Episcleritis and Scleritis

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Compiled by: Dr. Iddi Ndyabawe
EPISCLERITIS AND SCLERITIS
EPISCLERITIS
Episcleritis is benign recurrent inflammation of the episclera, involving the overlying Tenon's
capsule but not the underlying sclera.
It typically affects young adults, being twice as common in women than men.
Etiology
-Exact etiology is not known.
-It is found in association with gout, rosacea and psoriasis.
It has also been considered a hypersensitivity reaction to endogenous tubercular or streptococcal
toxins.
Pathology
Histologically, there occurs localised lymphocytic infiltration of episcleral tissue associated with
oedema
and congestion of overlying Tenon's capsule and conjunctiva.
Clinical picture
Symptoms. Episcleritis is characterised by:
-redness,
-mild ocular discomfort described as gritty, burning or foreign body sensation.
Many a time it may not be accompanied by any discomfort at all. Rarely, mild photophobia and
lacrimation may occur.
Signs. On examination two clinical types of episcleritis, diffuse (simple) and nodular may be
recognised.
Episclera is seen acutely inflamed in the involved area.
In diffuse episcleritis, although whole eye may be involved to some extent, the maximum

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inflammation is confined to one or two quadrants
In nodular episcleritis, a pink or purple flat nodule surrounded by injection is seen, usually
situated 2-3 mm away from the limbus.
The nodule is firm, tender and the overlying conjunctiva moves freely.
Clinical course. Episcleritis runs a limited course of 10 days to 3 weeks and resolves
spontaneously.
However, recurrences are common and tend to occur in bouts. Rarely, a fleeting type of disease
(episcleritis periodica) may occur.
Differential diagnosis
-inflamed pinguecula,
-swelling and congestion due to foreign body lodged in bulbar conjunctiva and
-very rarely with scleritis.
Treatment
1. Topical corticosteroid eyedrops instilled 2-3 hourly, render the eye more comfortable and
resolve the episcleritis within a few days.
2. Cold compresses applied to the closed lids may offer symptomatic relief from ocular
discomfort.
3. Systemic non-steroidal anti-inflammatory drugs (NSAIDs) such as flurbiprofen (300 mg OD),
indomethacin (25 mg three times a day), or oxyphenbutazone may be required in recurrent cases.

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SCLERITIS
Scleritis refers to a chronic inflammation of the sclera proper.
It is a comparatively serious disease which may cause visual impairment and even loss of the
eye if treated inadequately.
Fortunately, its incidence is much less than that of episcleritis.
It usually occurs in elderly patients (40-70 years) involving females more than the males.
Etiology
It is found in association with multiple conditions which are as follows:
1. Autoimmune collagen disorders, especially rheumatoid arthritis, is the most common
association.
Overall about 5% cases of scleritis are associated with some connective tissue
disease.
About 0.5 percent of patients (1 in 200) suffering from seropositive rheumatoid arthritis develop
scleritis.
Other associated collagen disorders are Wegener's granulomatosis, polyarteritis nodosa (PAN),
systemic lupus erythematosus (SLE) and ankylosing spondylitis.
2. Metabolic disorders like gout and thyrotoxicosis have also been reported to be associated with
scleritis.
3. Some infections, particularly herpes zoster ophthalmicus, chronic staphylococcal and
streptococcal infection have also been known to cause scleritis.
4. Granulomatous diseases like tuberculosis, syphilis, sarcoidosis, leprosy can also cause
scleritis.
5. Miscellaneous conditions like irradiation, chemical burns, Vogt-Koyanagi-Harada syndrome,
Behcet's disease and rosacea are also implicated in the etiology.
6. Surgically induced scleritis follows ocular surgery.
It occurs within 6 month postoperatively.
Exact mechanism not known, may be precipitation of underlying systemic cause.

