3. UVEITIS
A review report of 522 articles from several studies around the
world :
• Anterior uveitis accounts for 29% to 61% of all forms of uveitis
• Panuveitis 9% to 42%
• Posterior uveitis 7% to 31%
• Intermediate uveitis accounts for 7% to 16%
3
4. 4
15% of all uveitis, quite eye
external, floaters, VA reduced
with a cataract or chronic CME
5. INTERMEDIATE UVEITIS
￮ Chronic, relapsing disease of insidious onset.
￮ The vitreous is the primary site of inflammation
￮ 50% idiopathic or associated with a systemic disease
￮ It may be one of the features of serious or life threatening systemic
disease
￮ Systemic investigations are routinely performed especially
￮ in the presence of suggestive findings
￮ in older individuals
Pars Planitis: the most common form (86%-90% of cases).
￮ Affects persons aged 5-40 years
￮ Absence of associated infection or systemic disease
5
6. Intermediate Uveitis: Signs
￮ Inflammatory cells may aggregate in the vitreous ("snowballs"),
where some coalesce.
￮ Inflammatory exudate accumulation on the inferior pars plana is
("snow banking").
￮ Spill over anterior chamber cells, vitreous cells, & snowballs.
￮ Inferior peripheral retinal phlebitis with retinal venous sheathing
￮ Vitreous hemorrhage, which contracts leading to rhegmatogenous
RD.
￮ CME; (chronic and refractory in 10% of patients)
6
13. SARCOIDOSIS
￮ is a T-lymphocyte-mediated non-caseating granulomatous
inflammatory disorder
￮ Common in cold climates but more frequently affects patients of
African descent
￮ Affects mediastinal and superficial lymph nodes, lungs, liver,
spleen, skin, parotid glands, phalangeal bones and the eye.
￮ The frequency of ocular involvement ranges from 26% to 50%.
￮ Presents as intermediate disease in 7% of cases.
13
14. SARCOIDOSIS
The characteristics of ocular involvement when present include:
1. Seen generally early in the course of the disease
2. May co-exist with asymptomatic systemic disease and
3. Can precede systemic involvement by several years.
4. Most patients present between the ages of 20 to 40 years; however,
children and the elderly can be affected
Clinical presentation
￮ Sarcoidosis may have an acute onset or insidious onset
￮ 14
15. SARCOIDOSIS
The acute-onset sarcoidosis presents in young patient in the following ways:
1. Löfgren syndrome : characterised by erythema nodosum and bilateral hilar
lymphadenopathy often accompanied by fever, anorexia and arthralgia.
2. Heerfordt syndrome (uveoparotid fever): is characterized by uveitis, parotitis, fever
and cranial nerve palsy, usually the seventh nerve
OCULAR MANIFESTATION
￮ Uveitis is the most common and may be in the form of anterior, posterior or
intermediate uveitis
￮ Intermediate uveitis with snowballs or string-like opacities in the vitreous
￮ It is often associated with granulomatous anterior uveitis
15
16. MULTIPLE SCLEROSIS
￮ 30% of patients with MS will develop intermediate uveitis
￮ Uveitis is 10 times more common in this group of individuals than in
the general population
￮ Intermediate uveitis and pan uveitis are the most common
categories of MS-associated uveitis
￮ Up to 95% of cases are bilateral
￮ White women 20-50 years of age are more affected
￮ The onset of uveitis may precede the diagnosis of MS in up to 25% of
patients, and by 5-10 years.
￮ Up to 15% of patients with pars planitis may eventually develop MS
16
17. MULTIPLE SCLEROSIS
￮ HLA-DRI5 appears to be associated with the combination of MS
and uveitis
￮ Pathogenesis not very clear but may involve humoral, cellular,
and immunogenetic components directed against myelin.
17
18. Toxocariasis
￮ Toxocariasis is caused by an infestation with a common
intestinal ascarid (roundworm) of dogs called Toxocara
canis .
￮ Human infestation is by accidental ingestion of soil or food
contaminated with ova shed in dogs' faeces.
￮ Commonly affects children with pica
￮ In the human intestine, the ova develop into larvae which
penetrate the intestinal wall and travel to various organs,
such as the liver, lungs, skin, brain and eyes
￮ When the larvae die, they disintegrate and cause an
inflammatory reaction followed by granulation.
