Uvea

Question 0

What are the five symptoms of iritis (anterior uveitis, or iridocyclitis?)

Answer 0

1. Pain (an ache, aggravated by reading)
2. Redness
3. Photophobia (from reactive spasm of inflamed iris muscle)
4. Watering (lacrimation)
5. Blurred vision (secondary to inflammatory cells and flare in the anterior chamber; accommodation may be impaired).

Question 1

What is iritis, and what are some other terms for it?

Answer 1

Iritis is the term commonly used for inflammation of the anterior uvea.
However, because the anterior ciliary body is usually involved, the anatomically more correct designation is anterior uveitis or iridocyclitis.

It may be acute or chronic, and can affect one or both eyes. It is often idiopathic, likely to include both genetic and environmental factors. Recurrent episodes of idiopathic acute iritis are common.

Question 2

What are the six signs of iritis?

Answer 2

1. Redness (in early stages, may be confined to limbal area - ciliary flush, circumcorneal injection - eyelid conjunctiva spared)
2. Inflammatory cells and flare (proteinaceous exudate in anterior chamber - appearance similar to beam of a projector passing through smoke in a darkened room - or hypopyon as cells settle in inferior anterior chamber angle).
3. Keratic precipitates (KPs, clumps of inflammatory cells on corneal endothelium, usually on the inferior half).
4. Miosis (spasm of the sphincter muscle of the pupil).
5. IOP - can be low, high, or normal.
6. Fundus - examine thoroughly.

Question 3

What are posterior synechiae?

Answer 3

In iritis, after spasm of the sphincter muscle of the pupil (causing miosis), focal inflammatory adhesions between the pupil margin and the lens (posterior synechiae) are caused by inflammatory mediators and are typical of iritis.

Question 4

About half of patients with acute iridocyclitis are positive for what?

Answer 4

HLA B27, although its significance is not understood.

Question 5

Specific investigations for the most common causes of iridocyclitis are performed when?

Answer 5

Only if the iritis is recurrent, persistent, bilateral, resistance to standard therapy, and granulomatous inflammation.

Initial patient assessment includes an attempt to detect a cause. This involves a simple systems inquiry, which may suggest appropriate investigations. Then turn to specific Ix as indicated above.

Question 6

Name 4 ocular causes of iridocyclitis.

Answer 6

1. Herpes virus: infections of the eye and periocular structures - herpes zoster ophthalmicus, herpes simplex keratitis, etc. Acute glaucoma is an importsnt complication.
2. Fuchs’ heterochromic cyclitis
3. Trauma (incl. surgery) - common cause of anterior segment inflammation
4. Sympathetic ophthalmitis (a rare bilateral granulomatous panuveitis occurring weeks to months after penetrating injury).

Question 7

What is Fuchs’ heterochromic cyclitis?

Answer 7

A low grade but chronic unilateral iridiocyclitis (iritis) characterised by heterochromia (difference in colour between the two irises, owing to diffuse iris atrophy), file stellate KPs (keratic precipitates) across the entire endothelium and the absence of posterior synechiae.
Cataract and glaucoma are long-term complications.
Pts usually present with floaters and cataract.

Question 8

Try and name 10 systemic causes of iridocyclitis.

Answer 8

1. Seronegative arthritides (HLA B27 association)
2. Ankylosing spondylitis
3. Reiter’s syndrome
4. Psoriatic arthritis
5. IBD (Crohn’s, UC)
6. Juvenile chronic arthritis (cataract, glaucoma, macular oedema = common complications).
7. Sarcoidosis (granulomatous anterior uveitis)
8. Behçet’s disease (a systemic vasculitis)
9. Tuberculosis (rare cause)
10. Syphilis (granulomatous and non-granulomatous)
11. Masquerade syndromes (a range of conditions in which uveal inflammation is not the primary pathology. eg. Retinal detachment, neoplasia, bacterial endophthalmitis, presence of occult intraocular foreign body.

Question 9

Name 7 investigations which may be included when worried about iritis (as directed by clinical suspicion)?

Answer 9

1. FBE
2. ESR
3. serum ACE level
4. Syphilis serology
5. HLA typing
6. CXR
7. Sacroiliac joint Xray
   Refer to physician if evidence of systemic disease.

Question 10

What is the mainstay of iritis treatment?

Answer 10

Topical steroids (prednisolone, betamethasone, dexamethasone) which can be varied in concentration and frequency according to the severity of inflammation. 
Usually drops for daytime, and ointment at night. 

Subconjunctival steroids and mydriatics for severe cases.
Deep orbital injection of depot steroid preparations useful for posterior segment inflammation and for maculsr oedema.
Systemic steroids & immunosuppressants reserved for resistant, dight-threatening disease.

Question 11

Besides steroids, what else can help to ease the discomfort in iritis and what is a prophylactic bonus of this?

Answer 11

A topical mydriatic, such as cyclopentolate.

This also prevents the formation of posterior synechiae.

Question 12

What needs to be monitored regularly during active iritis?

Answer 12

IOP.

May be elevated by iritis itself, or by topical steroid use.

Question 13

Name three complications of iritis.

Answer 13

1. Cataract
2. Glaucoma
3. Macular oedema

Question 14

Define posterior uveitis.

Answer 14

Inflammation of the choroid (as opposed to anterior: iris and ciliary body).

Question 15

Name two main symptoms of posterior uveitis, and two rare symptoms.

Answer 15

1. Floaters (vitreous opacities consisting of debris and inflammatory cells)
2. Decreased vision
   Rare: pain and redness.

