2. OUTLINE
1. INTRODUCTION
2. CLASSIFICATION OF SCLERITIS
3. CLINICAL FEATURES
4. SYSTEMIC ASSOCIATIONS
5. INVESTIGATIONS
6. TREATMENT
7. COMPLICATIONS
8. CONCLUSION
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3. INTRODUCTION
โข Scleritis is inflammation (oedema and cellular infiltration) of the
sclera, which ranges from a trivial self-limiting episode to a vision-
threatening necrotizing process
โข The sclera is the opaque, elastic and resilient outer 5/6 of the outer
coat of the eye.
โข Episcleritis is the inflammation of the episclera, which is the thin
densely vascularized layer of connective tissue overlying the sclera
and is situated below the Tenonโs capsule
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5. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS
โข Diffuse
โข Diffuse disease is slightly more common in females and usually presents
in the 5th decade
โข Symptoms
โข Ocular redness progressing a few days later to pain that may radiate to the
face and temple. The discomfort typically wakes the patient in the early
hours of the morning, improves later in the day and responds poorly to
common analgesics
โข The vision may be blurred
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6. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS
โข Diffuse
โข Signs
โข Vascular congestion and dilatation associated with oedema If treatment is
started early the disease can be completely inhibited โ The redness may be
generalized or localized to one quadrant If confined to the area under the
upper eyelid the diagnosis may be missed
โข Chemosis
โข Eyelid swelling
โข Anterior uveitis
โข Raised IOP
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7. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS
โข Diffuse
โข Signs
โข As the oedema resolves, the affected area often takes on a slight grey/blue
appearance because of increased scleral translucency. This is due to
rearrangement of scleral fibres rather than a decrease in scleral thicknessm
โข Recurrences at the same location are common unless an underlying cause is
treated
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8. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS
โข Nodular
โข The incidence of nodular and diffuse anterior scleritis is the same but a
disproportionately large number of those with nodular disease have had a
previous attack of Herpes Zoster Ophthalmicus
โข The age of onset is similar to that of diffuse scleritis
Symptoms
โข The insidious onset of pain followed by increasing redness, tenderness of the
globe and the appearance of a scleral nodule
โข The vision is often reduced
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9. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS
โข Nodular
Signs
โข Scleral nodules may be single or multiple and most frequently develop in the
inter-palpebral region close to the limbus
โข They have a deeper blueโred colour than episcleral nodules and are immobile
โข In contrast to episcleritis, a slit lamp beam shows an elevated anterior scleral
surface
โข Multiple nodules may expand and coalesce if treatment is delayed
โข โ Instillation of 10% phenylephrine drops will constrict the conjunctival and
superficial episcleral vasculature but not the deep plexus overlying the nodule
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10. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
โข Necrotizing disease is the aggressive form of scleritis
โข The age at onset is later than that of non-necrotizing scleritis,
averaging 60 years
โข The condition is bilateral in 60% of patients and unless appropriately
treated, especially in its early stages, may result in severe visual
morbidity and even loss of the eye
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11. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Symptoms
โข Gradual onset of pain that becomes severe and persistent and radiates to the
temple, brow or jaw
โข It frequently interferes with sleep and responds poorly to analgesia
Signs
โข Signs vary according to the following three types of necrotizing disease.
โข Vaso-occlusive is commonly associated with rheumatoid arthritis Isolated
patches of scleral oedema with overlying non-perfused episclera and conjunctiva
are seen
โข The patches coalesce and if unchecked rapidly proceed to scleral necrosis
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12. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Signs
โข Granuloma formation may occur in conjunction with conditions such as
granulomatosis with polyangiitis or polyarteritis nodosa
โข The disease typically starts with injection adjacent to the limbus and then
extends posteriorly
โข Within 24 hours, the sclera, episclera, conjunctiva and adjacent cornea become
irregularly raised and oedematous
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13. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Signs
โข Surgically induced scleritis typically starts within 3 weeks of a procedure,
though much longer intervals have been reported
โข It may be induced by any type of surgery including strabismus repair,
trabeculectomy with excessive exposure to MMC, excision of pterygium and
scleral buckling
โข The necrotizing process starts at the site of surgery and extends outwards, but
tends to remain localized to one sector
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14. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NECROTIZING SCLERITIS (WITH INFLAMMATION)
