Scleritis: Moderator: DR Sangeetha Patil Presenter: DR Shraddha Sudarshan

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Scleritis

Moderator: Dr Sangeetha Patil


Presenter: Dr Shraddha Sudarshan
Sclera
ANATOMY
Sclera is an opaque, elastic and resilient tissue of the eye which forms 5/6th of
the outer coat of the eyeball.
Anteriorly - begins at limbus
Posteriorly- terminates at the optic nerve canal
Embryologically originates from the neural crest cells and mesoderm.
White in colour- due to the scattering of all wavelengths of light by dense
irregular bundles of collagen.
Thickest- Near the optic nerve (1mm) Thinnest-Near the insertion of
intraocular muscles(0.3mm)
Structure
Episclera: Thin densely vascularized layer of connective tissue
overlying the sclera and situated below the tenon’s capsule.Anteriorly
episclera blends with subconjunctival tissues and tenon’s capsule 1 to
3 mm behind the limbus.
Sclera proper: Also called substantia propria.Composed of collagen
bundles, elastic fibres, fibroblasts and ground
substances(proteoglycans and glycoproteins)
Lamina Fusca:Lamina fusca is the innermost layer of sclera. It is
characterised by abundance of pigmented cells or melanocytes, mostly
migrated from choroid.
Conjunctiva Conjunctival vessels
Tenon’s Superficial
capsule episcleral plexus
Deep episcleral
plexus

SCLERA
SCLERITIS

Scleritis is an uncommon condition characterised by


oedema and cellular infiltration of the entire thickness of
the sclera.
TYPES

Immune mediated Infectious


Immune -Mediated Scleritis

Immune mediated (non-infectious) scleritis is the most common


type
Frequently associated with an underlying systemic
inflammatory condition
Comprises a spectrum from trivial and self-limiting disease to a
necrotizing process that can involve adjacent tissues and
threaten vision.
CLASSIFICATION
NON INFECTIOUS SCLERITIS

ANTERIOR POSTERIOR

NON - NECROTIZING NECROTIZING

DIFFUSE NODULAR WITH WITHOUT


INFLAMMATION INFLAMMATION
Anterior non-necrotizing scleritis
Diffuse
Diffuse disease is slightly more common in females and usually presents in the
fifth 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 and
improves later in the day
responds poorly to common analgesics.
Signs
Vascular congestion and dilatation associated with oedema - redness
may be generalized or localized to one quadrant.
Secondary features can include chemosis, eyelid swelling, anterior
uveitis and raised IOP.
As the oedema resolves slight grey/blue appearance because
of increased scleral translucency
Recurrences at the same location are common unless an underlying
cause is treated.
Nodular
The incidence is more in persons with previous attack of herpes
zoster ophthalmicus.
Symptoms
The insidious onset of pain increasing redness
Tenderness of the globe and the appearance of a scleral nodule.
Signs
Scleral nodules may be single or multiple and most frequently
develop in the interpalpebral region close to the limbus
Deeper blue–red colour than episcleral nodules and are immobile.
Slit lamp beam shows an elevated anterior scleral surface(both
anterior and posterior slit beams are elevated)
Instillation of 10% phenylephrine drops will constrict the conjunctival
and superficial episcleral vasculature but not the deep plexus overlying the
nodule.
As the inflammation in the nodule subsides, increased translucency of the
sclera becomes apparent.
More than 10% of patients with nodular scleritis develop
necrotizing disease, but if treatment is instituted early
superficial necrosis does not occur and the nodule heals
from the centre leaving a small atrophic scar.
Anterior necrotizing scleritis with inflammation
Aggressive form of scleritis.
The age at onset - average 60 years.
Bilateral in 60% of patients
May result in severe visual morbidity and even loss of the eye.
Clinical features

Symptoms
Gradual onset of pain that becomes severe and persistent and
radiates to the temple, brow or jaw
Frequently interferes with sleep and responds poorly to analgesia.
Anterior necrotizing scleritis with inflammation

