Episclera/Sclera/Uvea

Question 1

What are your top differentials for severe ocular pain?

Answer 1

Uveitis
Acute angle closure
Scleritis
Corneal pathology

Question 2

Describe scleritis. What is Tx?

Answer 2

Granulomatous inflammation of sclera that is associated with systemic disease 50% of time. Most likely collagen vascular (ex. rheumatoid/SLE). W>M. Scleritis is much less common than episcleritis. Signs include uveitis, glaucoma, cataracts, corneal involvement.

Typically presents with SEVERE BORING PAIN that radiates to forehead, brow or jaw. Sclera may be blue. Vessels will not blanche with phenyl. Anterior > posterior in presentation.

Non-necrotizing 85%: diffuse most common characterized by diffuse hyperemia; nodular characterized by deep focal injection overlying and immobile nodule

Necrotizing: with inflammation = worst form. 33% of patients die within few years d/t autoimmune disease. 40% lose vision; without inflammation = scleromalacia perforans. Results from rheumatoid arthritis and presents with complete lack of symptoms and minimal injection with large scleral thinning.

Treatment includes:
identifying underlying disorder
NO TOPICALS; oral NSAIDs or steroids or immunosuppressive agents
Glasses or eye shields for significant tinning or risk of perforation

Question 3

Uveitis is a breakdown of…

Answer 3

blood aqueous barrier - tight junctions in non-pigmented ciliary body epithelium, iris vessels and Schlemm’s canal

Allows WBCs and protein to enter AC

Question 4

What are KPs?

Answer 4

WBCs attached to endothelium

Mutton-fat KPs are clusters of macrophages and epithelioid cells. When seen on the iris, they are called Koeppe and Busacca nodules. All mean granulomatous etiology (TB, Sarcoid)

Fresh KPs are white; old KPs are pigmented

Question 5

What are common causes of anterior uveitis?

Answer 5

Ankylosing spondylitis (most common anterior uveitis cause) - young males with lower back pain and stiffness after inactivity

Reactive arthritis (most common in children)- young males with urethritis, arthritis in hip/knee/ankle, and conjunctivitis with increased ESR

Inflammatory bowl disease - includes Crohn’s disease and ulcerative colitis with chronic intermittent diarrhea and constipation. Uveitis is more common in ulcerative colitis

Behcet’s disease - Young adults with acute recurrent hypopyon iritis and mouth and genital ulcers. Seen in asians and middle easterners

Lyme disease - h/o tick bite, skin rash, and arthritis

Glaucomatocyclitic crisis (Posner Sclossman) - mild iritis with recurrent, self-limiting episodes of elevated IOP, fine KPs and open angle on gonio. IOP elevates d/t trabeculitis. Treated with steroids and topical hyptensives

Question 6

What are common causes of posterior uveitis?

Answer 6

Toxoplasmosis (most common cause in adults)- unilateral vitritis in young, healthy patient and leads to unilateral red eye and decreased vision. “headlights in the fog”

Sarcoidosis - Granulomatous panuveitis with retinal vasculitis with vitritis and cotton ball opacities. Also, yellow-white exudates (candle wax droppings) and retinal vessel sheathing

Syphilis - causes acute multifocal chorioretinitis and vitrifies. Seen with salt and pepper fundus and flame shaped retinal hemorrhages.

Pars planitis - intermediate uveitis in young and old patients with snow banking on inferior pars plana

Cytomegalovirus - seen in immunocompromised - white patches of necrotic retina with hemorrhagic retinitis, and vascular sheathing. Most common in AIDS patients with CD4 count <50

Question 7

What are the following tests used to detect in uveitis?
PPD
Chest x-ray
ACE
HLA-B27
RPR VDRL FTA-Abs

Answer 7

PPD - tuberculosis reading of 15mm or more = +

x-ray - sarcoidosis and tuberculosis and ankylosing spondylitis

ACE - sarcoidosis or inflammatory conditions

HLA-B27 - anterior uveitis (non-specific). CRAP - crowns, reiter’s, ankylosing, psoriatic arthritis

RPR/VDRL/FTA-Abs - active syphilis; FTA-Abs tells you if they have ever had syphilis for life

Question 8

How is Uveitis treated?

Answer 8

Overall goal:
resolve ocular issues and determine underlying systemic etiology

Treat inflammation with Pred Forte q1-2h and Homatropine q12h or cyclopentolate QID. Taper steroid and once it is down to QID, then you can taper at a rate of 1 drop per week every 7 days. Durezol is an alternative but is more potent and dosed less frequently (q3-4h or QID).

Take home point! Slow taper at the end is crucial so that inflammation does not rebound.

Subtenon and steroid injections are options for severe cases that do not resolve.

Refer to internal medicine for further eval in unresolving cases.