Non-infectious ocular inflammatory diseases

1. NON-INFECTIOUS
OCULAR
INFLAMMATORY
DISEASES
P R E S E N T E R : D R . I D D I N D YA B AW E
M O D U L ATO R : D R . L U S O BYA R E B E C C A

2. LECTURE OUTLINE
• Non-infectious scleritis
• Acute uveitis
• Acute non-granulomatous uveitis
• HLA-B27: Related Diseases: ankylosing spondylitis, reactive arthritis syndrome,
inflammatory bowel dz, psoriatic arthritis
• Tubulointestinal nephritis and uveitis syndrome
• Glaucomatocyclitis crisis
• Lens-associated uveitis: Phacoanaphylactic, phacotoxic
• Postoperative inflammation: infectious endophthalmitis; IOL-associated uveitis
• UGH and IOLs
• Drug-induced uveitis
• Chronic Anterior Uveitis
• Juvenile Chronic Arthritis (JCA)

3. LECTURE OUTLINE
• Idiopathic Anterior Uveitis
• Intermediate Uveitis (pars planitis)
• Ddx: of Posterior Uveitis
• Polyarteritis nodosa
• Birdshot retinochoroidopathy
• Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
• Serpiginous Choroidopathy
• Multifocal Choroiditis (MFC)
• Punctate Inner Choroiditis
• Progressive Subretinal Fibrosis and Uveitis Syndrome (SFU)
• Multiple Evanescent White Dot Syndrome
• Acute Retinal Occult Outer Retinopathy
• Behcet’s dz
• Panuveitis
• Sympathetic Ophthalmitis
• Vogt Koyanagi Harada Syndrome

4. NONINFECTIOUS SCLERITIS
• Scleritis, inflammation of the sclera, is a typically painful, destructive condition that carries a
potential risk of permanent ocular structural damage with visual compromise.
• Scleritis may be classified anatomically and etiologically.
• Scleritis may be anterior or posterior;
• Etiologies include infection, autoimmunity, trauma, or drug induced.
• Approximately 40% of scleritis cases are associated with a systemic disease, especially
rheumatoid arthritis

5. TREATMENT OF NONINFECTIOUS
SCLERITIS
• Nonsteroidal anti-inflammatory drugs
NSAIDs may be effective in the treatment of non-necrotizing scleritis.
• Relieve pain and reduce inflammation, more useful in milder cases.
• Indomethacin, flurbiprofen, and naproxen have all been used successfully.
• Corticosteroids
Topical corticosteroids like difluprednate and prednisolone; used in mild cases.
• Subconjunctival injections of corticosteroid effective in anterior nonnecrotizing scleritis,
but this route of admin has controversy.
• Systemic corticosteroids orally or high-dose IV

6. .
• Immunomodulatory therapy
Antimetabolites such as methotrexate, azathioprine, and mycophenolate mofetil have
been used successfully, and calcineurin inhibitors
• Scleritis associated with granulomatosis with polyangiitis and polyarteritis nodosa
require more aggressive therapy with rituximab or cyclophosphamide.
• Biologic agents such as the TNF inhibitors and rituximab help in recalcitrant scleritis.
•

7. PAIN MANAGEMENT
• Oral NSAIDs may be of benefit.
• The judicious use of oral narcotics may be appropriate for brief periods while
antiinflammatory therapy is instituted.
• Topical cycloplegia may also be of benefit.

8. .
• Surgery
Scleral reinforcement surgery may be needed:
-to address scleral thinning and
- to avoid the complications of globe rupture
• Materials used for grafting include cadaveric donor sclera, autogenous periosteum

9. ANTERIOR UVEITIS
• Most common form of uveitis,
• More than 90% of cases in a community-based practice.
• Incidence in the United States varies by age, from approximately 7 cases per 100,000
person-years in persons aged 14 years and younger
• Up to approximately 220 per 100,000 persons aged 65 years and older.

10. ACUTE
NONGRANULOMATOUS
ANTERIOR UVEITIS
• C/O: sudden onset of pain, redness, and photophobia that can be associated with
decreased vision.
• O/E:
-Fine keratic precipitates (KPs) dust the corneal endothelium in most cases.
-AC shows an intense cellular response and variable flare. Protein coagulum in the aqueous
or, less commonly, a hypopyon in severe cases.
-A fibrin net forms across the pupillary margin causing seclusion membrane and iris bombe.
- Iris vessels may be dilated, and, rarely, a spontaneous hyphema occurs.
- Cells occasionally in anterior vitreous.
- Fundus lesions are not characteristic, although CME and disc edema may be noted.
- -Occasionally, IOP may be elevated because of trabeculitis, blockage of the trabecular
meshwork by debris and cells, or pupillary block

11. .

12. .

13. .
• Prognosis:
• The inflammation usually lasts several days to weeks, up to 3
months.
• Two patterns may occur. In the first type, the attack is acute and
unilateral. Either eye may be affected, but recurrence is rarely
bilateral.
• The second pattern is acute and bilateral.
•

14. .
• Corticosteroids are the mainstay of treatment. Topical corticosteroids,
periocular or oral.
• Corticosteroid-sparing drugs, such as antimetabolites, sulfasalazine,
NSAIDs, and others may be needed for long-term therapy in chronic or
frequently recurrent anterior uveitis.
• Anti-TNF therapy has been effective in recalcitrant anterior uveitis,
particularly in HLA-B27-positive patients.
• Cycloplegic agents
• Admin: topically or with conjunctival cotton pledgets soaked in
tropicamide, cyclopentolate, or phenylephrine hydrochloride

15. .