Question 16

Name three other causes of cells in the vitreous.

Answer 16

1. Neoplasia
2. Retinal tears
3. HIV infection

Question 17

Is posterior uveitis due to a local disorder or systemic disease?

Answer 17

As with anterior uveitis, it can be either local or due to a systemic disease.

Question 18

Name some ophthalmoscopic features of posterior segment inflammation (uveitis):

Answer 18

1. Vitritis: inflammatory cells in the vitreous
2. Vasculitis (?candlewax exudate, venous sheathing, RV occlusion)
3. Cotton wool spots
4. Macular oedema (cystoid)
5. Optic disc swelling
6. Foci of chorioretinal inflammation or scarring

There may be mild anterior uveitis, seen on slit lamp examination only.

Question 19

After general and ocular Hx to indicate a disease process, name 7 other investigations of posterior segment inflammation.

Answer 19

1. FBE
2. ESR
3. ACE levels (elevated in sarcoidosis)
4. Syphilis serology
5. Toxoplasma serology
6. CXR (sarcoidosis and tuberculosis)
7. Fundus fluorescein angiogram: can show disease-specific lesions (occult vasculitis, macular oedema or ischaemia); baseline for future reference; identify cause of loss of vision.

Question 20

What is the most common cause of posterior segment inflammation in the developed world?

Answer 20

Toxoplasmosis (toxoplasma gondii, a protozoan).
About 50% of population positive for Abs, but not indicative of active inflammation so a positive result is unhelpful. A negative result in an immunocompetent pt makes a diagnosis of toxoplasma retinochoroiditis unlikely.

Question 21

How would toxoplasma retinochoroiditis appear on ophthalmoscopy?

Answer 21

One or more foci of activity will usually be visible (in active disease) as a whitish patch with moderately well define borders and overlying vitreous inflammation, sometimes located at the edge of an old pigmented (blackish) scar.
Reactivation of an old ocular lesion tends to occur early in adulthood, causing floaters and blurred vision.

Question 22

Treatment of toxoplasma retinochoroiditis?

Answer 22

Most cases of reactivation resolve spontaneously without impairment of vision, so do not require Rx.
Systemic Rx indicated where vision is significantly affected or threatened, such as when a lesion is close to the macula or optic nerve.
Oral steroids suppress inflammation, but concurrent antimicrobial Rx important. ABx, often in combination, include clindamycin (beware pseudomembranous colitis), sulphonamides, and pyrimethamine (with folic acid).

Question 23

How would sarcoidosis posterior uveitis present?

Answer 23

Unusual for the fundus to be involved without some degree of anterior uveitis, therefore it tends to present with pain and redness in addition to floaters and loss of vision.

Question 24

Name three features on ophthalmoscopy which are characteristic of sarcoidosis posterior uveitis.

Answer 24

1. Snowballs (vitreous inflammatory cells and opacities)
2. Venous sheathing
3. Candlewax drippings (perivenous exudates)

Retinal and choroidal nodules may be seen. The optic disc can be swollen as a result of of infiltration.

Question 25

Treatment of sarcoidosis posterior uveitis?

Answer 25

Systemic Rx via physician.
Mild ocular inflammation may be treated with a topical steroid and a mydriatic. Periocular depot steroid injections, systemic steroids and systemic cyclosporin A are reserved for severe or resistant cases in which permanent visual loss is likely.

Question 26

Name three systemic vasculidites in which the eye may experience vasculitis (posterior uveitis).

Answer 26

1. SLE
2. Polyarteritis nodosa
3. Wegener’s granulomatosis

Question 27

What can be a cause of posterior uveitis, iridocyclitis, vitritis, and occlusive vasculitis, and how is it treated?

Answer 27

Syphilis can cause all of the above. Always check it out with non-specific signs. Treatment with penicillin or erythromycin.

Question 28

What is the pattern of pigmentary retinopathy in congenital syphilis chorioretinitis?

Answer 28

Salt and pepper pattern.

Question 29

In whom is CMV retinochoroiditis seen, and what in pattern does it present on ophthalmoscopy?

Answer 29

Seen almost exclusively in immunocompromised pts, esp. AIDS.
“Pizza” pattern, with white areas of necrotic retinal exudate mixed eith flame haemorrhages.
Treatment with systemic or intravitreal ganciclovir or systemic foscarnet. Resolution leaves a highly atrophic retina which is prone to detach.

Question 30

Ocular features of Behçet’s disease?

Answer 30

Anterior uveitis (typically with a hypopyon), and an occlusive vasculitis.
Venous occlusion and macular oedema are typical.

Question 31

In posterior uveitis, which investigation may help with the diagnosis of the rare, miscellaneous localised inflammatory disorders?

Answer 31

Fundus fluorescein angiography.

Question 32

What is AMPPE (one of the rare inflammatory disorders in posterior uveitis)?

Answer 32

Acute posterior multifocal placoid pigment epitheliopathy.
(Presented at grand round).
Presents in young adults shortly after a viral illness, with blurred vision owing to patchy exudative retinal detachment. Spontaneous resolution to normal occurs in a few weeks.

Question 33

What is pars planitis?

Answer 33

An inflammation of the ciliary body that also causes visual floaters, macular oedema and cataract.
Treat with intensive topical, periocular or systemic steroids (Rx only required if vision significantly decreased to 6/12 or worse).

Question 34

What is a pathognomic feature of pars planitis on ophthalmoscopy?

Answer 34

“Snowbanking” (exudate) in the area of the anterior retina and pars plana.