PERIPHERAL INFLAMMATORY CORNEAL

DISEASE (NON-INFECTIOUS KERATITIS)

BY :MOHAMMED S.

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WHY PERIPHERAL CORNEA IS BEING A
TARGET SITE FOR SYSTEMIC DISEASE?
 Contiguity with the limbal vasculature

 IG & complements reach from the conjunctiva

 Prominent site of reservoir inflammatory cells( the of

Langerhans cells )

Such unique anatomical and physiologic features allow the

peripheral cornea to be the target of various local and
systemic immune mediated disease.
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NON-INFECTIOUS KERATITIS
 Corneal inflammation with no known infectious cause.

 Diverse group of diseases with corneal inflammation as the

common feature.

 It is characterized by persistent corneal epithelial defects,

stromal inflammation, and enzymatic degradation of the corneal
collagen.

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ETIOLOGY
 Local and systemic immune –mediated disease
 Systemic infections

 Dermatologic
 Mechanical

 Trauma
 Postsurgical

 Nutrition
 Lacrimal

 Neurologic 4
 Idiopathic
ETIOLOGICAL CLASSIFICATION
 Local immune –mediated disease
 Marginal keratitis

 Phlylctenulosis
 VKC

 Graft-versus-hostdisease
 Atopic keratoconjunctivitis

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CONT…
 Neurologic
 Neurotrophic keratitis
 Neuroparalytic keratitis

 Nutritional
 Keratomalacia

 Keratitis associated with diseases of skin

 Rosaceakeratitis
 Mucous membrane pemphigoid
 Erythema multiforme

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CONT…

 Keratitis associated with Systemic immune-Mediated

disease (PUK)

 Keratitis associated with trauma

 Photokeraitis

 Chemical burn

 Thermal burn

 Mechanical

 Keratitis associated with local & systemic Infections

( HSV,TB,syphilis…)
 Interstitial keratitis
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CONT…
 Mechanical
 Dellen
 Exposure keratitis
 Lacrimal
 Keratoconjunctivitis sicca (primary, secondary)

 Idiopathic
 Mooren’s ulcer
 Thygeson Superficial Punctate Keratitis
 Superior limbic keratoconjunctivitis

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MARGINAL KERATITIS
 It is a bacterial hypersensitivity-mediated corneal disease

 Characterized by gray -white, well circumscribed, marginal

infiltrates and located approximately 1 mm inside the limbus

 Occur where the eyelid margins intersect with corneal surface:

10, 2, 4, and 8 o'clock positions.

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CONT…
 In chronic disease, superficial blood vessels may cross the
clear interval

 The epithelium overlying marginal infiltrates may be intact,

show punctate epithelial erosions, or be ulcerated.

 Finally ,Stromal opacification, peripheral corneal thinning,

and/or Pannus may develop

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PREDISPOSING FACTORS

 Blepharoconjunctivitis
 Contact lens wear

 Trauma

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TREATMENT
 Treat the underline cause

 Weak topical steroid such as prednisolone 0.5% q.i.d. for

1 week combined with a topical antibiotic for
symptomatic disease

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PHLYLCTENULOSIS
 Phlylctenulosis is usually a self-limiting disease

 Characterized as a small white nodule associated with intense

local hyperaemia on the conjunctiva or limbus

 A limbal phlycten may then extend progressively onto the

Cornea.

 A healed corneal phlycten leaves a triangular limbal-based scar

associated with superficial vascularization and thinning .

 Spontaneous resolution usually occurs within 2–3 weeks, 14

CONT…
 Associated with
 Hypersensitivity reaction to staphylococcal antigen,
 Tuberculosis or
 Helminthic infestation

RX
 A short course of topical steroid accelerates healing.

 Recurrent disease may require an oral tetracycline.

 It is also important to treat associated chronic blepharitis

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ROSACEA KERATITIS
 Corneal ulceration is seen in about 10 percent cases of
acne rosacea, which is primarily a disease of the
sebaceous glands of the skin.