16. HLA-B27-RELATED DISEASES
• HLA-B27 is a major histocompatibility complex (MHC) class I antigen
• ½ of patients with acute anterior uveitis are HLA-B27 positive.
• Several autoimmune diseases a.k.a seronegative spondyloarthropathies are strongly
associated with both acute anterior uveitis and HLA-B27.
• Patients with these diseases, by definition, do not test positive for rheumatoid factor. The
seronegative spondyloarthropathies include:
-ankylosing spondylitis
-reactive arthritis syndrome
-inflammatory bowel disease
-psoriatic arthritis
• Indistinguishable. F>M

17. ANKYLOSING SPONDYLITIS
• C/O: lower back pain and morning stiffness
• ophthalmologist may be the first physician to suspect AS in an individual patient
• Sacroiliac imaging studies should be obtained
• Refer to rheumatologist; speak of risk of deformity
• Prognosis: Pulmonary apical fibrosis and cardiovascular disease (aortic valvular
insufficiency)
• Rx: NSAIDs; sulfasalazine, anti-TB meds as 2nd line. Exercise, physicals, stop smoking

18. REACTIVE ARTHRITIS
SYNDROME
• Reiter syndrome,
• Classic diagnostic triad of nonspecific urethritis, polyarthritis, and conjunctival
inflammation, often accompanied by nongranulomatous anterior uveitis.
• More in young men
• Triggers: episodes of diarrhea or dysentery without urethritis. Ureaplasma urealyticum
as well as Chlamydia, Shigella, Salmonella, and Yersinia species

19. CLINICAL FEATURES
• c/o: Arthritis begins within 30 days of infection in most pts.
• The knees, ankles, feet, and wrists are affected asymmetrically and in an oligoarticular (4 or
fewer joints) distribution.
• Sacroiliitis is present in as many as 70% of patients.
• Eye involvement occurs in approximately 20%. Mucopurulent conjunctivitis. Punctate non-
granulomatous uveitis in 10% cases.

20. REACTIVE ARTHRITIS SYNDROME WITH PEDAL DISCOID KERATODERMA
BLENNORRHAGICUM

21. INFLAMMATORY BOWEL
DISEASE
• Ulcerative colitis and Crohn disease (granulomatous ileocolitis) are both associated
with acute anterior uveitis.
• Twenty percent of patients with inflammatory bowel disease (IBD) have sacroiliitis; of
these, 60% are HLA-B27 positive.
• Patients with both acute anterior uveitis and IBD are more likely to be HLA-B27
positive and have sacroiliitis.
• Patients with IBD may also develop sclerouveitis, but these individuals are more
commonly HLA-B27 negative.
• HLA-B27-negative IBD patients may also be more likely to develop intermediate uveitis

22. PSORIATIC ARTHRITIS
• Diagnosed according to findings of typical cutaneous changes, terminal phalangeal
joint inflammation and ungual involvement.
• Associations: sacroiliitis, IBD, anterior uveitis.
• The uveitis is more often chronic than is in other spondyloarthropathies.
• Uveitis may be more severe in HLA-B27-positive patients.
• Treatment consists of cycloplegic and mydriatic agents and corticosteroids, which are
usually given topically.
• In severe cases, periocular or systemic corticosteroids may be required, and chronic
cases may need immunomodulatory therapy

23. PSORIATIC ARTHRITIS WITH CLASSIC
ERYTHEMATOUS, HYPERKERATOTIC RASH.

24. PSORIATIC ARTHRITIS WITH SAUSAGE DIGITS RESULTING FROM TISSUE
SWELLING AND DISTAL INTERPHALANGEAL JOINT INFLAMMATION

25. TUBULOINTERSTITIAL NEPHRITIS AND
UVEITIS SYNDROME
• occurs predominantly in adolescent girls and women up to early 30s; mean onset age 21
years.
• Uveitis is typically a bilateral, nongranulomatous, anterior uveitis.
• Ocular symptoms and findings are more severe in patients with recurrent disease, with
development of fibrin, posterior synechiae, larger KPs, and, rarely, hypopyon.
• Posterior segment involvement is atypical but may include mild vitritis as well as optic nerve
and macular edema.
• Patients may present with ophthalmic findings before systemic symptoms and tubulointerstitial
nephritis develop.

26. .• The following criteria are required for a clinical diagnosis of TINU syndrome:
-abnormal serum creatinine level or decreased creatinine clearance
-abnormal urinalysis findings, with increased b2-microglobulin level, proteinuria, presence of
eosinophils, pyuria or hematuria, urinary white cell casts, and normoglycemic glycosuria
-associated systemic illness, consisting of fever, weight loss, anorexia, fatigue, arthralgias,
and myalgias; there may also be abnormal liver function, eosinophilia, and an elevated
erythrocyte sedimentation rate
• The etiology remains unclear.
• Rx: high-dose oral corticosteroids. Immunomodulatory therapy for a few cases

27. GLAUCOMATOCYCLITIC CRISIS
•
a.k.a Posner-Schlossman syndrome,
• usually manifests as a recurrent unilateral, mild, acute nongranulomatous anterior uveitis.
• Symptoms: discomfort, blurred vision, and halos.
• Signs: markedly elevated IOP, corneal edema, KPs that may vary in size and/or distribution,
low-grade cell and flare, and a slightly dilated pupil.
• Episodes last from several hours to several days, and recurrences are common over many
years.
• Treatment: topical corticosteroids and antiglaucoma medication, +/- systemic carbonic
anhydrase inhibitors.
• Recent studies link infection with herpetic viruses such as CMV with glaucomatocyclitic crisis, indicating a possible role for antiviral
therapy

28. LENS-ASSOCIATED UVEITIS
• An immune reaction to lens material may result in ocular inflammatory disease.
• Phacoantigenic: after disruption of lens capsule
• Phacolytic: after leakage of lens protein through the intact lens capsule in mature or
hypermature cataracts
• Mechanism: an immune reaction to lens protein. Patients previously sensitized to lens protein
(eg, after cataract extraction in the fellow eye) can experience inflammation within 24 hours after
capsular rupture

29. .

