INTERMEDIATE AND POST. UVEITIS

By
Dr. VIJAY PRATAP SINGH
MLBMC, JHANSI

INTRODUCTION
Inflammation of uveal tract – UVEITIS

 Intermediate uveitis (IU) is described as
inflammation in the anterior vitreous, pars
plana and the peripheral retina.
 The diagnostic term pars planitis should be
used only for that subset of IU where there is
snow bank or snowball formation occurring in
the absence of an associated infection or
systemic disease

 Pars plana is a flat extension from posterior
aspect of ciliary processes to ora serrata
 3.5 to 4 mm in length
 Around 3 mm from limbus- ciliary processes
Pars plana

 Incidence in population – 1 in 15000
 % of IU in cases of uveitis- 8 to 22%
 Incidence in indian referral eye hospital-
19.8%
 Male: female- 54:46
 Average age in various studies- 23 to 28 years
 Bilateral- 70 to 90% at presentation

 The IUSG (International Uveitis Study Group)
suggested the term IU to denote an idiopathic
inflammatory syndrome, mainly involving the
anterior vitreous, peripheral retina and the
ciliary body with minimal or no anterior
segment or chorioretinal sign
 IU is not hereditary though it has been
observed in families.
 patients who are HLA-DR15-positive and
have IU may have systemic findings of
another HLA-DR15-related disorder- multiple
sclerosis, optic neuritis, and narcolepsy

 Exact theory yet to be known
 may be initiated by an unknown antigen, leading
to a clinical picture of vasculitis and vitreous
cells.
 antigen may be infectious(Lyme's, syphilis and
cat-scratch fever) , autoimmune-(multiple
sclerosis and sarcoidosis)
 Type II collagen in the vitreous may be an
autoantigen in some patients
contd...

 Intermediate uveitis seems to be a T-cell-mediated
disease
 Lymphocytic infiltration of the retinal venules leads to
the clinical picture of vasculitis.
 T-cells are the predominant cell type in the vitreous up
to 95% of all cells, of which CD4+ cells are 35-90%.
 Macrophages -second most important cells
 In active inflammation epitheloid cells and
multinucleated giant cells are seen
 HLA associations include HLA-DR, B8, and B51, the most
significant being HLA-DR which occurs in 67-72% of
patients

 Histological studies of the peripheral retina and
ciliary body demonstrate – condensed vitreous,
fibroblasts, spindle cells, lymphocytes and blood
vessels and prominent lymphocyte cuffing of
retinal veins.
 Pars plana exudates appear to consist of loose
fibrovascular layer containing scattered
mononuclear inflammatory cells and a few
fibrocyte-like cells adjacent to the hyperplastic
nonpigmented epithelium of the pars plana.

One of the most under diagnosed uveitic
disease- lack of routine examination of pars plana,
lack of awareness
SYMPTOMS-(initially)
 floaters
 Mild blurring of vision
 Mild photophobia
 Uncommon- pain, redness

SIGNS-
 Conjunctiva- mild congestion
 Anterior chamber- no or minimal findings
 Cells and flare, KPs, posterior synechiae
( usually inferiorly)
 Vitritis is a characteristic feature of IU, and it is
typically described as vitreous haze ranging from
trace to 4+

FUNDOSCOPY-
 Characteristic mobile, globular, yellow-white
"snowballs" ("ants' eggs") seen in the inferior
peripheral vitreous.
 They lie close to the retina, but are not in
contact with it.
 Inflammatory exudates accumulate over pars
plana to form SNOWBANK
 Periphlebitis of peripheral veins in vicinity of
exudates

CONTD....
 Later the vitreous shows degenerative changes
with fibre-like cylindrical condensations of
coarse vitreous strands.
 Posterior vitreous detachment (PVD) is
common
 Retinal changes in IU include tortuosity in
arterioles and venules, sheathing of peripheral
veins, neovascularizations and retinal
detachments
 Cystoid macular edema

