Scleritis
I. Describe the approach to establishing the diagnosis
A. Describe the etiology of the disease
1. 50-75% idiopathic
2. 25-50% have underlying systemic disease
a. More common: rheumatoid arthritis, Wegener granulomatosis, relapsing polychondritis
b. Less common: ankylosing spondylitis, systemic lupus erythematosus, polyarteritis nodosa, inflammatory bowel disease, gout, post-herpes zoster ophthalmicus, sarcoidosis, syphilis
3. Deposition of immune complexes in vessel walls appears to be important
4. Disordered immune response leads to tissue and blood vessel damage
B. List the pertinent elements of the history
1. Severe eye pain, boring quality
2. Red eye
3. Pain on movement of eye
4. Tearing
5. Slow onset
6. May be recurrent
C. Describe pertinent clinical features
1. Classification
a. Anterior
i. Diffuse - most common presentation, least severe (See Cornea Figure 46)
ii. Nodular
iii. Necrotizing with inflammation- most severe, greatest potential for visual loss (See Cornea Figure 47)
iv. Necrotizing without inflammation (scleromalacia perforans)- association with rheumatoid arthritis
b. Posterior
2. Signs
a. Anterior: pain on palpation, red-violet hue to sclera (seen best with natural lighting), inflammation of sclera and episclera, scleral nodules, uveitis, peripheral keratitis
b. Posterior: hyperopia, proptosis, lid edema, ophthalmoplegia, disc edema, exudative retinal detachment, macular edema, choroidal folds
D. Describe appropriate laboratory testing for establishing the diagnosis
1. Medical workup: complete blood count, erythrocyte sedimentation rate, rapid plasma reagin test or Venereal Disease Research Laboratory, fluorescent treponemal antibody absorption test, rheumatoid factor, antinuclear antibody panel, angiotensin converting enzyme test, cytoplasmic-staining anti-neutrophil cytoplasmic antibody, perinuclear-staining anti-neutrophil cytoplasmic antibody, chest radiograph, purified protein derivative (tuberculosis skin test), urinalysis
2. Ultrasound (if posterior scleritis is suspected)
3. Consider computed tomography scan (if posterior scleritis is suspected)
II. Define the risk factors
A. Depends on etiology
B. May be idiopathic
C. Genetic factors: certain major histocompatibility antigens may predispose some individuals
III. List the differential diagnosis
A. Episcleritis
B. Conjunctivitis
IV. Describe patient management in terms of treatment and follow-up
A. Oral nonsteroidal anti-inflammatory drugs
B. Systemic corticosteroids (start with 1 mg/kg/day and slowly taper)
C. Systemic immunosuppressive agents in consultation with internist, oncologist, rheumatologist
1. Cyclophosphamide particularly for systemic vasculitis, polyarteritis nodosa, Wegener granulomatosis
2. Methotrexate
3. Azathioprine
4. Cyclosporine
D. Follow-up in 2-7 days depending on severity of presentation
V. List the complications of treatment, their prevention, and management
A. Nonsteroidal anti-inflammatory drugs
1. Gastrointestinal disturbance
2. Impaired renal function
B. Systemic corticosteroids
1. Hypertension
2. Elevated blood glucose
3. Weight gain
4. Cushingoid appearance
C. Systemic Immunosuppressive agents
1. Cyclophosphamide
a. Bone marrow suppression
b. Hemorrhagic cystitis
c. Secondary malignancies
2. Methotrexate
a. Hepatoxicity
b. Interstitial pneumonitis
c. Bone marrow suppression
d. Gastrointestinal disturbance
e. Alopecia
f. Mouth sores
3. Azathioprine
a. Bone marrow depression
b. Hepatotoxicity
c. Gastrointestinal disturbance
4. Cyclosporine
a. Renal toxicity
b. Hepatotoxicity
c. Hypertension
d. Secondary malignancy
e. Increased risk of infection
f. Growth of body hair
D. Prevention and management
1. Taper medications as appropriate
2. Know toxicities and monitor appropriately
3. Combination therapy may allow for tapering of systemic corticosteroids
4. Manage in concert with physician experienced in use of these medications (e.g., rheumatologist, oncologist, primary care physician)
VI. Describe disease-related complications
VII. Describe appropriate patient instructions
A. Emphasize importance of close follow-up
B. Discuss possible systemic side effects of medications
C. Discuss natural history of disease
Additional Resources
1. AAO, Focal Points: Scleritis and Episcleritis: Diagnosis and Management, Module #9, 1995.
2. AAO, Basic and Clinical Science Course. Section 8: External Disease and Cornea, 2004-2005.
3. Dana MR, Reddy CV, Foster CS. Adult rheumatoid arthritis. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. Vol 5 2nd ed. Philadelphia: Saunders; 2000:4555-4563.