30. .• PHACOANAPHYLACTIC UVEITIS
It is an immunologic response to lens
proteins in the sensitized eyes presenting
as severe granulomatous anterior uveitis.
• The disease may occur following extracapsular
cataract extraction, trauma to lens or leak of
proteins in hypermature cataract.
• Clinical features. These include severe
pain, loss of vision, marked congestion and
signs of
granulomatous iridocyclitis associated with
presence of lens matter in the anterior
chamber.
• Treatment. It consists of removal of
causative lens matter, topical steroids and
cycloplegics.
• Visual prognosis is usually poor
• PHACOTOXIC UVEITIS
It is an ill-understood entity.
• This term is used to describe mild
iridocyclitis associated with the
presence of lens matter in the anterior
chamber either following trauma or
extracapsular cataract extraction or
leak from hypermature cataracts.
• The uveal response due to direct toxic
effect of lens matter or a mild form of
allergic reaction is yet to be
ascertained.
• Treatment. It consists of removal of
lens matter, topical steroids and
cycloplegics.

31. POSTOPERATIVE INFLAMMATION:
INFECTIOUS ENDOPHTHALMITIS
•
Infectious endophthalmitis must be included in the differential diagnosis of postoperative
inflammation and hypopyon.
• Infection with low-virulence organisms such as Propionibacterium acnes and Staphylococcus
epidermidis as well as fungal species can cause delayed or late-onset endophthalmitis after
cataract surgery

32. POSTOPERATIVE INFLAMMATION: IOL-
ASSOCIATED UVEITIS
• Ranges from mild inflammation to the uveitis-glaucoma-hyphema (UGH) syndrome.
• Surgical manipulation results in breakdown of the blood-aqueous barrier, leading to
vulnerability in the early postoperative period.
• Retained lens material from extracapsular cataract extraction may exacerbate the usual transient postoperative
inflammation.
• Iris chafing caused by the edges or loops of IOLs on either the anterior or the posterior
surface of the iris can result in mechanical irritation and inflammation.
• Iris-supported IOL of AC IOL may cause intermittent corneal touch and lead to corneal endothelial damage or
decompensation, low-grade anterior uveitis, peripheral anterior synechiae, recalcitrant glaucoma, and CME.
• These lenses should be removed and exchanged when penetrating keratoplasty is
performed.

33. PSEUDOPHAKIC BULLOUS KERATOPATHY AND CHRONIC ANTERIOR UVEITIS
CAUSED BY AN IRIS-FIXATED ANTERIOR CHAMBER IOL, WITH CORNEAL TOU CH,
IRIS STROMAL EROSION, AND CHRONIC RECALCITRANT CYSTOID MACULAR
EDEMA

34. UGH AND IOLS
• UGH syndrome may be caused by irritation of the iris root by any intraocular implant.
• Flexible anterior chamber IOLs (ACIOLs) are less likely than older rigid ACIOLs to cause UGH
syndrome.
• Because ACIOL use is rare, UGH syndrome is encountered most commonly with sulcus placement of a single-piece
acrylic IOL.
• UGH syndrome may also occur even with the appropriate placement of a 3-piece IOL in the sulcus.
• Ultrasound biomicroscopy or anterior segment optical coherence tomography (OCT) can be
helpful in evaluating lens position in cases of chronic pseudophakic uveitis.
• Rx: topical corticosteroids; +/- IOL explantation or repositioning.
• As a general rule, the more biocompatible the IOL material, the less likely it is to incite an
inflammatory response.

35. DRUG-INDUCED UVEITIS
• Drugs that induce intraocular inflammation:
-rifabutin (Rx Mycobacterium avium-intracellulare infection),
-systemic fluoroquinolones (especially moxifloxacin),
-bisphosphonates,
-sulfonamides,
-diethylcarbamazine (an antifilarial drug), and oral contraceptives.
• Vaccines such as BCG vaccine, influenza vaccines.
• Purified protein derivative (PPD) used in the tuberculin skin test, have also been implicated in the development
of uveitis.
Topical antiglaucoma medications: metipranolol, anticholinesterase inhibitors, and prostaglandin F2a analogues.
• Drugs that are injected directly into the eye. These medications include antibiotics, urokinase (a plasminogen
activator), cidofovir and anti-VEGF.
• Treatment generally involves topical corticosteroids and cycloplegic drugs, if necessary. Recalcitrant cases may
require cessation or tapering of the offending medication

36. CHRONIC ANTERIOR UVEITIS
• Inflammation of the anterior segment that is persistent and relapses less than 3
months
after discontinuation of therapy;
• it may persist for years.
• This type of inflammation usually starts insidiously, with variable amounts of redness,
discomfort, and photophobia.
• Some patients have no symptoms.
• The disease can be unilateral or bilateral, and the amount of inflammatory activity is
variable.
• CME is common

37. JUVENILE CHRONIC ARTHRITIS (JCA)
• An idiopathic chronic inflammatory arthritis involving multiple joints (knee, elbow, ankle
and interphalangeal joints) in children below the age of 16 years.
• The disease is also referred as Juvenile rheumatoid arthritis, though the patients are
sero-negative for rheumatoid factor.
• In 30 percent cases, polyarthritis is associated with hepatosplenomegaly and other
systemic features, and the condition is labelled as Still’s disease.
• Anterior uveitis associated with JCA is a bilateral (70%), chronic non-granulomatous
disease, affecting female children more than the male (4:1).

38. .
• It usually develops before the age of 6 years.
• Nearly half of the patients are positive for HLA-DW5 and 75 percent are positive for
antinuclear antibodies (ANA).
• The onset of uveitis is asymptomatic and the eye is white even in the presence of severe
uveitis. Therefore,
slit-lamp examination is mandatory in children suffering from JCA.
• Complications like posterior synechiae, complicated cataract and band-shaped keratopathy
are fairly common.
• Treatment is on the usual lines.

39. JUVENILE IDIOPATHIC ARTHRITIS WITH CHRONIC ANTERIOR UVEITIS AND
CATARACT.

40. JUVENILE IDIOPATHIC ARTHRITIS WITH CHRONIC CALCIFIC BAND
KERATOPATHY

41. .