18
19. Toxocariasis
Human infestation can take one of the following forms:
1. Visceral larva migrans (VLM) is caused by severe systemic
infection which usually occurs at about the age of 2 years
￮ Clinically patient may have low-grade fever,
hepatosplenomegaly, pneumonitis, convulsions. The
blood shows leucocytosis and marked eosinophilia
2. Ocular toxocariasis may present as one of the following
clinical forms:
i. Chronic endophthalmitis
19
20. Toxocariasis
￮ Presentation is between the ages of 2 and 9 years with
leukocoria strabismus or unilateral visual loss
￮ Anterior uveitis and vitritis.
￮ peripheral granuloma
￮ The peripheral retina and pars plana may be covered by a
dense greyish-white exudate, similar to the snowbanking seen
in pars planitis
￮ The peripheral retina and pars plana may be covered by a
dense greyish-white exudate, similar to the snowbanking seen
in pars planitis
20
21. Toxocariasis
The main causes of visual loss are tractional
retinal detachment and hypotony with
phthisis bulbi, the latter caused by separation
of the ciliary body from the sclera by
contraction of a cyclitic membrane
Ultrasonography may be useful in establishing
the diagnosis in eyes with hazy media and in
excluding other causes of leukocoria
21
22. LYME DISEASE
￮ Lyme disease is a multisystem disorder caused by the
spirochete Borrelia burgdorferi (B. burgdorferii) that is
transmitted to humans by the bite of a tick.
￮ Lyme disease is a disorder associated with multisystem
abnormalities whose most prominent manifestations affect
skin, nervous system, musculoskeletal system and heart.
22
23. LYME DISEASE
￮ A primary infection occurs at the site of the tick bite. B. burgdorferi
entries through a break in skin or mucous membrane and disseminates
to all parts of the body via spirochetemia.
￮ The direct invasion by B. burgorferi may cause mechanical tissue
damage.
￮ The bacterium itself and its outer surface protein A (OspA) is a strong
antigen that stimulates natural, humoral and cell immunologic
responses of the host, subsequently causing intense inflammation in the
different involved parts of the host body
23
24. LYME DISEASE
CLINICAL PRESENTATION
The disease has three defined clinical stages
￮ Stage I
I. begins within 1 month of an infected tick bite, usually in the
summer, and is manifested by an oval or annular skin rash
of varying severity, often with a clear center at the area of
the bite: erythema migrans
II. The lesion may itch or be painful, but is often
asymptomatic.
III. Other symptoms include: headache, malaise, fatigue,
fever, and arthralgias; lymphadenopathy may also occur
at the early stage
24
25. LYME DISEASE
Stage II follows several weeks to months after infection and is
characterized by potential involvement of nervous system and
heart.
1. The neurological symptoms may include severe headache
and stiff neck, meningitis, peripheral radiculopathy, and
cranial nerve palsies.
2. The symptoms of headache, nausea, photophobia and
vomiting often indicate meningeal involvement.
3. Cranial nerve palsy may be unilateral or bilateral, and most
often affecting the facial nerves (Bell's palsy).
25
26. LYME DISEASE
Stage III may occur up to 2 years after the initial infection
and is characterized by prolonged episodes of migratory
oligoarthritis and chronic neurologic syndromes.
￮ Neurological features include: ataxia, chronic
encephalopathy, seizure, dementia, myelitis, spastic
paraparesis, and psychiatric disturbances. Other
symptoms include fatigue, lymphadenopathy,
splenomegaly, sore throat, dry cough, nephritis,
hepatitis, or orchitis
26
27. LYME DISEASE
￮ Ocular manifestations occur at any stage but are more common in
the last two stages
￮ The most common ocular finding in stage I is conjunctivitis.
￮ During the second and third stages, ocular involvement include:
anterior, intermediate, and posterior uveitis, endophthalmitis, keratitis
(stromal opacities, punctuate superficial keratitis or peripheral
ulcerative keratitis), and conjunctivitis
￮ Neuroophthalmic features can also occur, including involvement of
third, sixth, and seventh cranial nerves (Bell's palsy, most
common)optic nerve (optic neuritis and perineuritis, papilledema,
ischemic optic neuropathy, optic nerve atrophy).
27
28. DIAGNOSIS
￮ Diagnosis of Lyme disease is primarily based on clinical
presentations and serologic finding
￮ Eryethma migrans is the typical clinical marker for Lyme
disease and is present in 60% to 80% of patients.