VASO-OCCLUSIVE GRANULOMATOUS SURGICALLY


NECROTIZING NECROTIZING INDUCED
SCLERITIS SCLERITIS NECROTIZING
SCLERITIS
VASO-OCCLUSIVE NECROTIZING SCLERITIS

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
GRANULOMATOUS NECROTIZING SCLERITIS

Occurs in conjunction with conditions


such as granulomatosis or polyarteritis
nodosa.
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
SURGICALLY INDUCED NECROTIZING SCLERITIS

Typically starts within 3 weeks of a procedure


It may be induced by any type of surgery including
Strabismus repair
Trabeculectomy
Scleral buckling
Excision of pterygium with adjunctive mitomycin C
The necrotizing process starts at the site of surgery and extends
outwards, but tends to remain localized to one sector.
Scleromalacia perforans

Specific type of progressive scleral thinning without inflammation that


typically affects elderly women with long standing rheumatoid arthritis
Perforation of the globe is extremely rare as integrity is maintained by a thin
layer of fibrous tissue.

Symptoms
Mild non-specific irritation pain is absent and vision unaffected
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
DIFFERENTIAL DIAGNOSES
Conjunctivitis usually can be differentiated from scleritis by the presence
of discharge, superficial inflammation, and the lack of severe aching or
pain.
Episcleritis may sometimes be confused with scleritis, although the two
conditions can usually be differentiated based on careful history and
clinical examination.
Ciliary flush (injection) that accompanies acute iritis may be confused
with scleritis. However, the ciliary flush is usually restricted to the area
adjacent to the limbus, and iritis appears to be the predominant finding.
Posterior scleritis

Potentially blinding condition in which diagnosis is commonly


delayed, with an adverse prognostic effect.
The inflammatory changes in posterior and anterior scleral disease
are identical and can arise in both segments simultaneously or
separately.
The age at onset is often less than 40 years
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%.
Choroidal folds are usually confined to the posterior pole and
orientated horizontally Exudative retinal detachment occurs in
around 25%
Yellowish-brown subretinal exudative material can be mistaken
for a choroidal tumour
Uveal effusion with choroidal detachment may be present
Disc oedema
Myositis is common and gives rise to diplopia, pain on eye
movement, tenderness to touch and redness around a muscle
insertion.
Proptosis is usually mild and is frequently associated with ptosis.
Occasional features include raised IOP, periorbital oedema and
chemosis.
• Ultrasonography - 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
SYSTEMIC ASSOCIATIONS

Rheumatoid arthritis
Most common systemic association of scleritis
Characterized by a symmetrical deforming inflammatory polyarthropathy,
with a spectrum of possible extra-articular manifestations.
Presentation is commonly in the third decade with joint swelling, usually of
the hands.
More common in females than males.
Rheumatoid factor auto antibodies are present in 80–90%.
All forms of immune-mediated scleritis have been described in RA
Other ocular manifestations of RA include keratoconjunctivitis sicca
(secondary Sjögren syndrome), ulcerative keratitis and acquired superior
oblique tendon sheath syndrome.
Wegener granulomatosis (granulomatosis with polyangiitis)
Idiopathic multisystem granulomatous disorder characterized by small vessel
vasculitis typically affecting primarily the paranasal sinuses, lower respiratory
tract and the kidneys.
There is a male predominance.
Presentation is in the fifth decade on average, often with pulmonary
symptoms.
Antineutrophil cytoplasmic antibodies (cANCA) are found in over 90% of
patients with active disease.
Scleritis is often rapidly progressive, necrotizing and granulomatous.
Other ocular manifestations include peripheral ulcerative keratitis,
occlusive retinal vasculitis, orbital inflammatory disease, nasolacrimal
obstruction, dacryocystitis and, rarely, tarsal–conjunctival disease.
Relapsing polychondritis
Rare idiopathic condition characterized by small vessel vasculitis
involving cartilage resulting in recurrent, often progressive,
inflammatory episodes involving multiple organ systems such as the ears,
respiratory system, heart and joints.
Scleritis is often intractable and may be necrotizing or non-necrotizing.
Isolated anterior uveitis may also occur.
Polyarteritis nodosa
Idiopathic aneurysmal vasculitis affecting medium-sized and small
arteries, with a wide range of manifestations across multiple organ
systems.
Presentation is in the third to sixth decades, often with
constitutional symptoms.
The male :female ratio is about 3:1.
Ocular involvement may precede the systemic manifestations by
several years.
About a third of patients have hepatitis B infection.
Scleritis is often aggressive and necrotizing.
Peripheral ulcerative keratitis, orbital pseudo tumour and occlusive
retinal periarteritis can also be seen
Investigations
Erythrocyte sedimentation rate (ESR),
C-reactive protein (CRP),
Full blood count (e.g. anaemia related to inflammatory connective tissue disease,
eosinophilia for polyarteritis nodosa, atopy or Churg–Strauss syndrome),
Rheumatoid factor,
Antinuclear antibodies (ANA),
Antineutrophil cytoplasmic antibodies (ANCA) and anti-cyclic citrullinated peptide
(CCP) antibodies
Serum uric acid
Syphilis serology
Lyme serology
Hepatitis B surface antigen (polyarteritis nodosa)
Antiphospholipid antibodies
Investigation for tuberculosis, sarcoidosis or ankylosing spondylitis