 Typically occurs in elderly women in the form of facial

eruptions presenting as butterfly configuration,
predominantly involving the malar and nasal area of face

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C/F
 Inferior PEE
 Peripheral vascularization

 Marginal infiltrates (inferonasal and inferotemporal)

 Circumferential spread.

 Corneal thinning (located inferiorly)

 Perforation as result of severe peripheral or central melting.

 Corneal scarring and vascularization

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TREATMENT

 The most effective treatment of rosacea keratitis is a long

course of systemic tetracycline (250 mg QID × 3 weeks,
TDS × 3 weeks, BID × 3 weeks, and once a day for 3
months).

 Topical steroids

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 DELLEN
 Dellen is caused by localized tear tilm instability which may be
idiopathic or secondary to raised limbal lesions or contact lens
wear

 Characterized by localized saucer-like thinning of the

peripheral cornea, with dehydration of the corneal stroma
compaction of its lamellae.

 Treatment involves eliminating causative pathology promoting

corneal rehydration by padding and to lubricants.

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THYGESON SUPERFICIAL PUNCTATE KERATITIS
 It is an uncommon, bilateral recurrent condition of unknown
aetiology.

 Characterized by round or oval conglomerate of

distinct,granular,greyish,elevated.punctate epithelial lesions
with ocular irritation and watering.

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EXPOSURE KERATITIS

 Normally, cornea is covered by eyelids during sleep and

is constantly kept moist by blinking movements during
awaking.

 When eyes are covered insufficiently by the lids and

there is loss of protective mechanism of blinking the
condition of exposure keratopathy (keratitis
lagophthalmos) develops.

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CAUSES
 Extreme proptosis
 Bell's palsy or any other cause of facial palsy.

 Ectropion of severe degree .

 Symblepharon causing lagophthalmos.

 Deep coma associated with inadequate closure of lids.

 Physiological lagophthalmos.

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CLINICAL FEATURES
 Fine punctate epithelial keratitis which is followed by
necrosis, frank ulceration and vascularization

 Bacterial superinfection may cause deep suppurative

ulceration which may even perforate.

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MANAGEMENT
 Prophylaxis.
 Artificial tear eye drops.

 Ointment and closure of lids by a tape or bandage during

sleep.

 Softbandage contact lens

 Treatment of cause of exposure

 Treatment of corneal ulcer

 Tarsorrhaphy

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NEUROPARALYTIC KERATITIS
 Neuroparalytic keratitis occurs due to paralysis of the
sensory nerve supply of the cornea.

 Interruption of corneal innervation results in depletion of

substance P, a neurogenic chemical known to regulate corneal
physiological functions.

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 CAUSE

Congenital
 Familial dysautonomia (Riley-
Day syndrome)
 Congenital insensitivity
 Anhidrotic ectodermal
dysplasia.
Acquired
 Highly acholol consumption
 A neoplasm
 Acute infection of ganglion
 Syphilitic (luetic) neuropathy.
 leprosy.

 Injury to Gasserian ganglion.

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CLINICAL FEATURES
 Painless , no lacrimation, and complete loss of corneal
sensations.

 Ciliary congestion is marked.

 Corneal sheen is dull.

 PEE in the inter-palpebral area followed by ulceration due to

exfoliation of corneal epithelium.

 Relapses are very common, even the healed scar quickly

breaks down again
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TREATMENT
 Initial treatment with antibiotic and atropine eye ointment
with patching is tried.

 Healing is usually very slow.

 Topical nerve growth factor drops and amniotic membrane

transplantation.

 Lateral tarsorrhaphy (relapsing )

 Prolonged use of artificial tears is also recommended

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INTERSTITIAL KERATITIS
 Interstitial keratitis denotes an inflammation of the
corneal stroma without primary involvement of the
epithelium or endothelium

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CAUSES
 Syphilis
 Tuberculosis

 Cogan's syndrome

 Trypanosomiasis
 Malaria

 Leprosy
 Sarcoidosis 34
SYPHILITIC (LUETIC) INTERSTITIAL KERATITIS

 Associated more frequently (90 percent) with congenital

syphilis than the acquired syphilis.

