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INTRODUCTION TO UVEITIS
DR SHRUTI LADDHA
• Inflammation of the uveal tract comprised by the iris, ciliary body, and
choroid.
• Infections of the eye, autoimmune disorders, trauma to the eye,
certain medications which can incite ocular inflammation, and rarely
malignancies
EPIDEMIOLOGY
• 10 % blindness worldwide
• Prevalence-115-204 /lac
• Incidence-17-52/lac
• Mean age-40 yrs
• M=F
SYMPTOMS
• dependent upon the anatomic location of the inflammation in the eye, rapidity of
onset of the inflammation, and duration and course of disease.
• Anterior segment (ocular injection, light sensitivity, pain, blurry vision, epiphora)
• Intermediate and posterior segment (floaters, flashing lights, blurry vision)
SIGNS
ANTERIOR SEGMENT
• Cells
• Flare
• Fibrin
• Hypopyon
• Synechiae (both anterior and posterior)
• Iris nodules
• Iris atrophy
• Keratic precipitates
• Band keratopathy
INTERMEDIATE SEGMENT
• vitreal cells
• “snowball” opacities in the vitreous
• exudates over the pars plana (snowbanking)
• neovascularization of the pars plana
POSTERIOR SEGMENT
• Vascular sheathing (arteries, veins, or both)
• Retinal pigment epithelial hypertrophy or atrophy
• Cystoid macular edema
• Atrophy or swelling of the retina, choroid, or optic nerve head
• Exudative, tractional, or rhegmatogenous retinal detachment
• Retinal or choroidal neovascularization
CLASSIFICATION
The classification of uveitis is important for multiple reasons that are as follows:
• The location of ocular inflammation may assist in diagnosis or at least narrow the
potential etiologies (e.g., Fuch’s heterochromic iridocyclitis involves the anterior chamber;
ocular toxoplasmosis primarily effects the retina with significant inflammatory spillover
into the choroid and vitreous)
• Uveitis may be a manifestation of an underlying serious or potentially lethal systemic
disease. The correct diagnosis can be sight and on occasion life-preserving.
• Uveitis may be caused by a vast number of conditions including infections, autoimmune
disorders, medication induced, traumatic, and neoplastic. The correct characterization of
the ocular manifestations may assist in identifying an underlying etiology.
The Standardization of Uveitis Nomenclature (SUN) Working Group guidance on uveitis
terminology, endorsed by the International Uveitis Study Group (IUSG), categorizes uveitis
anatomically
Anterior • Iritis
• Iridocyclitis
• Anterior cyclitis
Intermediate • Pars planitis
• Posterior cyclitis
• Hyalitis
Posterior • Choroiditis (focal, multifocal, diffuse)
• Chorioretinitis
• Retinochoroiditis
• Retinitis
• Neuroretinitis
Panuveitis
THE SUN WORKING GROUP
GRADING SCHEME FOR ANTERIOR CHAMBER CELLS
Grade Cells per field
0 <1
0.5+ 1-5
1+ 6-15
2+ 16-25
3+ 26-50
4+ >50
To grade cells and flare,
the slit lamp is set to
maximum intensity and
both the width and length
of the beam at 1mm.