Radiological imaging.
Chest, sinus, joint and other imaging may be indicated in the
investigation of a range of conditions such as tuberculosis, sarcoidosis,
Churg–Strauss syndrome, Wegener granulomatosis, ankylosing
spondylitis and other conditions.
Treatment of immune-mediated scleritis
Topical steroids
Do not affect the natural history of the scleral inflammation, but may
relieve symptoms and oedema in non-necrotizing disease.
Systemic NSAIDs
Should be used alone only in non necrotizing disease
Systemic steroids
(e.g. prednisolone is 1–1.5 mg/kg/day) are used when NSAIDs are
inappropriate or inadequate (necrotizing disease).
Intravenous methylprednisolone may be used for emergent cases.
Immunosuppressives and/or biological blockers
Considered if control is incomplete with steroids alone, as a steroid-
sparing measure in long-term treatment or for underlying systemic
disease.
SYSTEMIC NSAIDS

Indomethacin 50 mg three times a day or, in the


sustained-release form, 75 mg twice a day
Piroxicam 20 mg daily
Ibuprofen, naproxen, tolmetin, sulindac
SYSTEMIC STEROIDS
Systemic corticosteroids -moderate to severe scleritis.
The usual starting dose is 1 mg/kg/day of prednisone but, in severe
cases, doses up to 1.5 mg/kg/day may be required.
The prednisone is then slowly tapered to a best-tolerated dose
Pulse intravenous methylprednisolone at 0.5–1 g may be required in
some patients with severe scleritis.
IMMUNOSUPPRESSIVE THERAPY
Required in patients with scleritis who are unresponsive to
or intolerant of prednisone, or who require long-term
therapy
Oral or subcutaneous methotrexate (7.5–25 mg weekly)
has been reported to be of benefit in reducing or
eliminating the need for systemic corticosteroid therapy.
Azathioprine at a dose of 1.5–2day also may reduce or
eliminate the need for corticosteroids.
Mycophenolate mofetil- lower toxicity and higher efficacy.
Cyclosporine- At doses of 10 mg/kg/day it is nephrotoxic,
so it is almost always used at lower doses, such as 5
mg/kg/day as an initial dose and 3–5 mg/kg/day as a
maintenance dose
SURGICAL

Surgery for scleritis may be performed when scleral


perforation or extensive thinning exists with significant risk for
scleral rupture
Reinforcement of the sclera- available agents include fresh or
preserved donor sclera, periosteum, or fascia lata.
INFECTIOUS SCLERITIS

