Uvea consists of the middle, pigmented, vascular structures of the eye and includes the iris, ciliary body, and choroid Uveitis is broadly defined as inflammation (ie, -itis)of the uvea (from the Latin uva, meaning "grape") It emphasizes the close relationship between the anatomically distinct parts of the uveal tract, for inflammatory processes tend to involve the uvea as a whole and are generally not limited to a single region

1. Uveitis
Signs and Symptoms
January 8 2020
Dr Shayri Pillai
Ist Year Ophthalmology Resident
Liberia Eye Centre
JFK Memorial Medical Center
L V Prasad Eye Institute

2. CONTENT
 INTRODUCTION
 CLASSIFICATION
 CLINICAL FEATURES
 REFERENCES

3. INTRODUCTION
 Uvea consists of the middle, pigmented, vascular structures
of the eye and includes the iris, ciliary body, and choroid
 Uveitis is broadly defined as inflammation (ie, -itis)of the
uvea (from the Latin uva, meaning "grape")
 It emphasizes the close relationship between the
anatomically distinct parts of the uveal tract, for
inflammatory processes tend to involve the uvea as a
whole and are generally not limited to a single region

4. CLASSIFICATION

5. Standardization of Uveitis Nomenclature
 The Standardization of Uveitis Nomenclature (SUN)
Working Group guidance on uveitis terminology,
endorsed by the International Uveitis Study Group
(IUSG)
 SUN Working Group in 2005 develop an anatomical
classification system, descriptors, standardized grading
systems, and terminology
 The system was adopted by leading uveitis specialists
from all over the world

6.  Based on uveitic entities into etiologic categories
(infectious or noninfectious) and then follows this basic
anatomical classification into 4 groups:
 Anterior- Anterior chamber is the primary site of
inflammation
 Intermediate- Primarily vitreous inflammation; includes
pars planitis
Posterior- Retina and/or choroid
 Pan uveitis- All uveal structures are involved

7. Anatomical classification of uveitis

8. The SUN Working Group Anatomical Classification of
Uveitis

9. The SUN Working Group based on etiology

10. The SUN Working Group Descriptors in Uveitis

11. The SUN Working Group Activity of Uveitis
Terminology

12.  Apart from the classifications based on the anatomical
site of involvement, uveitis can also be categorized by
clinical course as acute, chronic, recurrent and by
pathology
 They are of two types—
 Granulomatous
 Non-granulomatous

13.  Granulomatous iridocyclitis is characterized by the
presence of large, greasy ‘mutton fat’ keratic
precipitates
 They are deposits of white blood cells (mainly
lymphocytes) derived from the aqueous, on the corneal
endothelium and dense posterior synechiae with
clusters of inflammatory cells on the pupillary border
Koeppe nodules or on the peripheral part of the
anterior surface of the iris (Busacca nodules)

14. ‘Mutton fat’ keratic precipitates

15. Posterior synechiae

16. Granulomatous uveitis with Koeppe nodules at
the pupillary margin

17.  Non-granulomatous uveitis is characterized by the
presence of fine keratic precipitates, acute onset and
short duration, diffuse in extension and without focal
lesions in the iris
 There is a considerable flare and cells in the anterior
chamber
 Keratic precipitates are few and composed of lymphoid
cells and polymorphs

18. ANTERIOR UVEITIS

19.  Anterior uveitis is inflammation involving the anterior uveal
tract – the iris and the anterior part (pars plicata) of the
ciliary
body – and is the most common 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
 Acute anterior uveitis (AAU) is the most common
presentation, of which HLA-B27-related and idiopathic
forms make up the largest proportion

20. Clinical features

21. Symptoms
 AAU consist of the rapid onset of unilateral pain,
photophobia, redness and watery discharge, sometimes
preceded by mild ocular discomfort for a few days
 Blurring of vision is related to severity
 As recurrent disease is very common, especially with
the idiopathic and HLA-B27related types, there will often
be a history of previous similar episodes
 CAU may be of insidious or acute onset, and can be
asymptomatic until the development of complications
such as cataract

22.  Visual acuity is variably impaired depending on the
severity of inflammation and the presence of
complications
 It is frequently only mildly reduced in AAU

23.  Ciliary injection (perilimbal injection, ciliary flush or just
‘injection’) is circumcorneal conjunctival hyperaemia
with a violaceous (purplish) hue due to involvement of
deeper blood vessels , and is typically seen in anterior
uveitis of acute onset
 Ciliary injection is characteristically absent in some
forms of CAU, and occasionally AAU
Ciliary injection

