Complications of Scleritis

1. DISCUSS THE
COMPLICATIONS
OF SCLERITIS
NPMCN ANTERIOR SEGMENT QUESTION 5
BY
DR CHIMA EMMANUEL EDOGA
ON
JUNE 21, 2023
TITLE SLIDE

2. OUTLINE
1. INTRODUCTION
2. CLASSIFICATION OF SCLERITIS
3. CLINICAL FEATURES
4. SYSTEMIC ASSOCIATIONS
5. INVESTIGATIONS
6. TREATMENT
7. COMPLICATIONS
8. CONCLUSION
SLIDE 2 OF 32

3. INTRODUCTION
• Scleritis is inflammation (oedema and cellular infiltration) of the
sclera, which ranges from a trivial self-limiting episode to a vision-
threatening necrotizing process
• The sclera is the opaque, elastic and resilient outer 5/6 of the outer
coat of the eye.
• 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 Tenon’s capsule
SLIDE 3 OF 32

4. CLASSIFICATION OF SCLERITIS
SCLERITIS
Anterior
Non-Necrotizing
Diffuse Nodular
Necrotizing
Without Inflammation
• Vaso-occlusive
• Granulomatous
• Surgically induced
• Infective
With Inflammation
Scleromalacia
Perforans
Posterior
SLIDE 4 OF 32

5. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS
• Diffuse
• Diffuse disease is slightly more common in females and usually presents
in the 5th decade
• Symptoms
• 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
• The vision may be blurred
SLIDE 5 OF 32

6. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS
• Diffuse
• Signs
• Vascular congestion and dilatation associated with oedema If treatment is
started early the disease can be completely inhibited ○ The redness may be
generalized or localized to one quadrant If confined to the area under the
upper eyelid the diagnosis may be missed
• Chemosis
• Eyelid swelling
• Anterior uveitis
• Raised IOP
SLIDE 6 OF 32

7. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS
• Diffuse
• Signs
• 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
• Recurrences at the same location are common unless an underlying cause is
treated
SLIDE 7 OF 32

8. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS
• Nodular
• 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
• The age of onset is similar to that of diffuse scleritis
Symptoms
• The insidious onset of pain followed by increasing redness, tenderness of the
globe and the appearance of a scleral nodule
• The vision is often reduced
SLIDE 8 OF 32

9. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS
• Nodular
Signs
• Scleral nodules may be single or multiple and most frequently develop in the
inter-palpebral region close to the limbus
• They have a deeper blue–red colour than episcleral nodules and are immobile
• In contrast to episcleritis, a slit lamp beam shows an elevated anterior scleral
surface
• Multiple nodules may expand and coalesce if treatment is delayed
• ○ Instillation of 10% phenylephrine drops will constrict the conjunctival and
superficial episcleral vasculature but not the deep plexus overlying the nodule
SLIDE 9 OF 32

10. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
• Necrotizing disease is the aggressive form of scleritis
• The age at onset is later than that of non-necrotizing scleritis,
averaging 60 years
• 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
SLIDE 10 OF 32

11. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Symptoms
• Gradual onset of pain that becomes severe and persistent and radiates to the
temple, brow or jaw
• It frequently interferes with sleep and responds poorly to analgesia
Signs
• Signs vary according to the following three types of necrotizing disease.
• Vaso-occlusive is commonly associated with rheumatoid arthritis Isolated
patches of scleral oedema with overlying non-perfused episclera and conjunctiva
are seen
• The patches coalesce and if unchecked rapidly proceed to scleral necrosis
SLIDE 11 OF 32

12. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Signs
• Granuloma formation may occur in conjunction with conditions such as
granulomatosis with polyangiitis or polyarteritis nodosa
• The disease typically 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
SLIDE 12 OF 32

13. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NECROTIZING SCLERITIS (WITHOUT INFLAMMATION)
Signs
• Surgically induced scleritis typically starts within 3 weeks of a procedure,
though much longer intervals have been reported
• It may be induced by any type of surgery including strabismus repair,
trabeculectomy with excessive exposure to MMC, excision of pterygium and
scleral buckling
• The necrotizing process starts at the site of surgery and extends outwards, but
tends to remain localized to one sector
SLIDE 13 OF 32

14. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NECROTIZING SCLERITIS (WITH INFLAMMATION)
• Scleromalacia perforans (5% of scleritis) is a specific type of progressive scleral
thinning without inflammation
• typically affects elderly women with longstanding rheumatoid arthritis, but has
also been described in association with other systemic disorders
• Despite the nomenclature, perforation of the globe is rare as integrity is
maintained by a thin layer of fibrous tissue
Symptoms
• Mild non-specific irritation
• Pain is absent and vision unaffected and keratoconjunctivitis sicca may be
suspected
SLIDE 14 OF 32

15. CLINICAL FEATURES OF SCLERITIS
• ANTERIOR NON-NECROTIZING SCLERITIS (WITH INFLAMMATION)
Signs
• Necrotic scleral plaques near the limbus without vascular congestion
• Coalescence and enlargement of necrotic areas
• Slow progression of scleral thinning, with exposure of underlying uvea
SLIDE 15 OF 32

16. CLINICAL FEATURES OF SCLERITIS
• POSTERIOR SLERITIS
Symptoms
• Pain does not correlate well with the severity of inflammation but tends to be more
severe in those with accompanying orbital myositis
• Photophobia is not a dominant feature
Signs
• The disease is bilateral in 35%
• Proptosis
• Raised IOP
• Periorbital oedema and chemosis
• Associated anterior scleritis
SLIDE 16 OF 32

17. CLINICAL FEATURES OF SCLERITIS
• POSTERIOR SLERITIS
Signs
• Fundus mass – subretinal in nature
• Myositis is common and gives rise to diplopia, pain on eye movement, tenderness to
touch and redness around a muscle insertion
• Disc oedema with accompanying reduction of vision is common and is caused by
spread of inflammation into the orbital tissue and optic nerve
• Choroidal folds are usually confined to the posterior pole, often temporal and
orientated horizontally, surrounding the subretinal mass
• Choroidal detachment
• RD – Exudative retinal detachment occurs in around 25%
SLIDE 17 OF 32

18. SYSTEMIC ASSOCIATIONS OF SCLERITIS
• Immune-Mediated (Non-Infectious)
• Rheumatoid Arthritis (most
common systemic association of
scleritis and can manifest with any
form of immune-mediated scleral
inflammation)
• Systemic Lupus Erythematosus
• Ankylosis spondylitis
• Reactive arthritis
• Psoriatic arthritis
• Arthritis & Inflammatory Bowel Dx
• Relapsing polychondritis
• Vasculitic Diseases
• Polyarteritis nodosa
• Giant Cell Arteritis
• Behcet’s Disease
• Granulomatosis with polyangitis
(Wegener)
• Allergic granulomatous angitis
(Churg-Strauss syndrome)
• Cogan’s syndrome
SLIDE 18 OF 32

19. SYSTEMIC ASSOCIATIONS OF SCLERITIS
• Infectious
• Viral e.g. Herpes Zooster - most
common infective cause
• Bacterial – Leprosy,
Tuberculosis, Syphilis,
Pseudomonas aeruginosa,
Nocardia
• Fungal
• Parasitical
• Others
• Atopy
• Rosacea
• Gout
SLIDE 19 OF 32

20. INVESTIGATIONS FOR SCLERITIS
• Ocular
• CVF
• Fundus Photography
• OCT
• Ultrasonography
• In Posterior Scleritis may show increased scleral thickness, scleral
nodules, separation of Tenon capsule from sclera, disc oedema, choroidal
folds and retinal detachment
• Fluid in the Tenon space may give a characteristic ‘T’ sign, the stem of the
T being formed by the optic nerve and the cross bar by the fluid-
containing gap
SLIDE 20 OF 32

21. INVESTIGATIONS FOR SCLERITIS
B-scan ultrasonography
in posterior scleritis
showing scleral
thickening and fluid in
the sub-Tenon space
(arrow), with the
characteristic ‘T’ sign
SLIDE 21 OF 32

22. INVESTIGATIONS FOR SCLERITIS
• Ocular
• Fluorescein angiography of the anterior segment
• helps to distinguish necrotizing disease by the presence of non-
perfusion and can be used for monitoring
• Occlusion is predominantly venular in inflammatory disease and
mainly arteriolar in scleromalacia perforans
• Indocyanine green (ICGA) is a more accurate indicator of disease
activity
SLIDE 22 OF 32