Infection may follow surgical or accidental trauma, endophthalmitis, or may


occur as an extension of corneal infection.
Causes
Herpes zoster is the most common infective cause. Necrotizing scleritis is
extremely resistant to treatment and may result in a thinned or punched-out
area
Tuberculous scleritis is rare and difficult to diagnose. The sclera may be
infected by direct spread from a local conjunctival or choroidal lesion, or
more commonly by haematogenous spread. Involvement may be nodular or
Leprosy- Recurrent necrotizing scleritis can occur, even after
apparent systemic cure.
Nodular disease may be seen in lepromatous leprosy.
Syphilis-Diffuse anterior scleritis may occur in secondary syphilis,
and occasionally scleral nodules may be a feature of tertiary syphilis
Treatment
Specific antimicrobial therapy
Topical and systemic steroids may also be used to reduce the
inflammatory reaction.
If appropriate, surgical debridement can be used to debulk a
focus of infection and facilitates the penetration of antibiotics.
Staphyloma
Definition

A staphyloma is a clinical condition characterized by an


ectasia of the outer coats of the eye with an incarceration
of the uveal tissue
CLASSIFICATION (based on location)

1) Anterior
2) Intercalary
3) Ciliary
4) Equatorial
5) Posterior
ANTERIOR STAPHYLOMA
Involving the anterior ⅙ th of the outer coat- CORNEA
Associated with ectasia of cornea or iris
Due to perforating corneal ulcer or injury
Partial or total depending on which part of cornea is involved
Lined internally by the iris and externally by the pseudocornea.
INTERCALARY STAPHYLOMA
Located at the limbus
Lined by the root of the iris and anterior part of the ciliary body
Seen externally from limbus to 2mm behind the limbus
Caused by weakening of the globe at the limbus
Perforating injuries of peripheral cornea, marginal corneal ulcer, anterior
scleritis,
Scleromalacia perforans, poor wound opposition in cataract surgery.
TREATMENT
Local excision and repair with corneal and scleral patch graft
Cosmetic disfigurement - staphylectomy and keratoplasty or
enucleation
CILIARY STAPHYLOMA
Affects the ciliary zone that includes the region upto 8mm behind the
limbus
Ciliary body is incarcerated in the region of the scleral ectasia
Blue colour with a lobulated surface
Causes- developmental glaucoma, scleritis, trauma
EQUATORIAL STAPHYLOMA
At the equatorial region of the eye with incarceration of the choroid
Approximately 14mm behind the limbus
At the region of sclera which are perforated by vortex veins
Causes : scleritis, degeneration of sclera in pathological myopia
POSTERIOR STAPHYLOMA
Affects the posterior pole of the eye and is lined by choroid.
Causes : Pathological myopia, posterior scleritis, perforating injuries
Up to 50% of patients with pathologic myopia are reported to have a
staphyloma
Congenital disease associations include any condition causing defects
in Bruch’s membrane, such as retinitis pigmentosa, Alport’s
syndrome, pseudoxanthoma elasticum, and tilted-disc syndrome
Pathophysiology

Local choroidal factors and a decreased resistance of the sclera leading to


a protruding Bruch’s membrane can result in staphylomas.

Myopic eyes have increased elasticity due to its longer axial length, which
causes it to expand and gradually thin to form outpouchings

Secondary etiologies such as trauma or infection can disrupt the structure


of the sclera, placing the injured region at risk for subsequent scleral
thinning
Fundus: Posterior outward curvature of the globe- Crescentic shadow
in the macular region
Retinal vessels change direction and dip down into the region
B-scan to confirm diagnosis
Treatment
Currently no gold standard treatment of staphyloma due to a generally
unclear pathogenesis.
Monitor progression of the staphyloma.
Many do not require treatment and remain stable over time.
If there is observed thinning of the sclera- posterior scleral reinforcement
(PSR).
Involves the placement of a graft in the posterior aspect of the fundus to
prevent further progression of staphylomas- controversial treatment
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