24.  Miosis due to pupillary sphincter spasm predisposes to
the formation of posterior synechiae
Miosis

25.  Anterior chamber cells are a dependable indicator of
inflammatory activity
 Grading (SUN Working Group) is performed by
estimating the number of cells in a 1 mm by 1 mm slit
beam field, employing adequate light intensity and
magnification
 This must be performed before pupillary dilatation,
which can lead to shedding of pigment cells into the
aqueous
 Inflammatory cells are commonly also seen in the
anterior vitreous

26. Standardization of Uveitis Nomenclature (SUN)
Working Group grading of anterior chamber cells (1
mm by 1 mm slit beam)

27.  Hypopyon refers to a whitish purulent exudate
composed of myriad inflammatory cells in the
inferior part of the anterior chamber (AC), forming a
horizontal level under the influence of gravity
 Hypopyon is common in HLA-B27-associated AAU
when a high fibrin content makes it immobile and
slow to absorb
Hypopyon

28.  In patients with Behçet disease the hypopyon
contains minimal fibrin and so characteristically
shifts according to the patient’s head position

29.  Keratic precipitates (KP) are deposits on the corneal
endothelium composed of inflammatory cells such as
lymphocytes, plasma cells and macrophages
Fresh KP in early anterior uveitis

30.  They are usually concentrated inferiorly, often in a
triangular pattern with the apex pointing up (Arlt
triangle) under the influence of gravity and aqueous
convection currents; a notable exception is Fuchs
uveitis syndrome (FUS), in which they are diffusely
distributed

31.  KP characteristics indicate the probable type of
uveitis:
 Typically smaller in the non-granulomatous
inflammation typical of AAU
 Medium to large in (classically chronic) granulomatous
inflammation in which cell types may include epithelioid
and multinucleated cells
 Large greasy appearing granulomatous KP are in a
‘mutton fat’ appearance

32. Large ‘mutton fat’ keratic precipitates

33.  KP are small to medium and adopt a star-shaped
(‘stellate’) or filamentous morphology in FUS
 KP usually resolve as acute inflammation subsides:
long-standing non-granulomatous KP may become
pigmented; granulomatous KP may become
pigmented and/or assume a ‘ground glass’
appearance
 Endothelial dusting by numerous individual cells
precedes the formation of true KP aggregates

34. Stellate KPs in Fuchs uveitis
syndrome
Old pigmented granulomatous KP
Endothelial cellular ‘dusting’ and early KP formation

35.  Aqueous flare is haziness of the normally clear fluid in
the anterior chamber, reflecting the presence of protein
due to breakdown of the blood–aqueous barrier
 Presence of flare indicates active inflammation with a
resultant higher risk of complications over the longer term
 Flare may be graded clinically using a slit lamp to assess
the degree of interference with visualization of iris and
lens
 When available, laser flare photometry gives greater
objectivity

36. Aqueous fare (4+) in a patient with acute iritis

37. SUN Working Group slit lamp grading scheme for
anterior chamber flare

38.  Fibrinous exudate in the anterior chamber is
common in severe AAU, and as with hypopyon is
often seen with HLA-B27-related inflammation
Fibrinous exudate

39.  Iris nodules: Koeppe nodules are located on the
pupillary margin and may be the site of posterior
synechiae formation They can occur in both
granulomatous and non-granulomatous anterior uveitis
Koeppe nodules in Fuchs uveitis syndrome

40.  Busacca nodules involve the iris stroma and are a
feature of granulomatous uveitis
 Yellowish nodules can develop from dilated iris vessels
(roseolae) in syphilitic uveitis.
Busacca and Koeppe nodules Very large nodule in sarcoid uveiti

41.  Iris ‘pearls’ may be seen in lepromatous chronic anterior
uveitis
 Iris crystals (Russell bodies), thought to consist of
immunoglobulin deposits, are a rare finding in some
cases of chronic uveitis including FUS
Iris crystals (Russell bodies) in chronic syphilitic uveitis

42.  Posterior synechiae (PS) are inflammatory adhesions
between the pupil margin and the anterior lens capsule
and may be particularly likely to form at the location of a
Koeppe nodule
 They can develop rapidly, and to prevent their formation
initial prophylaxis with a mydriatic agent is routine in all but
very mild AAU
 Once established, every attempt must be made to break
PS before they become permanent

43. Extensive synechiae and
pigment on the lens following
severe acute anterior uveitis
Adhesions in active acute anterior
uveitis

