocular or systemic disorder.
2