42. FUCHS HETEROCHROMIC UVEITIS
• Fuchs’ heterochromic iridocyclitis is a chronic nongranulomatous type of low grade anterior uveitis.
• A.k.a Fuch’s Uveitis Syndrome (FUS)
• It typically occurs unilaterally in middle-aged persons.
• The disease is characterised by:
(i) heterochromia of iris,
(ii) diffuse stromal iris atrophy,
(iii) fine KPs at back of cornea,
(iv) faint aqueous flare,
(v) Absence of posterior synechiae,
(vi) a fairly common rubeosis iridis, sometimes associated with neovascularization of the angle of
anterior chamber, and
(vii) comparatively early development of complicated cataract and secondary glaucoma (usually open
angle type).
Treatment. Topical corticosteroids are all that is required. Cycloplegics are not required as usually there
are no posterior synechiae

43. .

44. .

45. IDIOPATHIC ANTERIOR UVEITIS
• Inability to positively identify a diagnosis, as is the case in many patients with chronic
anterior uveitis, does not preclude treatment with topical steroids and/or cycloplegics,
assuming infectious causes have been ruled out.
• In some cases initially labeled idiopathic, repeat diagnostic testing at a later date may
reveal an underlying systemic condition

46. INTERMEDIATE UVEITIS (PARS
PLANITIS
• It denotes inflammation of pars plana part of ciliary body and most peripheral part of
the retina.
• Etiology. It is an idiopathic disease usually affecting both eyes (80 percent) of children
and young adults.
• Pars planitis is a rather common entity, constituting 8 percent of uveitis patients.

47. .
• Clinical features:
• Symptoms. Most of the patients present with history of floaters. Some patients may
come with defective vision due to associated cystoid macular oedema.
• Signs.
• The eye is usually quiet. Slit-lamp examination may show: mild aqueous flare, and
KPs at the back of cornea.
• Anterior vitreous may show cells.
• Fundus examination with indirect ophthalmoscope reveals the whitish exudates
present near the ora serrata in the inferior quadrant.
• These typical exudates are referred as snow ball opacities. These may coalesce to
a grey white plaque called snow banking.

48. .
• Complications of long-standing pars planitis include:
-cystoid macular oedema,
-complicated cataract and
-tractional retinal detachment.
• Treatment
1. Corticosteroids administered systemically and a repeated periocular injections may
effective in some cases.
2. Immunosuppressive drugs may be helpful in steroid resistant cases.
3. Peripheral cryotherapy is also reported to be effective.

49. .

50. DDX OF PARS PLANITIS
• syphilis,
• tuberculosis,
• Lyme uveitis,
• sarcoidosis,
• intermediate uveitis associated with MS, and
• toxocariasis.

51. COMPLICATIONS OF PARS PLANITIS
• cataract,
• glaucoma,
• CME,
• retinal neovascularization,
• vitreous hemorrhage, and
• tractional or rhegmatogenous retinal detachment.

52. DDX: OF POSTERIOR UVEITIS
• INFECTIOUS:
-Bartonella (cat scratch disease)
-Syphilis
-Lyme
-Toxoplasmosis
-TB
-HIV
-Herpes viruses
• NON-INFECTIOUS
-Pars planitis
-MS
-SLE
-Sarcoidosis
-Behcet’s dz
-Eale’s dz
-Other systemic inflammatory
conditions:
RF/GPA/Crohns dz/polyarteritis
nodosa/relapsing chrondritis
-Idiopathis

53. WORKUP
• CBC
• Cre
• Urinalysis
• ACE
• ESR
• CRP
• RF
• ANA
• TB: PPD, CXR
• Toxoplasmosis
• Syphilis
• HIV
• Lyme
• Bartonella henselae

54. POSTERIOR UVEITIS
• Posterior uveitis refers to inflammation of the choroid (choroiditis).
• Since the outer layers of retina are in close contact with the choroid and also depend on it
for the nourishment, the choroidal inflammation almost always involves the adjoining retina,
and the resultant lesion is called chorioretinitis.
• Etiology and pathology: These are same as described for uveitis in general
considerations.
• Clinical types
I. Suppurative choroiditis (Purulent inflammation of the choroid). It usually does not occur
alone and
almost always forms part of endophthalmitis

55. II. NON-SUPPURATIVE CHOROIDITIS.
• It may be nongranulomatous or granulomatous (more common).
• Non-suppurative choroidal inflammation is characterised by exudation and cellular
infiltration, resulting in a greyish white lesion hiding the normal reddish hue of choroidal
vessels.
• Non-suppurative choroiditis is usually bilateral and morphologically (depending upon
the number and location of lesions) can be classified into diffuse, disseminated and
circumscribed (localised) choroiditis.

56. .• 1. Diffuse choroiditis. It refers to large spreading lesions involving most of the choroidal
tissue. It is usually tubercular or syphilitic in origin.
• 2. Disseminated choroiditis. It is characterised by multiple but small areas of
inflammation scattered over the greater part of choroid.
Such a condition may be due to syphilis or tuberculosis, but in many cases the cause is
obscure.
•
3. Circumscribed/localised/focal choroiditis. It is characterised by a single patch or a
few small patches of inflammation localised in a particular area.
Such patches of choroiditis are described by a name depending upon the location of the
lesion which are as follows:

57. .
• i. Central choroiditis. As the name indicates it involves the macular area and may occur
either
alone or in combination with disseminated choroiditis.
• A typical patch of central choroiditis may occur in toxoplasmosis, histoplasmosis,
tuberculosis, syphilis and rarely due to visceral larva migrans.
• ii. Juxtacaecal or juxtapapillary choroiditis. It is the name given to a patch of choroiditis
involving an area adjoining the optic disc.
• One example is Jensen’s choroiditis which typically occurs in young persons.