The hallmark of pars planitis are:
 the white or yellowish-white pars plana
exudates ("posterior hypopyon") and
 collagen band (snowbank) over the pars
plana.
 These exudates are preretinal, peripheral,
typically inferior but may also be superior or
divided into multiple foci or extend 360
degrees over the entire pars plana

Contd...
MOST COMMON-
 Idiopathic
 Syphilis
 Lyme disease
 Multiple sclerosis
 Sarcoidosis
 Bartonella
LESS COMMON-
 Lymphoma
 HTLV
 Toxocara
 behcets

Multiple sclerosis:
About 3-27% of patients develop pars planitis
and 7.8-14.8% of patients with IU/pars planitis
develop MS.
 characterized by pars plana snowbanks, retinal
periphlebitis (in 5-20%) and panuveitis are the
commonest manifestations of MS and up to 95%
are bilateral
Intraocular lymphoma:
10-20% the disease commences as vitreous or
retinal infiltrates mimicking uveitis and 95% are
non-Hodgkins B-cell lymphomas

Sarcoidosis: About 23-26% of patients with
sarcoidosis develop IU
 typical ocular findings-CME, optic disc
swelling, periphlebitis, and retrobulbar optic
neuritis were seen in patients with IU, both
with or without sarcoidosis
 It is commonly bilateral, and presents as IU
and granulomatous anterior uveitis

Syphilis: uveitis is the commonest presentation
of syphilis.
 Anterior uveitis( granulomatous and
nongranulomatous), posterior uveitis,
panuveitis, vitritis, vasculitis, retinitis,
placoid choroiretinitis and optic nerve
involvement are also seen in eyes with
syphilitic uveitis.
 IU has been described to occur in Lyme's
disease caused by another spirocheate-
Borrelia burgdorferi, both in adults and in
children

 Diagnosis is based on clinical findings
 patient's history should focus-duration of
symptoms, the number of recurrences, and
findings that might be associated with
systemic disorders

Ancillary test-
FFA- detection of CME
- capillary and disc hyperflourescence,
staining of vessel wall, fern pattern radial
hyperflourescence

 USG- exudates over pars plana
 ERG- abnormal b wave implicit time
 UBM- exudates and vitritis

 Baseline invs- CBC, ESR, RFT,LFT,
 PPD test - exclude tuberculosis/sarcoidosis
 Chest X-ray-- findings indicative of
sarcoidosis or tuberculosis.
 Sarcoidosis – CT scan lungs, ACE enzymes
 Serologic testing for- cat-scratch disease,
syphilis (VDRL, FTA-ABS)and Lyme's
 HLA DR2 typing
 Vitreous tap

1)CME-
Upto 50% patients
Most common cause of visual loss
Prevalance increases with severity of
inflammation
Epiretinal membrane

CATARACTS-
 Either due to inflammation or steroids
 Most often type – PSC
 15-50% of eyes
 GLAUCOMA

 Optic disc edema- due to intraocular inflammation
20% patients
 optic neuritis – may or may not be associated with
multiple sclerosis
 NVD/NVE

 Venous sheathing- most often benign
 VASCULITIS- associated with ischaemia
Sheathing +/-
 Retinal detachment
A) Serous RD
B) Rhegmatogenous RD- associated with dialysis at
snow bank

Four step approach by Kaplan
Step 1-
 Posterior sub tenon injection of steroids- methyl
prednisolone 40mg or triamcinolone 40 mg (0.75 to 1 ml)
 Preferably in upper temporal quadrant
 2 to 3 injections at interval of 3-4 weeks- resolution
 Topical 1% prednisolone acetate
 Systemic steroids 1mg/kg can be added (mantoux test to
be done)
 Improvement in vision 67% cases
 Appropriate antibiotics for infections causes
Compliactions- rise in iop, ptosis, globe perforation,
necrotising scleritis