THE SUN WORKING GROUP
GRADING SYSTEM FOR ANTERIOR CHAMBER FLARE
Grade Description
0 Complete absence
+ Faint, barely detectable
++ Moderate - iris and lens details clear
+++ Marked—iris and lens details hazy
++++ Intense--fixed coagulated aqueous with fibrin
Category Descriptor Comment
Onset
Sudden
Insidious
Duration
Limited
Persistent
≤3 months’ duration
>3 months’ duration
Course Acute
Recurrent
Chronic
Episode characterized by sudden onset and limited duration
Repeated episodes separated by periods of inactivity without
treatment ≥3 months duration
Persistent uveitis with relapse in <3 months after discontinuing
treatment
THE SUN WORKING GROUP DESCRIPTORS IN UVEITIS
Term Definition Comment
Inactive Grade 0 cells (anterior chamber)
Worsening activity
2-step increase in level of inflammation
(eg, anterior chamber cells, vitreous haze) or
increase from grade 3+ to 4+
Improved activity
2-step decrease in level of inflammation
(eg, anterior chamber cells, vitreous haze) or
decrease to grade 0
≤3 months’ duration
Remission
Inactive disease for ≥3 months after
discontinuing all treatments for eye disease >3 months’ duration
THE SUN WORKING GROUP ACTIVITY OF UVEITIS TERMINOLOGY
FEATURES OF GRANULOMATOUS AND NON GRANULOMATOUS UVEITIS
Features Granulomatous Non Granulomatous
Onset Insidious Acute
Course Chronic Short (may be recurrent)
Circumcorneal congestion + +++
Pain + +
Iris nodule +++ -
KP’s Large mutton fat Small or medium
Flare + +++
Posterior Segment Commonly involved Rarely involved
Vitreous Heavy exudates Fine opacities
Nodular lesions Diffuse involvement with edema
Clinical classification
a. Infectious:
i. Bacterial-TB, Syphilis, leprosy
ii. Viral-HSV, HZV, CMV
iii. Fungal-POHS, pneumocystis, Candida, Aspergillus
iv. Parasitic-Toxoplasma ,Toxocara, Cystecercosis
b. Non-infectious:
i. Known systemic association
ii. No known systemic association
iii. Masquerade:
Pathological Classification
i. Granulomatous and non-granulomatous
ii. Suppurative and exudative
Etiological Classification
• Infectious
i. Exogenous: Staphyloccous, Pseudomonas, Propionibacterium acnes.
• Secondary—Iridocyclitis associated with herpetic keratitis, iridocyclitis associated with
anterior and posterior scleritis
ii. Endogenous:
• Bacterial - TB, Syphilis
• Viral - Herpes simplex, CMV, Measles, Influenza
• Fungal- Histoplasmosis, Candidiasis
• Parasitic – Toxoplasmosis, Toxocariasis, Onchocerciasis, Pneumocystis carinii
Hypersensitivity/autoimmune
• Lens induced—autoimmune reaction to lens protein
• Sympathetic ophthalmia—autoimmunity to uveal pigment.
• VKH
• Behcet’s suspected autoimmune origin
Associated with Systemic Conditions
• Ankylosing spondylitis (AS)
• Rheumatoid arthritis (RA)
• Juvenile rheumatoid arthritis (JRA)
• Psoriatic arthritis
• Associated with GIT disorders: Ulcerative colitis
• Associated with respiratory system : Sarcoidosis, leprosy, TB.
Idiopathic
• i. Specific—Fuch’s
• ii. Nonspecific—Account for 25% of all uveitis.
Associated with Neoplasms
• Retinoblastoma, choroidal melanoma.
Non-infective systemic diseases
• Sarocoidosis
• Polyarteritis nodosa (PAN)
• Disseminated lupus erythematosus (DLE)
• Diseases of skin (psoriasis, lichen planus, erythema nodosum, pemphigus).
Traumatic uveitis
accidental or operative injuries to the uveal tissue
HISTORY TAKING
Questionnaire by Dr
Stephen Foster
IOP-
• high in 42 % cases
• Diseases thought to have a higher rate of ocular hypertension include Fuch’s heterochromic
iridocyclitis (FHIC), glaucomatocyclitic crisis or Posner-Schlossman syndrome, sarcoidosis,
juvenile rheumatoid arthritis, VKH, toxoplasmosis, and herpetic keratouveitis.
KERATIC PRECIPITATES-
• helpful in defining between acute versus chronic inflammation, and based on the appearance,
may also give clues into the pathogenesis
• Fine precipitates - more common in spondyloarthropathies and juvenile arthropathies.
• Stellate precipitates seen involving the superior cornea (as opposed to the typical inferior
corneal base down triangular appearance of most precipitates) are often seen with Fuch’s
heterochromic iridocyclitis.
• Mutton fat” precipitates are larger and are formed from macrophages and epithelioid cells.
These may be indicative of a granulomatous disease
Granulomatous kps non granulomatous kps
HYPOPYON-
• enough cells(leucocytes) as well as fibrin to clump it
• The most common etiologies include infectious (both bacterial and viral), HLA-B27
associated uveitis, and Behcet’s disease.