58. .
• iii. Anterior peripheral choroiditis. It implies occurrence of multiple small patches of
choroiditis (similar to disseminated choroiditis) only in the peripheral part of choroid
(anterior to equator).
•
Such lesions are often syphilitic in origin.
• iv. Equatorial choroiditis. It involves the choroid in the equatorial region only.

59. CLINICAL PICTURE
• Symptoms.
• Choroiditis is a painless condition, usually characterised by visual symptoms due to
associated vitreous haze and involvement of the retina.
• Therefore, small patches situated in periphery may be symptomless and are usually
discovered as healed patches on routine fundus examination.
• On the contrary, a central patch produces marked symptoms which draw immediate
attention. Various visual symptoms experienced by a patient of choroiditis are
summarised below:

60. .1. Defective vision. It is usually mild due to vitreous haze, but may be severe as in
central choroiditis.
2. Photopsia. It is a subjective sensation of flashes of light resulting due to irritation of
rods and
cones.
3. Black spots floating in front of the eyes. It is a very common complaint of such
patients. They
occur due to large exudative clumps in the vitreous.
4. Metamorphopsia. Herein, patients perceive distorted images of the object. This results
due
to alteration in the retinal contour caused by a raised patch of choroiditis.

61. .
• 5. Micropsia which results due to separation of visual cells is a common complaint. In
this the
objects appear smaller than they are.
• 6. Macropsia, i.e., perception of the objects larger than they are, may occur due to
crowding together of rods and cones.
•
7. Positive scotoma, i.e., perception of a fixed large spot in the field of vision,
corresponding to the lesion may be noted by many patients

62. SIGNS
• Usually there are no external signs and the eye looks quiet. However, fine KPs may be
seen on biomicroscopy due to associated cyclitis. Fundus examination may reveal
following signs:
1. Vitreous opacities due to choroiditis are usually present in its middle or posterior
part. These may be fine, coarse, stringy or snowball opacities.
2. Features of a patch of choroiditis.
i. In active stage it looks as a pale-yellow or dirty white raised area with ill-defined
edges.
This results due to exudation and cellular infiltration of the choroid which hide the
choroidal vessels.
The lesion is typically deeper to the retinal vessels.
The overlying retina is often cloudy and oedematous.

63. .
• ii. In atrophic stage or healed stage, when active inflammation subsides, the affected
area
becomes more sharply defined and delineated from the rest of the normal area.
• The involved area shows white sclera below the atrophic choroid and black pigmented
clumps at the periphery of the lesion.
• A healed patch of chorioretinitis must be differentiated from the degenerative
conditions
such as pathological myopia and retinitis pigmentosa.

64. COMPLICATIONS
• extension of the inflammation to anterior uvea,
• complicated cataract,
• Vitreous degeneration,
• macular oedema,
• Secondary periphlebitis retinae and
• retinal detachment.

65. TREATMENT
• It is broadly on the lines of anterior uveitis.
• 1. Non-specific therapy consists of topical and systemic corticosteroids.
Posterior sub-tenon injections of depot corticosteroids are effective in checking the acute
phase of posterior uveitis.
Rarely, immunosuppresive agents may be required to check the inflammation.
• 2. Specific treatment is required for the causative disease such as toxoplasmosis,
toxocariasis,
tuberculosis, syphilis, etc.

66. .

67. .

68. POLYARTERITIS NODOSA
• An idiopathic aneurysmal vasculitis affecting medium-sized and small arteries, with a
wide range of manifestations across multiple organ systems.
• Presentation is in the third to sixth decades, often with constitutional symptoms.
• The male : female ratio is about 3 : 1.
• Ocular involvement may precede the systemic manifestations by several years.
• About a third of patients have hepatitis B infection.
• Scleritis is often aggressive and necrotizing. Peripheral ulcerative keratitis, orbital
pseudotumour and occlusive retinal periarteritis are other reported ocular features

69. .

70. BIRDSHOT RETINOCHOROIDOPATHY
•Introduction
Birdshot retinochoroidopathy is an uncommon idiopathic chronic bilateral disease
predominantly affecting middle-aged women.
Clinical features
• Symptoms. Insidious impairment of central vision associated with photopsia and
floaters.
• Vitritis is prominent

71. .
• Fundus. Multiple ill-defined ovoid cream-coloured choroidal patches, less than one
disc diameter in size, in the posterior pole and midperiphery.
• The lesions often appear to radiate outward from the disc but usually spare the macula
itself. Inactive lesions consist of welldelineated atrophic spots.
• CMO, epiretinal membrane and CNV may develop
• Prognosis. About one-third of patients have an eventual best visual acuity of less than
than 6/60

72. .
• Investigation
• HLA-A29: over 95% of patients are positive.
• OCT will confirm macular oedema

73. .
• Autofluorescence will generally show more numerous hypoautofluorescent lesions
than are seen on examination.
• FA shows extensive vascular leakage, with disc and vessel staining and CMO lesions are
initially hypofluorescent with later staining.
• ICGA: lesions are more numerous; they are hypofluorescent during the early and
intermediate phases and isofluorescent later.
• ERG is normal in early disease but with time shows rod and cone abnormalities

74. TREATMENT
• Systemic and intraocular steroids are reasonably effective in many patients, as are a
variety of immunosuppressants and biological blockers such as infliximab.

75. .

76. .

77. ACUTE POSTERIOR MULTIFOCAL PLACOID
PIGMENT EPITHELIOPATHY (APMPPE)
APMPPE is an uncommon idiopathic inflammatory disorder.
• Affects young to middle-aged adults of both genders equally.
• There is often a viral prodrome, and it is speculated to occur as a result of cell-mediated immunity to
viral antigen.
• Associated cerebral vasculitis is relatively common and can cause stroke.
• Erythema nodosum and other systemic manifestations of vasculitis have been reported. The clinical
picture of APMPPE can be mimicked by other entities such as sarcoidosis and tuberculosis.