23. INVESTIGATIONS FOR SCLERITIS
• Systemic - Routine
• FBC, ESR, SEUCr, FBS, and LFT to establish baseline and for monitoring
treatment response
• Systemic - Specific (Non-infectious) – Labouratory and b
• Rheumatoid Arthritis – 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)
• Tuberculosis – 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
SLIDE 23 OF 32

24. INVESTIGATIONS FOR SCLERITIS
• Systemic- Specific
• Systemic Lupus Erythematosus (SLE) – Antinuclear antibodies
(ANAs), Antiribosomal antibodies, antibodies to double-stranded DNA
(anti dsDNA), Anti-Sm (Smith) antibodies
• Sarcoidosis – 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.
• Wegner’s granulomatosis (WG) – Antineutrophil cytoplasmic antibody
(ANCA). cANCA is more specific than pANCA
SLIDE 24 OF 32

25. INVESTIGATIONS FOR SCLERITIS
• Specific (Infectious)
• Microscopy
• Gram Staining
• Giemsa staining
• 10% KOH mount
• Culture and Sensitivity (using appropriate transport media)
SLIDE 25 OF 32

26. TREATMENT OF SCLERITIS
• MEDICAL
• NSAIDS (systemic)
• Ibuprofen (400-800mg tds), Diclofenac (75mg SR bd), Piroxicam (20mg tds)
• CORTICOSTEROIDS
• Topical (Prednisolone 1%, Loteprednol 0.5%)
• Periocular (should not be used in necrotizing scleritis)
• Oral (1-1.5mg/kg/day)
• Intravenous (0.5–1 g daily for 3 days)
• IMMUNOSUPPRESSIVE AGENTS
• 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
SLIDE 26 OF 32

27. TREATMENT OF SCLERITIS
• MEDICAL
• IMMUNOSUPPRESSIVE AGENTS
• 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
• Alkylating agents e.g. Chlorambucil, Cyclophosphamide
• T-cell inhibitors e.g. Cyclosporine, Tacrolimus
• Biological blockers e.g. infliximab, rituximab
SLIDE 27 OF 32

28. TREATMENT OF SCLERITIS
• SURGICAL
• Sclera Patch Grafts
• Donor Sclera (Fresh, glycerine preserved or frozen)
• Fascia lata, periosteum, aortic tissue, dura matter, processed
pericardium, split thickness dermis, polytetrafluoroethylene
• Conjunctiva-Muller Muscle flap
SLIDE 28 OF 32

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
• In granulomatous scleritis, the destruction extends directly from the sclera into the limbus and
cornea
• This characteristic pattern is seen in granulomatosis with polyangiitis, polyarteritis nodosa
and relapsing polychondritis
• Peripheral corneal ulceration can occur at any stage of a necrotizing scleritis and, in rare cases,
precede its onset
SLIDE 29 OF 32

30. COMPLICATIONS OF SCLERITIS
COMPLICATIONS OF ANTERIOR SCLERITIS
OTHERS
• Uveitis, if severe, may denote aggressive scleritis
• Hypotony (rarely phthisis) may be the result of ciliary body detachment, inflammatory damage
or ischaemia
• 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
• Sclera cyst
• Sclera Abscess
• acquired superior oblique tendon sheath syndrome rare
• Perforation of the sclera as a result of the inflammatory process alone is extremely rare
SLIDE 30 OF 32

31. COMPLICATIONS OF SCLERITIS
COMPLICATIONS OF POSTERIOR SCLERITIS
• Exudative retinal detachment occurs in around 25%
• The yellowish-brown sub-retinal exudative material can be mistaken for a choroidal tumour
• Uveal effusion with choroidal detachment may be present
• 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
• Treatment must not be delayed in these patients as permanent visual loss can rapidly ensue
SLIDE 31 OF 32

32. CONCLUSION
• SCLERITIS IS A RARE BUT SERIOUS OCULAR DISEASE THAT
REQUIRES A MULTI-DISCIPLINARY MANAGEMENT, PROMPT
AND SOMETIMES PROLONGED TREATMENT TO AVOID
SERIOUS COMPLICATIONS
SLIDE 32 OF 32