Scleritis: Moderator: DR Sangeetha Patil Presenter: DR Shraddha Sudarshan
Scleritis: Moderator: DR Sangeetha Patil Presenter: DR Shraddha Sudarshan
Scleritis: Moderator: DR Sangeetha Patil Presenter: DR Shraddha Sudarshan
SCLERA
SCLERITIS
ANTERIOR POSTERIOR
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
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
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
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
Myopic eyes have increased elasticity due to its longer axial length, which
causes it to expand and gradually thin to form outpouchings
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