78. .
• Clinical features
• Symptoms. Subacute moderate visual impairment; central/paracentral scotomata;
photopsia is frequent.
The fellow eye is affected within a few days or weeks.
Headache and other neurological symptoms are common and can commence many months
after ocular disease onset.
• Anterior uveitis and vitritis are usually very mild.
• Fundus. Multiple large deep yellow–white placoid lesions, initially at the posterior pole.
Within weeks the majority fade, with residual RPE disturbance of varying severity.
Subretinal macular fluid may be seen. Vasculitis and papillitis are rare.
• Prognosis. In 25% of patients recovery is to only 6/15 or worse, with recurrence in up to 50%

79. APMPPE
• Investigation
• Alternative diagnoses should be excluded.
• HLA-B7 and HLA-DR2 are associated in a substantial proportion of patients.
• OCT for macular assessment.
• FA of active lesions shows early dense hypofluorescence and late staining.
• ICGA demonstrates non-perfusion of the choriocapillaris
• CNS imaging and lumbar puncture should be performed in patients with neurological
symptoms.
• Treatment
• Steroids should be considered, especially for macular involvement.
• Steroids and possibly ciclosporin may be given for cerebral vasculitis. Patients should be
instructed to seek medical advice urgently if neurological symptoms occur

80. .

81. .

82. .

83. SERPIGINOUS CHOROIDOPATHY
• usually bilateral, though asymmetrical.
• typically occurs in middle age, M>F and is assoc. with HLA-B7.
• The disease is generally recurrent over years, with a relatively poor prognosis. TB uveitis can give a
similar clinical picture (‘serpiginoid’).
Clinical features
• Symptoms. Initially unilateral blurring of central vision, scotoma or metamorphopsia.
• Anterior uveitis and vitritis are common but usually mild.
• Fundus. Active lesions are grey–white and may remain active for several months before becoming
scalloped and atrophic. The disease typically starts around the optic disc and spreads centrifugally.
Recurrence results in extensive chorioretinal atrophy
• Complications. CNV (15–35%), subretinal fibrosis, preretinal neovascularization.
Rx: steroids + immunosuppresants;
Ix: FA, ICGA… hypofluoro

84. .

85. .

86. MULTIFOCAL CHOROIDITIS AND
PANUVEITIS (MFC, MCP)
• Introduction
MCP is an uncommon, usually bilateral but asymmetrical, chronic/recurrent disease that typically affects
young and middle-aged adult females. Severity and prognosis are very variable.
• Along with PIC and SFU, it is sometimes termed pseudo-POHS.
Clinical features
• Symptoms. Blurring, floaters and photopsia.
• Anterior uveitis (50%).
• Vitritis.
• Fundus. Multiple discrete, ovoid, yellowish-grey lesions 50–350 µm in diameter at the posterior pole
and/or
periphery, sometimes with linear clusters and/or streaks.

87. .
• Inactive lesions have sharply defined margins and pigmented borders
resembling POHS. Peripapillary atrophy may be seen.
• The course is prolonged with the development of new lesions and recurrent
inflammatory episodes. CNV occurs in 25–35%, CMO and subretinal fibrosis
resembling SFU can develop.
• Optic disc oedema and blind spot enlargement may be present.
Investigation
• Visual fields may show large defects not corresponding with examination
findings.
• FA. Early hypofluorescence and late hyperfluorescence. Old inactive lesions
show window defect

88. .
• ICGA shows hypofluorescent acute lesions which may not be clinically
apparent. Old lesions remain hypofluorescent throughout.
• ERG remains normal until there is advanced retinal atrophy.
• Treatment
Systemic and local steroids. Steroid-resistant patients require
immunosuppressive therapy. CNV is treated with steroids and anti-
VEGF agents

89. .

90. .

91. .

92. PUNCTATE INNER CHOROIDITIS
• Introduction
PIC typically affects young myopic women. Both eyes are frequently
sequentially involved.
• It has similarities with MCP but involvement is predominantly macular. It is
sometimes categorized with MCP as ‘pseudo-POHS’.
• Clinical features
• Symptoms. Blurring, floaters and photopsia.
• Anterior uveitis and vitritis are usually absent or very mild.
• Fundus. Several small yellow–white macular spots with fuzzy borders at
the level of the inner choroid and retina, sometimes with an overlying serous
sensory retinal detachment.
• These evolve into sharply demarcated atrophic scars with little pigmentation, similar to the histo spots of POHS.

93. .
• • Prognosis. Guarded; central vision may be compromised by a lesion at the fovea or,
commonly, by CNV (up to 40%)
• Investigation
FA shows early hyperfluorescence and late staining of lesions, and demonstrates CNV.
• Treatment
This is usually reserved for CNV, though steroids may be considered for a foveal
lesion.

94. .
• .

95. .

96. PROGRESSIVE SUBRETINAL FIBROSIS
AND UVEITIS SYNDROME (SFU)
• SFU, also known as diffuse subretinal fibrosis, is an extremely rare chronic
condition.
• It typically affects myopic young women, causing gradual blurring of vision in
one then both eyes.
• Anterior uveitis and vitritis accompanies subretinal mounds at the posterior
pole and midperiphery progressing to widespread subretinal fibrosis.
• Steroids may be effective early in the disease, but the prognosis is poor. Some
experts view SFU as part of a spectrum with MFC/MCP and PIC

97. .

98. .

99. .

100. MULTIPLE EVANESCENT WHITE DOT
SYNDROME
• Introduction
MEWDS is an uncommon idiopathic disease typically occurring in young adult females; 25–50%
describe a preceding viral-like illness.
• Subsequent progression to AZOOR has been reported in some patients.
• Clinical features
• Presentation. Common: painless monocular blurring (6/9–6/60) and photopsia; less common:
floaters, scotomata (microscotoma), dyschromatopsia.
• Subtle posterior vitritis in 50%. But usually no anterior segment inflammation
• Posterior pole lesions. Numerous small (100–300 µm) ill-defined deep grey-white patches
sparing the fovea, which has a characteristic orange granular appearance and a dulled reflex. At
the level of the outer retina and the RPE.
A mild afferent pupillary defect also reported
• Optic disc oedema is occasionally present
• • Recovery occurs over weeks, often leaving subtle residual signs. Recurrence occasionally (10%)
occurs.