￮ About 40 to 60% of patients with Lyme disease have elevated
antibody titers (IgM and IgG) to B. burgdorferi several weeks
after infection
￮ Polymerase chain reaction (PCR) is a very specific
28
30. OCULAR BARTONELLOSIS
￮ Caused by Bartonella henselae
￮ Gram negative bacteria and is the etiologic agent of
cat-scratch disease.
￮ Humans are usually infected through a cat's scratch or
bite, but a bite by cat fleas may also be the origin of
infection
￮ More common in children and young adults, it usually
presents with a wide range of systemic and ocular
symptoms
￮ Systemic signs and symptoms usually precede ocular
involvement
30
31. OCULAR BARTONELLOSIS
￮ Erythematous papule on the skin on the site of inoculation
￮ Seven to 14 days after the exposure a follicular conjunctivitis
may appear.
￮ Fourteen to 21 days after the inoculation regional
lymphoadenopathy may occur which is usually associated
with myalgias, fatigue and low-grade fever.
￮ The association of conjunctivits and regional
lymphoadenopathy is well known as Parinaud's
oculoglandular syndrome (POGS).
31
32. OCULAR BARTONELLOSIS
￮ Anterior uveitis, intermediate uveitis and orbital abscess
￮ Enzyme immunoassay and Western Blot, along with PCR
analysis
￮ Treatment – Azithromycin for 21 days or doxycycline for 2-6
weeks.
32
33. Retinitis pigmentosa
￮ Patients with retinitis pigmentosa (RP) often have variable
numbers of vitreous cells
￮ Distinguishing features of RP that differentiate it from uveitis
include
nyctalopia, positive family history, and on fundus examination, waxy
disc pallor, attenuation of arterioles, and a bone-spicule pattern of
pigmentary changes in the mid periphery.
33
34. INTERMEDIATE UVEITIS
TREATMENT
￮ Therapy should be directed toward treating the underlying cause of
the inflammation, if possible.
￮ Otherwise, anti-inflammatory therapy is used
￮ Treatment is begun if visual acuity is affected and the patient is
symptomatic or if CME and retinal vasculitis are present.
￮ Mild cases without CME may not require treatment.
￮ A classic 4-step approach has been described.
34
35. Step 1
￮ Periocular corticosteroids usually constitute the first line of therapy.
￮ Triamcinolone or methylprednisolone using local injection or depot.
Injections every 4 weeks until 4 injections have been administered.
￮ Intravitreal triamcinolone injections may be an alternative to
periocular injections in severe refractory cases
￮ Complications include: Corticosteroid-induced lOP elevation, Aponeurotic ptosis,
Enophthalmos, Cataract formation
￮ Systemic corticosteroid therapy may be started if local therapy is not effective (1- 1.5
mg/kg/day) tapered.
35
36. Step 2
￮ If corticosteroid therapy fails, peripheral ablation of the pars plana
snowbank with cryotherapy and/or indirect laser photocoagulation to
the peripheral retina can be performed.
￮ Re-treatment is sometimes necessary.
￮ How peripheral laser photocoagulation and cryotherapy decrease
inflammation is unknown.
￮ Cryotherapy shouldn’t be performed in the presence of a tractional RD
with peripheral neovascularization.
￮ It has an increased risk of progressive traction and development of
rhegmatogenous retinal detachment.
36
37. Step 3
￮ If cryotherapy fails, pars plana vitrectomy with induction of posterior
hyaloidal separation and peripheral laser photocoagulation of the pars
plana snowbank may be performed.
￮ Vitrectomy treats severe visual loss caused by dense vitreous cellular
accumulation, vitreal hemorrhage or traction, RD, and CME.
￮ Separation of the posterior hyaloid membrane during vitrectomy may
have a beneficial effect in reducing CME.
￮ Potential complications include retinal detachment, endophthalmitis,
and cataract formation.
37
38. Step 4
￮ Systemic immunomodulatory agents such as methotrexate,
cyclosporine, azathioprine, mycophenolate mofetil, and
cyclophosphamide may also be tried
￮ These are indicated for treatment of bilateral disease.
￮ Rigid adherence to this stepwise approach to
pars planitis treatment may prevent the
benefits of combined therapy.
38
In their initial report, Aaberg and colleagues showed that following treatment with cryotherapy, 13 of 23 eyes (57%) had a decrease in vitritis and improvement in visual acuity.