• With infectious endophthalmitis the patient will typically have a history of recent surgery,
trauma, or have risk factors for endogenous infection (e.g.,intravenous drug use)
• Behcets disease- shifting hypopyon
• Pseudohypopyon, composed of tumor cells and debris can occur in some of the
masquerade syndromes.
Iris Changes-
• Sectoral atrophy- HSV, HZV, CMV (if associated with raised IOP ,should arise the suspicion
of Herpes)
• Nodules on iris-accumulation of inflammatory cells on or within the iris –more common
in granulomatous disease
• Heterchromia- Fuchs disease
Retinal/Choroidal findings
• VKH- Serous retinal detachments ,Dalen-Fuchs nodules (small, discrete, deep, yellow-
white chorioretinal lesions) may be associated with VKH and sympathetic ophthalmia.
• Acute retinal necrosis (ARN) is a type of necrotizing retinitis most commonly caused by
herpetic viruses (HSV, VZV). The classic posterior appearance includes vitritis, retinal
vascular arteriolitis, and peripheral retinitis. Typically, the retinitis begins as peripheral
areas of multifocal retinal yellowing, often flat with scalloped edges. This can eventually
progress into confluent whitening extending into the posterior pole.
• Classic toxoplasmosis lesions present as focal and white with overlying vitritis with a
“headlight in the fog” appearance, often with adjacent pigmented retinochoroidal
scarring.
• Cytomegalovirus (CMV) retinitis may also be identified clinically and should be
suspected in patients who are immunosuppressed.
The classic exam findings - peripheral or posterior yellow-white lesions that
follow the retinal vasculature centripetally, vasculitis with a “frosted branch”
appearance, and retinal hemorrhages. This constellation of findings has been
described as a “scrambled eggs or cottage cheese with ketchup” appearance.
There may be little to no vitritis, given the immunocompromised state of
these patients.
• Optic Nerve—Disc hyperemia, papillitis or papilledema can occur in many uveitic
disorders,
However, classically prominent disc hyperemia is noted in VKH.

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Introduction to uveitis

  • 2. • Inflammation of the uveal tract comprised by the iris, ciliary body, and choroid. • Infections of the eye, autoimmune disorders, trauma to the eye, certain medications which can incite ocular inflammation, and rarely malignancies
  • 3. EPIDEMIOLOGY • 10 % blindness worldwide • Prevalence-115-204 /lac • Incidence-17-52/lac • Mean age-40 yrs • M=F
  • 4. SYMPTOMS • dependent upon the anatomic location of the inflammation in the eye, rapidity of onset of the inflammation, and duration and course of disease. • Anterior segment (ocular injection, light sensitivity, pain, blurry vision, epiphora) • Intermediate and posterior segment (floaters, flashing lights, blurry vision)
  • 5. SIGNS ANTERIOR SEGMENT • Cells • Flare • Fibrin • Hypopyon • Synechiae (both anterior and posterior) • Iris nodules • Iris atrophy • Keratic precipitates • Band keratopathy
  • 6. INTERMEDIATE SEGMENT • vitreal cells • “snowball” opacities in the vitreous • exudates over the pars plana (snowbanking) • neovascularization of the pars plana
  • 7. POSTERIOR SEGMENT • Vascular sheathing (arteries, veins, or both) • Retinal pigment epithelial hypertrophy or atrophy • Cystoid macular edema • Atrophy or swelling of the retina, choroid, or optic nerve head • Exudative, tractional, or rhegmatogenous retinal detachment • Retinal or choroidal neovascularization
  • 8. CLASSIFICATION The classification of uveitis is important for multiple reasons that are as follows: • The location of ocular inflammation may assist in diagnosis or at least narrow the potential etiologies (e.g., Fuch’s heterochromic iridocyclitis involves the anterior chamber; ocular toxoplasmosis primarily effects the retina with significant inflammatory spillover into the choroid and vitreous) • Uveitis may be a manifestation of an underlying serious or potentially lethal systemic disease. The correct diagnosis can be sight and on occasion life-preserving. • Uveitis may be caused by a vast number of conditions including infections, autoimmune disorders, medication induced, traumatic, and neoplastic. The correct characterization of the ocular manifestations may assist in identifying an underlying etiology.