101. INVESTIGATION
• Visual fields. The blind spot may be enlarged; may show other scotomata.
• OCT may show inner-segment/outer-segment junction disruption. demonstrates defects located at the
level of the outer retina and RPE
• FAF. Hyper-autofluorescent spots corresponding to the macular lesions are visible during active
inflammation; FAF has been used to demonstrate subclinical lesions in patients with only foveal
granularity. These spots evolve over time and may persist or fade. The lesions are more numerous than
clinically seen
• FA shows subtle early hyperfluorescence of the dots with late staining; occasionally vessel wall
and disc staining may be seen.
• ICGA shows hypofluorescent spots that are often more numerous than visible clinically or on FA.
Interrmediate transient pinpoint hyperfluorescence
• ERG shows a transiently reduced a-wave amplitude.
Electrooculography (EOG) and visual evoked response (VER) abnormalities may be present.

102. TREATMENT
• This is generally not required, except for rare cases complicated by choroidal
neovascularization.
• Initial approach is observation

103. .

104. .

105. .

106. THE WHITE DOT SYNDROMES

107. ACUTE RETINAL PIGMENT EPITHELIITIS
• ARPE (Krill disease) is a rare, idiopathic, self-limited condition of the RPE; it is unilateral in 75% of
cases.
• The etiology of A RPE is unclear, although a viral etiology is suspected. Hep C, and bisphosphonates
• Presentation is in young adults with mild disturbance of central vision;
• 1–2 weeks after the onset of symptoms the macula shows 2–4 discrete clusters of subtle small (one-
fourth disc diameter) grey spots at the level of the RPE, surrounded by hypopigmented yellow
haloes. Key word ‘discrete tiny clusters’
• Over 6–12 weeks the lesions resolve and vision returns to normal.
• Recurrences are uncommon.
• OCT shows hyper-reflectivity at the photoreceptor outer segment layer.
• FA may be normal, or the spots may show a hypofluorescent centre with a hyperfluorescent halo.

108. .

109. .

110. ACUTE ZONAL OCCULT OUTER
RETINOPATHY (AZOOR)
• A group of rare conditions. initially described by Don Gass, MD, acute zonal occult outer retinopathy (AZOOR) is a syndrome of outer
retinal dysfunction associated with visual field and ERG abnormalities, which is most common in women in their fourth decade of life
• characterized by acute onset of loss of one or more zones of visual field, often temporal, in one or both eyes of young or middle-aged
females, some of whom have an antecedent viral-like illness;
• photopsia and mild vitritis are frequent, and subtle vasculitis is sometimes seen.
• The mechanism is undefined as yet. Dr. Gass postulated an infectious cause. (Hep B). Others say it comes after other white dot
syndromes
• Acute zonal occult outer retinopathy (AZOOR) is the most common of the AZOR syndromes.
• c/o: photopsias, acute progressive VF loss, typically bilateral, VA variably affected
• O’/e: RAPD, VITRITIS, RPE atrophy/ abnormal RPE pigmentation
• It’s characterized by minimal fundoscopic signs early in the disease course; the other postulated members of the group have more
evident findings.

111. .• Field loss may progress; in 50% stabilization occurs within 6 months but recovery is
infrequent.
• Later findings include RPE clumping and vascular attenuation in the involved area and
peripapillary region, although the fundus may remain normal.
• OCT, FA, ICGA and FAF may demonstrate abnormalities.
• ERG is important for diagnosis, characteristically showing a-wave and b-wave
amplitude reduction and delayed 30 Hz flicker –that is, cones tend to be affected more
than rods.
• EOG shows absence or severe reduction of the light rise.

112. .

113. .

114. BEHCET DISEASE (BD)
• Chronic, relapsing, occlusive systemic vasculitis of unknown etiology
• Uveitis that can affect both anterior and posterior segment of the age
• The diagnosis of B.D is clinical and is based on the presence of multiple systemic
findings
• Recurrent and aphthous ulcer is the most common consistent feature of Behcet’s dz
• If you are considering Behcet’s dz and the patient lacks oral ulcer; you better think
twice!!!
• Etiology. It is still unknown; the basic lesion is an obliterative vasculitis probably
caused by circulating immune complexes.
• The disease typically affects the young men who are positive for HLA-B51. m>f

115. .

116. CLINICAL FEATURES
• Uveitis seen in Behcet’s disease is typically bilateral, acute recurrent iridocyclitis associated with
hypopyon.
• It may also be associated with posterior uveitis, vitritis, periphlebitis retinae and retinitis in the form of
white necrotic infiltrates

118. .

119. INVESTIGATIONS

120. .

121. TREATMENT
• No satisfactory treatment is available, and thus the disease has got
comparatively poor visual prognosis.
• Corticosteroids may by helpful initially but ultimate response is poor. In
some cases the disease may be controlled by chlorambucil.

122. .

123. .

124. .

125. .

126. .

127. PANUVEITIS
• Panuveitis (or diffuse uveitis) requires involvement of all anatomical compartments of the eye--
namely, the anterior chamber, vitreous, and retina or choroid-with no single predominant site of
inflammation.
• Generally, panuveitis is bilateral, although 1 eye may be affected first and the severity is not
necessarily symmetric.
• The discussion of panuveitis in this chapter is limited to the noninfectious entities

128. SARCOIDOSIS
• Sarcoidosis is a multi-system disease of unknown etiology, characterised by formation of noncaseating
epithelioid cell granuloma in the affected tissue.
• The disease typically affects young adults, frequently presenting with bilateral hilar lymphadenopathy,
pulmonary infiltration, skin and ocular lesions.
• Ocular lesions occur in 20-50 percent patients and include: uveitis, vitritis with snowball opacities in
inferior vitreous, choroidal and retinal granulomas, periphlebitis retinae with ‘candle wax droppings',
conjunctival sarcoid nodule and keratoconjunctivitis sicca.
• Mutton fat’ KPs and/or small granulomatous KPs and/or iris nodules (Koeppe and/or Busacca ).
Trabecular meshwork (TM) nodules and/or tent-shaped PAS

129. .

