Scleritis

Scleritis and episcleritis

1. SCLERITIS & EPISCLERITIS

2. Anatomy 3 LAYERS Episcleral tissue Sclera proper Lamina fusca VASCULAR LAYERS THAT COVERS SCLERA Conjuctival vessels Superficial Episcleral Vessels Deep Vascular Plexus

3. EPISCLERITIS Benign, idiopathic, recurrent 2 types : Simple Nodular

4. Simple episcleritis

5. NODULAR EPISCLERITIS

6. SCLERITIS

7. Anterior non-necrotizing scleritis DIFFUSE 5 th decade, women ocular redness followed a few days later by aching may spread to the face and temple Vascular congestion and dilatation associated with oedema Recurrences at the same location are common

8. 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

9. Multiple nodules may coalesce, become confluent 10% of patients with nodular scleritis develop necrotizing

10. 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

11. Signs three types of necrotizing disease 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

12. 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

13. 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

14. 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

15. 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 .

16. 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

17. 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.

18. 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

19. 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.

20. 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

21. Signs a Exudative retinal detachment occurs in almost 25% of cases b Uveal effusion characterized by exudative retinal detachment and choroidal detachment c 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.

22. e Disc oedema with an accompanying slight reduction of vision is common. It is caused 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

23. 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

24. 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

25. 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 )

26. 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

27. Infectious scleritis CAUSES 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

28. 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

29. 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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