โข Scleromalacia perforans (5% of scleritis) is a specific type of progressive scleral
thinning without inflammation
โข typically affects elderly women with longstanding rheumatoid arthritis, but has
also been described in association with other systemic disorders
โข Despite the nomenclature, perforation of the globe is rare as integrity is
maintained by a thin layer of fibrous tissue
Symptoms
โข Mild non-specific irritation
โข Pain is absent and vision unaffected and keratoconjunctivitis sicca may be
suspected
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15. CLINICAL FEATURES OF SCLERITIS
โข ANTERIOR NON-NECROTIZING SCLERITIS (WITH INFLAMMATION)
Signs
โข Necrotic scleral plaques near the limbus without vascular congestion
โข Coalescence and enlargement of necrotic areas
โข Slow progression of scleral thinning, with exposure of underlying uvea
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16. CLINICAL FEATURES OF SCLERITIS
โข POSTERIOR SLERITIS
Symptoms
โข Pain does not correlate well with the severity of inflammation but tends to be more
severe in those with accompanying orbital myositis
โข Photophobia is not a dominant feature
Signs
โข The disease is bilateral in 35%
โข Proptosis
โข Raised IOP
โข Periorbital oedema and chemosis
โข Associated anterior scleritis
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17. CLINICAL FEATURES OF SCLERITIS
โข POSTERIOR SLERITIS
Signs
โข Fundus mass โ subretinal in nature
โข Myositis is common and gives rise to diplopia, pain on eye movement, tenderness to
touch and redness around a muscle insertion
โข Disc oedema with accompanying reduction of vision is common and is caused by
spread of inflammation into the orbital tissue and optic nerve
โข Choroidal folds are usually confined to the posterior pole, often temporal and
orientated horizontally, surrounding the subretinal mass
โข Choroidal detachment
โข RD โ Exudative retinal detachment occurs in around 25%
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18. SYSTEMIC ASSOCIATIONS OF SCLERITIS
โข Immune-Mediated (Non-Infectious)
โข Rheumatoid Arthritis (most
common systemic association of
scleritis and can manifest with any
form of immune-mediated scleral
inflammation)
โข Systemic Lupus Erythematosus
โข Ankylosis spondylitis
โข Reactive arthritis
โข Psoriatic arthritis
โข Arthritis & Inflammatory Bowel Dx
โข Relapsing polychondritis
โข Vasculitic Diseases
โข Polyarteritis nodosa
โข Giant Cell Arteritis
โข Behcetโs Disease
โข Granulomatosis with polyangitis
(Wegener)
โข Allergic granulomatous angitis
(Churg-Strauss syndrome)
โข Coganโs syndrome
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19. SYSTEMIC ASSOCIATIONS OF SCLERITIS
โข Infectious
โข Viral e.g. Herpes Zooster - most
common infective cause
โข Bacterial โ Leprosy,
Tuberculosis, Syphilis,
Pseudomonas aeruginosa,
Nocardia
โข Fungal
โข Parasitical
โข Others
โข Atopy
โข Rosacea
โข Gout
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20. INVESTIGATIONS FOR SCLERITIS
โข Ocular
โข CVF
โข Fundus Photography
โข OCT
โข Ultrasonography
โข In Posterior Scleritis may show increased scleral thickness, scleral
nodules, separation of Tenon capsule from sclera, disc oedema, choroidal
folds and retinal detachment
โข Fluid in the Tenon space may give a characteristic โTโ sign, the stem of the
T being formed by the optic nerve and the cross bar by the fluid-
containing gap
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21. INVESTIGATIONS FOR SCLERITIS
B-scan ultrasonography
in posterior scleritis
showing scleral
thickening and fluid in
the sub-Tenon space
(arrow), with the
characteristic โTโ sign
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22. INVESTIGATIONS FOR SCLERITIS
โข Ocular
โข Fluorescein angiography of the anterior segment
โข helps to distinguish necrotizing disease by the presence of non-
perfusion and can be used for monitoring
โข Occlusion is predominantly venular in inflammatory disease and
mainly arteriolar in scleromalacia perforans
โข Indocyanine green (ICGA) is a more accurate indicator of disease
activity
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23. INVESTIGATIONS FOR SCLERITIS
โข Systemic - Routine
โข FBC, ESR, SEUCr, FBS, and LFT to establish baseline and for monitoring
treatment response
โข Systemic - Specific (Non-infectious) โ Labouratory and b
โข Rheumatoid Arthritis โ Rheumatoid Factor (Classic Rose-Waaler
haemagglutination test for Rheumatoid factor in the serum),
Rheumatoid factor is an autoantibody directed against the Fc region of
Immunoglobulin G (IgG)
โข Tuberculosis โ Tuberculin Test (Mantoux Test) with false +ves due to
cross-reactivity with BCG and non-tuberculous mycobacteria in the
environment, CXR, Chest CT, Gene Xpert, Interferon-gamma release
assay
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24. INVESTIGATIONS FOR SCLERITIS
โข Systemic- Specific
โข Systemic Lupus Erythematosus (SLE) โ Antinuclear antibodies
(ANAs), Antiribosomal antibodies, antibodies to double-stranded DNA
(anti dsDNA), Anti-Sm (Smith) antibodies
โข Sarcoidosis โ Serum Angiotensin-converting Enzyme (ACE) and Serum
Lysozyme are non specific but suggestive. Bilateral Hilar
lymphadenopathy with CXR is considered pathognomonic, definitive
diagnosis is made by solid tissue biopsy showing classic noncaseating
granulomas.