44. Recently broken synechiae in a patient
with HLA-B27-associated acute anterior
uveitis

45.  Iris atrophy may offer useful diagnostic clues
 Diffuse stromal atrophy is seen in FUS, and patchy or
sectoral atrophy can occur in herpetic uveitis both patterns
may be seen in both simplex and zoster-related
inflammation, though the latter is said to more commonly
give a sectoral pattern
Extensive iris atrophy following herpes zoster
ophthalmicus – predominantly sectoral pattern

46.  Iris neovascularization (rubeosis iridis) can occur,
particularly in chronic inflammation
 The process tends to be less acute than with a
primary vascular cause such as central retinal vein
occlusion
 Abnormal iris vessels are very common in FUS, but
do not cause synechial angle closure

47. INTERMEDIATE UVEITIS

48.  Intermediate uveitis (IU) is a chronic, relapsing
disease of insidious onset in which, according to the
SUN Working Group, the vitreous is the primary site
of inflammation as determined clinically
 It incorporates pars planitis, posterior cyclitis and
hyalitis

49. Clinical features

50. Symptoms
 Presentation is with the insidious onset of blurred
vision, often accompanied by vitreous floaters; there is
usually no pain or redness
 Though initial symptoms are often unilateral, objective
findings are typically present asymmetrically in both
eyes

51.  Visual acuity is variably affected depending on
inflammatory activity and complications, particularly
CMO
 The disease may last as long as 15 years and
preservation of vision will depend largely on control of
macular disease
 In follow-up of up to 4 years, 75% of patients maintain
a visual acuity of 6/12 or better

52.  Anterior uveitis- In PP there may be a few cells and
small scattered KP which occasionally have an
inferior linear distribution
 In other forms of IU, anterior uveitis and its
associated findings such as PS can be more
prominent, especially in children, and in sarcoidosis
and Lyme disease

53.  Vitreous Vitreous cells with anterior predominance
are universal, with vitreous condensation and haze
in more severe cases
 Snowballs are whitish focal collections of
inflammatory cells and exudate, usually most
numerous in the inferior vitreous

54. Grading of vitreous haze

55. Vitreous inflammatory activity

56.  Peripheral periphlebitis is also very common
 Careful examination of a normal fellow eye in
apparently unilateral disease may reveal mild vascular
sheathing

57.  Snowbanking is characterized by a grey–white
fibrovascular and/or exudative plaque that may occur
in any or all quadrants
 Most frequently found inferiorly

58. Intermediate uveitis. (A) Snowballs; (B) peripheral periphlebitis
and snowballs; (C) inferior snowbanking and snowballs

59.  Neovascularization may occur, particularly in the
retinal periphery (often associated with snowbanks)
and on the optic nerve head; the latter usually
resolves when activity is controlled
 This can sometimes lead to vitreous haemorrhage,
retinal detachment and cyclitic membrane formation

60.  Optic disc swelling is common, especially in younger
patients
 CMO occurs in up to half of patients and is the major
cause of impaired visual acuity
 Macular Epiretinal Membrane, Retinal Detachment,
Cataract, Glaucoma can also be seen

61. POSTERIOR UVEITIS

62.  Inflammations of the posterior uvea exhibit the
general characteristics of those affecting the anterior
part of the uveal tract
 They may appear either in the form of isolated foci of
inflammation or they may be diffuse

63.  Typical symptoms of posterior uveitis include
the presence of ‘floaters’ with or without a
diminution of vision, which is often painless
 Occasionally accompanied by pain, photophobia
and some redness if there is associated
involvement of the anterior segment

64.  Signs include detectable inflammatory cells and
opacities in the vitreous (vitritis), exudates or infiltration
in the retina or choroid, oedema of the retina and choroid,
and sheathing of vessels
 Other less frequent features include:
Disc oedema, retinal haemorrhages, associated signs of
anterior segment inflammation such as posterior
synechiae, anterior aqueous flare and cells, i.e. ‘spill-over’
uveitis
 Late changes such as a complicated cataract,
glaucoma, retinal detachment or choroidal
neovascularization may occur

65. Ocular sarcoidosis

66. Active Toxoplasma retinitis

67. Acute retinal necrosis

68. HIV microangiopathy

69. Signs of Uveitis

70. REFERENCES
Skuta,G.L. et. al. American Academy of Ophthalmology
Intra ocular inflammation and Uveitis 2018 Edition. USA
Pg. 83-89
Bowling , B. Kanski’s Clinical Ophthalmology: A Systemic
Approach, 8th Ed., 2016. Australia. P.399-455
Sihota, R. et al. Parsons’ Diseases of the Eye, 22nd Ed.,
2015 p.232-239

71. Thank you!
Excellence Equity Efficiency
L V Prasad Eye Institute