Step 2-
 Not responded to peri ocular/ systemic steroids
 Cryopexy can be done
 Mechanism- destruction of hyperemic vascular
component of diseases by eliminating neovasclar
and ischaemic tissue
 Double freeze and thaw technique
 Impovement in vision- 32 to 67 percent
 Complications- RD, PVR
 Doesnt prevent recurrence
 Indirect laser also used

Step 3-
 Not responding to cryopexy also
 Pars plana vitrectomy is done
 Helps in early resolution of CME
Step 4-
 If step 1 fails- immunosuppresive agents can be
used
 Cyclophosphamide, azathioprine, chlorambucil
 Azathioprine- 50 mg thrice daily for four
months(tapered)
 Platelet counts, WBC regularly monitored

 Some advocate use of a combination of
betamethasone and depot
methylprednisolone(sub tenon) in an effort to
achieve early onset and prolonged duration of
action.
 Intravitreal triamcinolone acetonide injections
have been used to treat CME.
 use of somatostatin analogues (Octreotide) IM
and intravitreal bevacizumab (Avastin) in
patients with refractory uveitic CME.
 the surgical implantation of a fluocinolone
acetonide (Retisert) or dexamethasone
(Ozurdex) implant can be considered

 Cyclosporine, tacrolimus, azathioprine, and
methotrexate are the most commonly used agents with
documented efficacy in many uveitic conditions
 Chlorambucil can be considered for intractable cases
NEWER DRUGS USED-
 Infliximab, (anti-TNF) monoclonal antibody, has been
shown to be effective in improving macular thickness
and visual acuity in patients with uveitic refractory CME
due to intermediate uveitis or other noninfectious
uveitis.
 Daclizumab, an interleukin-2 receptor blocking
antibody, has been shown to be effective in
noninfectious uveitis in a multicenter nonrandomized
interventional case series
 Interferon-beta (INF-beta), which has an established
value in the treatment of MS, appears to have a
positive effect in terms of visual acuity, CME

 MANAGEMENT OF GLAUCOMA
 Rx OF CATARACT
 Anti VEGF for neovascularisation
 Supportive rx

POSTERIOR UVEITIS
Is Inflammation of retina or choroid posterior to
vitreous base.
Choroiditis
Chorioretinitis
Retinochoroiditis
Retinal vasculitis
Neuroretinitis

 Infective-
Viral , Fungal, Bacterial, Parasitic.
 Non infective –
Sarcoidosis, SLE, Krill’s Ds,

 CHOROIDITIS is a painless condition but
visual symptoms d/t vitreouse haze and
retinal involvement
 Visual symptoms
Defective vision
Photopsia
Floaters
Metamorphosia
Micropsia
Macropsia
Positive scotoma

 Ant. Segment sign-
Usually there is no external sign, however
fine KP’s may be seen
 Vitreouse opacities-
It is mainly d/t choroiditis
Choroiditis may be
Focal
Diffuse and
Multifocal

 Active patches
Pale yellow or dirty white
raised area with ill-
defined edge.
 Healed patches-
Sharply defined from the
rest of normal area d/t
atrophy of choroidal
tissue

 Diagnostic criteria-
Malar rash
Serositis
Arthritis etc.
Choroidopathy:In Fundus
examination, multiple serous
retinal detachments, RPE
detachments and a central serous
chorioretinopathy
Retinopathy: typically B/L , M/c
findings are cotton-wool spots and
hemorrhages. other findings
include hard exudates, retinal
edema, and vascular tortuosity.
Specific tests- Anti nuclear Ab
Assay.