  • 9. The Standardization of Uveitis Nomenclature (SUN) Working Group guidance on uveitis terminology, endorsed by the International Uveitis Study Group (IUSG), categorizes uveitis anatomically Anterior • Iritis • Iridocyclitis • Anterior cyclitis Intermediate • Pars planitis • Posterior cyclitis • Hyalitis Posterior • Choroiditis (focal, multifocal, diffuse) • Chorioretinitis • Retinochoroiditis • Retinitis • Neuroretinitis Panuveitis
  • 10. THE SUN WORKING GROUP GRADING SCHEME FOR ANTERIOR CHAMBER CELLS Grade Cells per field 0 <1 0.5+ 1-5 1+ 6-15 2+ 16-25 3+ 26-50 4+ >50 To grade cells and flare, the slit lamp is set to maximum intensity and both the width and length of the beam at 1mm.
  • 11. THE SUN WORKING GROUP GRADING SYSTEM FOR ANTERIOR CHAMBER FLARE Grade Description 0 Complete absence + Faint, barely detectable ++ Moderate - iris and lens details clear +++ Marked—iris and lens details hazy ++++ Intense--fixed coagulated aqueous with fibrin
  • 12. Category Descriptor Comment Onset Sudden Insidious Duration Limited Persistent ≤3 months’ duration >3 months’ duration Course Acute Recurrent Chronic Episode characterized by sudden onset and limited duration Repeated episodes separated by periods of inactivity without treatment ≥3 months duration Persistent uveitis with relapse in <3 months after discontinuing treatment THE SUN WORKING GROUP DESCRIPTORS IN UVEITIS
  • 13.
  • 14.
  • 15. Term Definition Comment Inactive Grade 0 cells (anterior chamber) Worsening activity 2-step increase in level of inflammation (eg, anterior chamber cells, vitreous haze) or increase from grade 3+ to 4+ Improved activity 2-step decrease in level of inflammation (eg, anterior chamber cells, vitreous haze) or decrease to grade 0 ≤3 months’ duration Remission Inactive disease for ≥3 months after discontinuing all treatments for eye disease >3 months’ duration THE SUN WORKING GROUP ACTIVITY OF UVEITIS TERMINOLOGY
  • 16. FEATURES OF GRANULOMATOUS AND NON GRANULOMATOUS UVEITIS Features Granulomatous Non Granulomatous Onset Insidious Acute Course Chronic Short (may be recurrent) Circumcorneal congestion + +++ Pain + + Iris nodule +++ - KP’s Large mutton fat Small or medium Flare + +++ Posterior Segment Commonly involved Rarely involved Vitreous Heavy exudates Fine opacities Nodular lesions Diffuse involvement with edema
  • 17.
  • 18. Clinical classification a. Infectious: i. Bacterial-TB, Syphilis, leprosy ii. Viral-HSV, HZV, CMV iii. Fungal-POHS, pneumocystis, Candida, Aspergillus iv. Parasitic-Toxoplasma ,Toxocara, Cystecercosis b. Non-infectious: i. Known systemic association ii. No known systemic association iii. Masquerade: Pathological Classification i. Granulomatous and non-granulomatous ii. Suppurative and exudative
  • 19. Etiological Classification • Infectious i. Exogenous: Staphyloccous, Pseudomonas, Propionibacterium acnes. • Secondary—Iridocyclitis associated with herpetic keratitis, iridocyclitis associated with anterior and posterior scleritis ii. Endogenous: • Bacterial - TB, Syphilis • Viral - Herpes simplex, CMV, Measles, Influenza • Fungal- Histoplasmosis, Candidiasis • Parasitic – Toxoplasmosis, Toxocariasis, Onchocerciasis, Pneumocystis carinii
  • 20. Hypersensitivity/autoimmune • Lens induced—autoimmune reaction to lens protein • Sympathetic ophthalmia—autoimmunity to uveal pigment. • VKH • Behcet’s suspected autoimmune origin Associated with Systemic Conditions • Ankylosing spondylitis (AS) • Rheumatoid arthritis (RA) • Juvenile rheumatoid arthritis (JRA) • Psoriatic arthritis • Associated with GIT disorders: Ulcerative colitis • Associated with respiratory system : Sarcoidosis, leprosy, TB.