130. .

131. .

132. .

133. .

134. .

135. .

136. CLINICAL TYPES
• Sarcoid uveitis accounts for 2 percent cases of uveitis. It may present as one of the following:
1. Acute iridocyclitis (non-granulomatous). It is frequently unilateral, associated with acute sarcoidosis
characterised by hilar lymphadenopathy and erythema nodosum.
2. Chronic iridocyclitis. It is more common than acute and presents with typical features of bilateral
granulomatous iridocyclitis. The disease is often seen in association with chronic sarcoidosis
characterised by pulmonary fibrosis.
3. Uveoparotid fever (Heerfordt’s syndrome). It is characterised by bilateral granulomatous panuveitis,
painful enlargement of parotid glands, cranial nerve palsies, skin rashes, fever and malaise.

137. DIAGNOSIS.
• Once suspected clinically, it is supported by positive Kveim test, abnormal X-ray chest (in 90
percent cases) and raised levels of serum angiotensin converting enzyme (ACE).
• Confirmation of the disease is made by histological proof from biopsy of the conjunctival nodule,
skin lesions or enlarged lymph node.
• Treatment. Topical, periocular and systemic steroids constitute the treatment of sarcoid uveitis,
depending
upon the severity.

138. SYMPATHETIC OPHTHALMIA
• Introduction
Sympathetic ophthalmitis (SO) is a bilateral granulomatous panuveitis occurring after penetrating
trauma; uveal prolapse may have been a feature of the trauma.
• Less frequently the condition occurs following intraocular surgery, usually multiple vitreoretinal
procedures.
• Presentation in trauma-induced cases is between 2 weeks and 3 months after initial injury in 65%.
• The incidence is probably 0.2–0.5% after injury and 0.01% following intraocular surgery.
• Histopathology shows a diffuse lymphocytic infiltration of the choroid. Scattered aggregates of
epithelioid cells are seen, many of which contain fine granules of melanin.
• Dalen–Fuchs nodules, which also occur in Vogt–Koyanagi–Harada syndrome are granulomas
located between Bruch membrane and the RPE

139. CLINICAL FEATURES
• Symptoms. There is a history of causative trauma; the exciting eye is frequently red and irritable. The
sympathizing eye develops irritation, blurred vision, photophobia and loss of accommodation.
• Anterior uveitis develops in both eyes; this may be mild or severe and is usually granulomatous. The
severity of inflammation may be markedly asymmetrical.
• Fundus: multifocal choroidal infiltrates develop in the midperiphery, with sub-RPE infiltrates
corresponding to Dalen–Fuchs nodules.
Exudative retinal detachment, vasculitis and optic disc swelling may all manifest.
As inflammation settles, residual chorioretinal scarring may confer a ‘sunset glow’ appearance similar to
VKH.

140. .
• Systemic manifestations similar to those in VKH can occur but are uncommon.
• Prognosis depends on the severity and location of disease and the response to treatment.
With aggressive therapy 75% of sympathizing eyes retain a visual acuity of better than 6/60.
Long-term follow-up is mandatory because relapses occur in 50% of cases, and may be delayed
for several years.

141. INVESTIGATION
• OCT is useful for quantifying and monitoring change.
• B-scan ultrasonography may demonstrate choroidal thickening.
• FA shows multiple foci of leakage at the level of the RPE, with subretinal pooling in the
presence of exudative retinal detachment.
• ICGA shows hypofluorescent spots in active disease, which resolve with treatment.
• Ultrasound may show choroidal thickening and exudative retinal detachment.

142. TREATMENT
Enucleation of a severely injured eye in the first week or so following injury has historically been
considered effective in preventing or reducing the severity of SO, but there is some evidence that little
useful effect is exerted, particularly with modern standards of surgical repair.
It may be considered for an injured eye with a hopeless visual prognosis.
Evisceration has conventionally been viewed as inadequate, though recent evidence has raised the
possibility of a protective effect provided all uveal tissue is removed.

143. .
• Steroids are the basis of treatment. High dose oral prednisolone is given for several
months, and gradually tapered according to response. Initiation with intravenous
methylprednisolone may be used in some cases.
Supplementary topical steroids and cycloplegics may be given to target anterior uveitis,
and peri- and intraocular steroids, including slow-release intravitreal implants, may
facilitate reduced systemic treatment.
• Immunosuppressives such as azathioprine, ciclosporin and methotrexate can be
used in resistant cases or as steroidsparing agents.
Biological blockers (e.g. infliximab, adalimumab) may be considered.

144. .

145. .

146. SYMPATHETIC OPHTHALMIA: PHOTOGRAPH SHOWS SYMPATHIZING EYE WITH
SYNECHIAE

147. .

148. .

149. .

150. VOGT-KOYANAGI-HARADA SYNDROME
• It is an idiopathic multisystem disorder which includes cutaneous, neurological and ocular lesions.
• The disease is comparatively more common in Japanese who are usually positive for HLA-DR4 and
DW15.
• Clinical features
1. Cutaneous lesions include: alopecia, poliosis and vitiligo.
2. Neurological lesions are in the form of meningism, encephalopathy, tinnitus, vertigo and deafness.
3. Ocular features are bilateral chronic granulomatous anterior uveitis, posterior uveitis and exudative
retinal detachment.
• Treatment. It comprises steroids administered topically, periocularly and systemically

151. .

152. .

153. .

154. .

155. .

156. .

157. .

158. .

159. REFERENCE
• AAO, BK 9;
• Kanski
• Khuranah
• Curbside Consults: Uveitis
• UoT 2020 Webinars