SCLERITIS&

EPISCLERITIS

Anatomy 3 LAYERS• Episcleral tissue• Sclera proper• Lamina fusca

VASCULAR LAYERS THAT COVERS SCLERA• Conjuctival vessels• Superficial Episcleral Vessels• Deep Vascular Plexus

EPISCLERITIS

• Benign, idiopathic, recurrent• 2 types : Simple Nodular

Simple episcleritis

NODULAR EPISCLERITIS

SCLERITIS

Anterior non-necrotizing scleritis

• DIFFUSE5th decade, womenocular redness followed a few days later by

achingmay spread to the face and templeVascular congestion and dilatation associated

with oedemaRecurrences at the same location are common

NODULAR

• Previous attack of herpes zoster ophthalmicus• Fifth decade• C/F - pain followed by increasing redness,

tenderness of the globe and the appearance of a scleral nodule

• Scleral nodule may be single or multiple and mostly 3–4 mm away from the limbus

• Multiple nodules may coalesce, become confluent• 10% of patients with nodular scleritis develop

necrotizing

Anterior necrotizing scleritis with inflammation

• the aggressive form of scleritis• 60 years • bilateral in 60%• may result in severe visual morbidity and

sometimes loss of the eye• Symptoms - gradual onset of pain which becomes

severe and persistent radiates to the temple, brow or jaw

Signs

• three types of necrotizing diseasea) Vaso-occlusive - often associated with rheumatoid

arthritis Isolated patches of scleral oedema

with overlying non-perfused episclera & conjunctiva The patches coalesce, and if unchecked

rapidly proceed to progressive scleral necrosis

b) Granulomatous

• often associated with Wegener granulomatosis and polyarteritis nodosa

• starts with injection adjacent to the limbus and then extends posteriorly

• Within 24 hours, the sclera, episclera, conjunctiva and adjacent cornea become irregularly raised and oedematous

c) Surgically-induced scleritis

• Within 3 weeks of the surgical procedure• strabismus repair, trabeculectomy , scleral buckling ,

treatment of pterygium with adjunctive mitomycin C

• necrotizing process starts at the site of surgery and then extends outwards

• But it tends to remain localized to one segment

Investigations• Laboratory – RF, ANA, ANCA (cANCA, pANCA) and

antiphospholipid antibodies

• FA – Most ptients - vascular non-perfusion Systemic vasculitis - transudation, localized areas of

vasculitis and new vessel formation

Complications

1 Acute infiltrative stromal keratitis may be localized or diffuse.

2 Sclerosing keratitis, characterized by chronic thinning and opacification in which the peripheral cornea adjacent to the site of scleritis resembles sclera

3 Peripheral ulcerative keratitis is characterized by progressive melting and ulceration. In granulomatous scleritis the destruction extends directly from the sclera into the limbus and cornea. This characteristic pattern is seen in Wegener granulomatosis, polyarteritis nodosa and relapsing polychondritis.

4 Uveitis, if severe, usually denotes aggressive scleritis.

5 Glaucoma is the most common cause of eventual loss of vision. The intraocular pressure is very difficult to control in the presence of active scleritis.

6 Hypotony may be the result of ciliary body detachment, inflammatory damage or ischaemia.

7 Perforation of the sclera as a result of the inflammatory process alone is extremely rare

Scleromalacia perforans

• necrotizing scleritis without inflammation• typically affects elderly women with longstanding

rheumatoid arthritis• perforation of the globe is extremely rare• Presentation - slight non-specific irritation and

keratoconjunctivitis sicca may be suspected; pain is absent and vision unaffected.

Signs – • Necrotic scleral plaques near the limbus

without vascular congestion• Coalescence and enlargement of necrotic

areas.• Very slow progression of scleral thinning

and exposure of underlying uvea

Treatment• Effective in patients with very early disease• Repair of scleral perforation must be attempted

otherwise phthisis bulbi ensues

• Differential diagnosis - scleral hyaline plaque oval, dark-greyish area located close to the

insertion of the horizontal rectus muscles.

Posterior scleritis

• serious, potentially blinding condition• Under the age of 40• Bilateral in 35%• Presentation may be with discomfort or pain. severe in those with accompanying

orbital myositis Tenderness to palpation is very

common

Signsa Exudative retinal detachment occurs in almost

25% of casesb Uveal effusion characterized by exudative retinal

detachment and choroidal detachmentc Choroidal folds represent an anterior

displacement of the choroid. They are usually confined to the posterior pole and run horizontally.

d Subretinal mass characterized by a yellowish-brown elevation which may be mistaken for a choroidal tumour.

e Disc oedema with an accompanying slight reduction of vision is common. It iscaused by spread of the granulomatous process into the orbital tissue and the optic nerve.

f Myositis is common and gives rise to diplopia, pain on eye movement, tenderness to touch and redness around a muscle insertion.

g Proptosis is usually mild and frequently associated with ptosis.

h Other features - glaucoma, periorbital oedema, chemosis and conjunctival injection

Ultrasound : • Increased scleral thickness, scleral nodules and

separation of Tenon capsule from the sclera.• Fluid in Tenon's space gives rise to the characteristic ‘T’ sign in which the stem of the T is

formed by the optic nerve on its side and the cross bar by the gap containing fluid

• Disc oedema, choroidal folds or retinal detachment.

MR and CT may show scleral thickening and proptosis

Differential Diagnosis

1 Subretinal mass - granuloma associated with some other pathology, amelanotic choroidal melanoma, choroidal metastasis and choroidal hemangioma.

2 Choroidal folds, retinal striae and disc oedema - orbital tumours, orbital inflammatory disease, thyroid eye disease, papilloedema and hypotony.

3 Exudative retinal detachment - Vogt–Koyanagi–Harada (VKH) syndrome and central serous retinopathy.

4 Orbital cellulitis

Treatment of immune-mediated scleritis

• Topical steroids – may relieve symptoms and oedema in non-necrotizing disease

• Systemic NSAIDs - should be used only in non-necrotizing disease

• Periocular steroid injections• Systemic steroids - when NSAIDs are

inappropriate or ineffective (necrotizing disease)

• Cytotoxic agents - cyclophosphamide, the drug of choice in Wegener disease, azathioprine, mycophenolate mofetil and methotrexate

• Immune modulators such as ciclosporin and tacrolimus

• Specific antibodies such as infliximab and rituximab show promise

Infectious scleritisCAUSES• Herpes zoster – extremely resistant to treatment

and may result in a punched-out area or a thinned scleral patch

• Tuberculous scleritis – by direct spread from a local conjunctival or choroidal lesion, or more commonly by haematogenous spread. Clinically involvement may be nodular or necrotizing

• Leprosy - Diffuse scleritis is associated with severe recurrent reactions. Nodular scleritis may occur in lepromatous leprosy. Necrotizing disease may occur as a result of scleral infection or as part of an immune response.

• Syphilis - Diffuse anterior scleritis may occur in secondary syphilis. Occasionally scleral nodules may be seen in tertiary syphilis.

• Lyme disease. Scleritis is common but typically occurs long after initial infection.

• Other causes include fungi , P. aeruginosa and Nocardia

Treatment

• specific antimicrobial therapy• Topical and systemic steroids• surgical debridement not only facilitates the

penetration of antibiotics but also debulks the infected scleral tissue

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