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7. Idiopathic. In many cases cause of scleritis is unknown.
Pathology
Histopathological changes are that of a chronic granulomatous disorder characterised by
fibrinoid necrosis, destruction of collagen together with infiltration by polymorphonuclear cells,
lymphocytes, plasma cells and macrophages.
The granuloma is surrounded by multinucleated epitheloid giant cells and old and new vessels,
some of which may show evidence of vasculitis.
Classification
It can be classified as follows:
I. Anterior scleritis (98%)
1. Non-necrotizing scleritis (85%)
(a) Diffuse
(b) Nodular
2. Necrotizing scleritis (13%)
(a) with inflammation
(b) without inflammation (scleromalacia perforans)
II. Posterior scleritis (2%)
Clinical features
Symptoms. Patients complain of moderate to severe pain which is deep and boring in character
and often wakes the patient early in the morning.
Ocular pain radiates to the jaw and temple.
It is associated with localised or diffuse redness, mild to severe photophobia and lacrimation.
Occasionally there occurs diminution of vision.
Signs. The salient features of different clinical types of scleritis are as follows:
1. Non-necrotizing anterior diffuse scleritis.
It is the commonest variety, characterised by widespread inflammation involving a quadrant or
more of the anterior sclera.
The involved area is raised and salmon pink to purple in colour.

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2. Non-necrotizing anterior nodular scleritis.
It is characterised by one or two hard, purplish elevated scleral nodules, usually situated near the
limbus.
Sometimes, the nodules are arranged in a ring around the limbus (annular scleritis).
3. Anterior necrotizing scleritis with inflammation.
It is an acute severe form of scleritis characterised by intense localised inflammation associated
with areas of infarction due to vasculitis.
The affected necrosed area is thinned out and sclera becomes transparent and ectatic with uveal
tissue shining through it.
It is usually associated with anterior uveitis.
4. Anterior necrotizing scleritis without inflammation (scleromalacia perforans).
This specific entity typically occurs in elderly females usually suffering from long-standing
rheumatoid arthritis.
It is characterised by development of yellowish patch of melting sclera (due to obliteration of
arterial supply); which often together with the overlying episclera and conjunctiva completely
separates from the surrounding normal sclera.
This sequestrum of sclera becomes dead white in colour, which eventually absorbs leaving
behind it a large punched out area of thin sclera through which the uveal tissue shines
Spontaneous perforation is extremely rare.
5. Posterior scleritis.
It is an inflammation involving the sclera behind the equator.
The condition is frequently misdiagnosed.
It is characterised by features of associated inflammation of adjacent structures, which include:
exudative retinal detachment, macular oedema, proptosis and limitation of ocular movements.

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Complications
These are quite common with necrotizing scleritis and include:
- sclerosing keratitis,
-keratolysis,
-complicated cataract and
-secondary glaucoma.
Investigations
Following laboratory studies may be helpful in identifying associated systemic diseases or in
establishing the nature of immunologic reaction:
1. TLC, DLC and ESR
2. Serum levels of complement (C3), immune complexes, rheumatoid factor, antinuclear
antibodies and L.E cells for an immunological survey.
3. FTA - ABS, VDRL for syphilis.
4. Serum uric acid for gout.
5. Urine analysis.
6. Mantoux test.
7. X-rays of chest, paranasal sinuses, sacroiliac joint and orbit to rule out foreign body especially
in patients with nodular scleritis.
Treatment
(A) Non-necrotising scleritis.
It is treated by topical steroid eyedrops and systemic indomethacin 100 mg daily for a day and
then 75 mg daily until inflammation resolves.
(B) Necrotising scleritis.
It is treated by topical steroids and heavy doses of oral steroids tapered slowly. In non-responsive
cases, immuno-suppressive agents like methotrexate or cyclophos-phamide may be required.
Subconjunctival steroids are contraindicated because they may lead to scleral thinning and
perforation.

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BLUE SCLERA
It is an asymptomatic condition characterised by marked, generalised blue discolouration of
sclera due to thinning.
It is a typical association of osteogenesis imperfecta. Its other causes are:
-Marfan's syndrome,
-Ehlers-Danlos syndrome,
-pseudoxanthoma elasticum,
-buphthalmos,
-high myopia and
-healed scleritis.