  • 21. Idiopathic • i. Specific—Fuch’s • ii. Nonspecific—Account for 25% of all uveitis. Associated with Neoplasms • Retinoblastoma, choroidal melanoma. Non-infective systemic diseases • Sarocoidosis • Polyarteritis nodosa (PAN) • Disseminated lupus erythematosus (DLE) • Diseases of skin (psoriasis, lichen planus, erythema nodosum, pemphigus). Traumatic uveitis accidental or operative injuries to the uveal tissue
  • 22. HISTORY TAKING Questionnaire by Dr Stephen Foster
  • 23.
  • 24.
  • 25.
  • 26.
  • 27. IOP- • high in 42 % cases • Diseases thought to have a higher rate of ocular hypertension include Fuch’s heterochromic iridocyclitis (FHIC), glaucomatocyclitic crisis or Posner-Schlossman syndrome, sarcoidosis, juvenile rheumatoid arthritis, VKH, toxoplasmosis, and herpetic keratouveitis.
  • 28. KERATIC PRECIPITATES- • helpful in defining between acute versus chronic inflammation, and based on the appearance, may also give clues into the pathogenesis • Fine precipitates - more common in spondyloarthropathies and juvenile arthropathies. • Stellate precipitates seen involving the superior cornea (as opposed to the typical inferior corneal base down triangular appearance of most precipitates) are often seen with Fuch’s heterochromic iridocyclitis. • Mutton fat” precipitates are larger and are formed from macrophages and epithelioid cells. These may be indicative of a granulomatous disease
  • 29. Granulomatous kps non granulomatous kps
  • 30. HYPOPYON- • enough cells(leucocytes) as well as fibrin to clump it • The most common etiologies include infectious (both bacterial and viral), HLA-B27 associated uveitis, and Behcet’s disease. • With infectious endophthalmitis the patient will typically have a history of recent surgery, trauma, or have risk factors for endogenous infection (e.g.,intravenous drug use) • Behcets disease- shifting hypopyon • Pseudohypopyon, composed of tumor cells and debris can occur in some of the masquerade syndromes.
  • 31. Iris Changes- • Sectoral atrophy- HSV, HZV, CMV (if associated with raised IOP ,should arise the suspicion of Herpes) • Nodules on iris-accumulation of inflammatory cells on or within the iris –more common in granulomatous disease • Heterchromia- Fuchs disease
  • 32. Retinal/Choroidal findings • VKH- Serous retinal detachments ,Dalen-Fuchs nodules (small, discrete, deep, yellow- white chorioretinal lesions) may be associated with VKH and sympathetic ophthalmia. • Acute retinal necrosis (ARN) is a type of necrotizing retinitis most commonly caused by herpetic viruses (HSV, VZV). The classic posterior appearance includes vitritis, retinal vascular arteriolitis, and peripheral retinitis. Typically, the retinitis begins as peripheral areas of multifocal retinal yellowing, often flat with scalloped edges. This can eventually progress into confluent whitening extending into the posterior pole. • Classic toxoplasmosis lesions present as focal and white with overlying vitritis with a “headlight in the fog” appearance, often with adjacent pigmented retinochoroidal scarring.
  • 33. • Cytomegalovirus (CMV) retinitis may also be identified clinically and should be suspected in patients who are immunosuppressed. The classic exam findings - peripheral or posterior yellow-white lesions that follow the retinal vasculature centripetally, vasculitis with a “frosted branch” appearance, and retinal hemorrhages. This constellation of findings has been described as a “scrambled eggs or cottage cheese with ketchup” appearance. There may be little to no vitritis, given the immunocompromised state of these patients.
  • 34. • Optic Nerve—Disc hyperemia, papillitis or papilledema can occur in many uveitic disorders, However, classically prominent disc hyperemia is noted in VKH.