โข Wegnerโs granulomatosis (WG) โ Antineutrophil cytoplasmic antibody
(ANCA). cANCA is more specific than pANCA
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25. INVESTIGATIONS FOR SCLERITIS
โข Specific (Infectious)
โข Microscopy
โข Gram Staining
โข Giemsa staining
โข 10% KOH mount
โข Culture and Sensitivity (using appropriate transport media)
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26. TREATMENT OF SCLERITIS
โข MEDICAL
โข NSAIDS (systemic)
โข Ibuprofen (400-800mg tds), Diclofenac (75mg SR bd), Piroxicam (20mg tds)
โข CORTICOSTEROIDS
โข Topical (Prednisolone 1%, Loteprednol 0.5%)
โข Periocular (should not be used in necrotizing scleritis)
โข Oral (1-1.5mg/kg/day)
โข Intravenous (0.5โ1 g daily for 3 days)
โข IMMUNOSUPPRESSIVE AGENTS
โข Indicated in Anterior necrotizing Scleritis, Posterior Scleritis not responding to
steroid or requiring long-term steroids, Scleritis associated with a systemic
autoimmune disease or collagen vascular disease
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27. TREATMENT OF SCLERITIS
โข MEDICAL
โข IMMUNOSUPPRESSIVE AGENTS
โข Anti-Metabolites e.g. Methotrexate (Oral, 0.1-0.5mg/kg/week starting
with 7mg/week and increasing to 25mg/week, given with folic acid analog,
FBC and LFT Monitoring essential due to bone marrow suppression and
hepatotoxicity), Azathioprine, mycophenolate mefetil
โข Alkylating agents e.g. Chlorambucil, Cyclophosphamide
โข T-cell inhibitors e.g. Cyclosporine, Tacrolimus
โข Biological blockers e.g. infliximab, rituximab
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28. TREATMENT OF SCLERITIS
โข SURGICAL
โข Sclera Patch Grafts
โข Donor Sclera (Fresh, glycerine preserved or frozen)
โข Fascia lata, periosteum, aortic tissue, dura matter, processed
pericardium, split thickness dermis, polytetrafluoroethylene
โข Conjunctiva-Muller Muscle flap
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29. COMPLICATIONS OF SCLERITIS
COMPLICATIONS OF ANTERIOR SCLERITIS
CORNEA (KERATITIS)
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 and may
constitute a severe risk to the integrity of the eye
โข In granulomatous scleritis, the destruction extends directly from the sclera into the limbus and
cornea
โข This characteristic pattern is seen in granulomatosis with polyangiitis, polyarteritis nodosa
and relapsing polychondritis
โข Peripheral corneal ulceration can occur at any stage of a necrotizing scleritis and, in rare cases,
precede its onset
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30. COMPLICATIONS OF SCLERITIS
COMPLICATIONS OF ANTERIOR SCLERITIS
OTHERS
โข Uveitis, if severe, may denote aggressive scleritis
โข Hypotony (rarely phthisis) may be the result of ciliary body detachment, inflammatory damage
or ischaemia
โข Glaucoma is the most common cause of eventual loss of vision. The IOP can be very difficult to
control in the presence of active scleritis
โข Sclera cyst
โข Sclera Abscess
โข acquired superior oblique tendon sheath syndrome rare
โข Perforation of the sclera as a result of the inflammatory process alone is extremely rare
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31. COMPLICATIONS OF SCLERITIS
COMPLICATIONS OF POSTERIOR SCLERITIS
โข Exudative retinal detachment occurs in around 25%
โข The yellowish-brown sub-retinal exudative material can be mistaken for a choroidal tumour
โข Uveal effusion with choroidal detachment may be present
โข Disc oedema with accompanying reduction of vision is common and is caused by spread of
inflammation into the orbital tissue and optic nerve. Optic atrophy may result after resolution
โข Treatment must not be delayed in these patients as permanent visual loss can rapidly ensue
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32. CONCLUSION
โข SCLERITIS IS A RARE BUT SERIOUS OCULAR DISEASE THAT
REQUIRES A MULTI-DISCIPLINARY MANAGEMENT, PROMPT
AND SOMETIMES PROLONGED TREATMENT TO AVOID
SERIOUS COMPLICATIONS
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Editor's Notes
Granulomatosis with polyangiitis (Wegener granulomatosis) is an idiopathic multisystem granulomatous disorder characterized by small vessel vasculitis typically affecting primarily the paranasal sinuses, lower respiratory tract, the kidneys and skin. There is a male predominance. Presentation is in the fifth decade on average, often with pulmonary symptoms.