 Generally bilateral in inherited syphilis and unilateral in

acquired syphilis.

 In congenital syphilis, manifestations develop between

5-15 years of age.

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PATHOGENESIS
 Now generally accepted that the disease is a
manifestation of local antigen-antibody reaction

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CLINICAL FEATURES
 Interstitial keratitis characteristically forms one of the
late manifestations of congenital syphilis.(Hutchinson's
triad)

 Interstitial
keratitis
 Hutchinson's teeth and
 vestibular deafness.

 The C/F of IK can be divided into three stages:

 Initial progressive stage,
 Florid stage and

 Stage of regression

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(A)Saddle-shaped nasal deformity; (B) sabre’ tibia; (C) 38
Hutchinson teeth
C/F
 Initial progressive stage
 Endothelial &stromal oedema
 Corneal haze (ground glass appearance.)
 Anterior uveitis
 KPs associated with pain, lacrimation, photophobia, blepharospasm
and circumcorneal injection
 Florid stage
 Deep brush-like vessels
 Superficial vascularization

 This stage lasts for about 2 months

 Stage of regression
 Ghost vessels.
 This stage may last for about 1 to 2 years.
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(A) ‘Salmon patch’; (B) ‘ghost vessels’ in inactive disease; (C) intrastromal
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corneal haemorrhage from re-perfused vessels; (D) patchy residual scarring
DIAGNOSIS

 Clinical
 Lab
 A positive VDRL or Treponema pallidum immobilization test
confirms the diagnosis

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RX
 Local treatment
 Topical corticosteroid drops e.g., dexamethasone 0.1% drops
every 2-3 hours
 Atropine eye ointment 1 percent 2-3 times a day

 Keratoplasty
 Systemic treatment
 Penicillin in high doses

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PUK ASSOCIATED WITH SYSTEMIC AUTOIMMUNE DISEASE

 Peripheral ulcerative keratitis (PUK) occurs association with

Systemic Immune-Mediated disease such as
 Vasculitides (RA,JRA,SLE,PAN…,)

 Inflammatory bowel disease (crohn’s disease,ulcerative

colitis)

 Blood dyscrasia(leukemia)

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PATHOGENESIS
 Circulating immune complex deposition, autoimmune
reactions to corneal antigens, and hypersensitivity
reactions to exogenous antigens have been proposed with
evidence suggesting that both humoral and cell-mediated
mechanisms (T cell and B cell) are involved

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CLINICAL PRESENTATION
 Symptoms of PUK are not specific.

 Foreign body sensation, pain, and photophobia are seen

 Crescentic ulceration and stromal infiltration at the limbus .

 Limbitis, episcleritis or scleritis

 Circumferentially and occasionally central spread; in contrast

to Mooren ulcer the process may also extend into the sclera.

 End-stage disease may result in a ‘contact lens’ cornea

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DIAGNOSIS
 Clinical
 Diagnostic & ancillary testing
 CBC
 RF

 ESR

 Urine analysis

 Radiographs
 Chest
 Sinus

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MANAGEMENT
 The goal of therapy is to provide local supportive
measures to decrease melting.

 This is achieved through

 Improving wetting,
 Promoting reepithelialization, and
 Suppressing the systemic-mediated inflammation.