 Ant seg- sarcoid
granulomas, iris nodules
 Post seg- vitritis,
periphlebitis (Snow balls)
 ‘candlewax’ drippings
 Diagnosis-
X-ray chest
Hypercalcemia
Serum ACE
Ga scan
Biopsy

 Usually a/w Pt. suffuering
from AIDS, on cytotoxic
chemotherapy or long term
immunosuppression.
 Ant. Segment sign- KP’s may
be seen.
 Post. Segment sign-
Haemorragic retinitis
Granular retinitis
Tx-
-HAART.
-Ganciclovir, valaganciclovir,
foscarnet and cidofovir

 M/c cause of Posterior
uveitis,
 The lesions develop in
deep retina, few to no
vitreous cells may be
present (Headlight in fog)
 Presents as a focal
necrotizing retinitis,
atrophic chorioretinal scar,
often located in macula in
congenital cases.

LABORATORY STUDIES- Serology
 Serum antitoxoplasma antibody titers
 Enzyme-linked immunosorbent assay (ELISA)
 Indirect fluorescent antibody test
 Indirect hemagglutination test
 Complement fixation
 Sabin-Feldman dye test
IMAGING STUDIES-
Fluorescein angiography (FFA)
Indocyanine green (ICG)
B-SCAN

 TRIMETHOPRIM AND SULFAMETHOXAZOLE with Para-
aminobenzoic acid (PABA).
 Pyrimethamine (Daraprim) with oral folinic acid.
 Clindamycin, spiramycin, tetracyclines, atovaquone,
azithromycin and clarithromycin.
 Topical steroids
 Photocoagulation or cryotherapy & Pars plana
vitrectomy are surgical option

 Intestinal roundworm.
 Young children
 Almost U/L
 Clinically p/w-
- Chronic toxocara endophthalmitis (leucocoria)
- Post pole / peripheral granuloma
 Dx-ELISA, USG, CT scan.
 Tx- Sub-Tenon steroid, pars plana vitrectomy

 It may occur disseminated, peripheral or
diffuse choroiditis.
 Dx- Confirmed by
FTA-ABS blood test
TPI test
VDRL tes
 Tx- usual uveitic Tx + Systemic penicillin
or other antisyhilitic drugs

 Common cause in developing
countries.
 Clinical presentations-
A) Ant. Uveitis
B) Post. Uveitis -Multiple miliary
tubercal in choroid
-Diffuse or multifocal
choroiditis.
-Choroidal garanuloma.
C) Vasculitis (Eales’ disease).
 Dx- Clinical based + skin test +
Isoniazid response test
 Tx- Chemotherapy with (R+Z)+
Usual Tx of uveitis

PRESUMED OCULAR HISTOPLASMOSIS
SYNDROME
More common in endemic area
Clinical features-
Histospot-
Atrophic scar scattered in mid-retinal
periphery
Macular histospot-
Atrophic macular scar f/b a hole in
Bruch’s membrane
Tx-
a) Systemic steroids in active macular
lesions
b) Laser photocoagulation in subretinal
neovacular membrane
c) Anti-VEGF

Ocular candidiasis
Opportunistic infection in
pt. suffering from AIDS
Cause multifocal
chorioretinits.
 Multiple small, round,
whitish area a/w area of
haemorrages with pale
center(Roth’s spot).
 Tx-
Topical cycloplegics &
antifungal
Systemic antifungal.
Pars plana vitrectomy

Behcet’s Disease-
A/w HLA-B51
 B/L ,acute recurrent iridocyclitis a/w
hypopyon
 Also a/w post. Uveitis, vitritis, periphlebitis
retinae and retinitis .
Tx-
 Corticosteroid
 Immunosuppressive drugs

 VKH syndrome is an idiopathic multisystem
autoimmune disease featuring inflammation of
melanocyte-containing tissues such as the uvea, ear
and meninges. a/w HLA-DR4/DW15
 Post. Uveitis lesion includes
- Multifocal choroidits
- Exudative RD
- Choroidal depigmentation(sun glow fundus)
- Dalen-fuch’s nodules
- Peripheral choroidal granuloma
Tx-Corticosteroid and immunosuppressive drugs

INTERMEDIATE AND POST. UVEITIS

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INTERMEDIATE AND POST. UVEITIS