 The degree of severity determines the RX option

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MEDICAL TREATMENT
 In milder, unilateral cases of PUK(not associated with a
systemic CVD)
 Topicalcorticosteroids
 Prophylactic topical antibiotics
 Lubricants

 Severe cases of PUK

 Systemic corticosteroids (oral prednisone 1 mg/kg/day )
 Systemic immunosuppressive agents
 Oral tetracycline such as doxycycline 100 mg bid
 Topical corticosteroids
 Topical lubricants (preservative free)
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INDICATIONS FOR SYSTEMIC
IMMUNOMODULATORY THERAPY
 PUK associated with a potentially lethal systemic
disease(RA,PAN,SLE…)

 PUK with associated scleritis

 Bilateral Mooren’s ulcer

 If the disease progress

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SYSTEMIC IMMUNOSUPPRESSORS

 Cyclophosphamide 1 to 2.5 mg/kg /day

WG and PAN (1st choice), RA (severe), RP (2nd choice)

 Cyclosporine (2 to 5 mg/kg/day)
Idiopathic (1st choice), RA, RP (2nd choice)

 Azathioprine (1 to 2 mg/kg/day)
RA, WG and RP (2nd choice)

 Methotrexate7.5 mg po once weekly or 2.5 mg every 12 hours for

3 doses/week or 15 mg IM once weekly
RA (1st choice), maintenance for WG

 Biological agents Unknown 51

 PREDNISONE
Adverse effect Precautions!!!!

 Cataract (PSC)  Antidiabetic agents:

 Glaucoma  Antifungal Agents

 Adrenal suppression  Ethanol

 Immunosuppression  Food

 Kaposi's sarcoma

 Myopathy

 Psychiatric disturbances

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SURGICAL MANAGEMENT
 Conjunctival excision
 Corneal gluing or amniotic membrane patching for perforation.

 Keratoplasty

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MOOREN ULCER
 It is a rare ,chronic, painful, idiopathic progressive
circumferential ulceration of the peripheral corneal stroma and
epithelium with later central spread.

 Patients with Mooren ulcer have deficiency of suppressor T

cells ,Increased level of IgA & concentration of plasma cells
and lymphocytes in the conjunctiva adjacent to the ulcerated
areas

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CLASSIFICATION
 Unilateral ulceration
 It is extremely painful

 slowly progressive ulceration

 Associated with ulceration of the superficial

juxtalimbal vascular plexus.
 Primarily affects elderly people

 No sex predilection

 usually responds well to medical therapy

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CONT….
 Bilateral aggressive ulceration
 Rapidly progressive circumferential ulceration with late
centripetal spread.
 Occurs primarily in young males.
 Less responsive to treatment.

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CLINICAL PRESENTATION

Symptoms
 Moderate-severe pain
 Photophobia
 Tearing
 Blurred vision

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SIGNS IN CHRONOLOGICAL ORDER:
 Peripheral ulceration involving the superficial 1/3 of the
stroma

 Progressive circumferential and central stromal thinning

 Vascularization involving the bed of the ulcer up to its

leading edge but not beyond

 No scleral involvement
 The healing stage is characterized by thinning,
vascularization and scarring
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COMPLICATIONS
 Severe astigmatism
 Perforation following minor trauma (spontaneous
perforation is rare)
 Secondary bacterial infection

 Cataract

 Glaucoma

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DIAGNOSIS
 Clinical
 Laboratory investigation

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 MANAGEMENT
Topical
 steroids combined with a low
frequency prophylactic topical antibiotic.

Artificial
 tears and collagenase inhibitors such
as acetylcysteine 10%.

Conjunctival
 resection, the excised area should
extend 4 mm back from the limbus and 2 mm
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beyond the margins of the lesion.
CONT…
 Systemic immunosuppression

 Options include Ciclosporin (5 mg/kg), prednisolone,

methotrexate and azathioprine.

 Systemic collagenase inhibitors such as doxycycline may

be beneficial.

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 Lamellar keratectomy
 SUMMARY
Suppurative keratitis Non-infectious keratitis

 Caused by active infection

 Due to immunological response
 Severe A/C rxn
 Mild A/C rxn
 Moderate –severe pain
 Mild except
 Central infiltrates
 Peripheral infiltrates
 Rapid progression
 Slow progression
 Purulent discharge
 Non-purulent discharge
 Epithelial defect-common
 Less common

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REFERENCES
 Yanoff
 BCSC

 Kanski (5th & 7 th edition )

 Comprehensive ophthalmology 4th edition

 Up todate 20.3

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Thank you

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