non infectious peripheral ulcerative keratitis [puk]
DESCRIPTION
NON INFECTIOUS PERIPHERAL ULCERATIVE KERATITIS [PUK]. A Clinical Approach DR. REKHA GYANCHAND Cornea consultant, Lions Eye Hospital BANGALORE. WHAT IS PUK ?. Potentially devastating Crescent shaped Juxtalimbal corneal stromal inflammation epithelial defect - PowerPoint PPT PresentationTRANSCRIPT
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NON INFECTIOUS PERIPHERAL ULCERATIVE KERATITIS [PUK]
A Clinical Approach
DR. REKHA GYANCHANDCornea consultant, Lions Eye HospitalBANGALORE
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WHAT IS PUK ?
Potentially devastating
Crescent shaped Juxtalimbal corneal stromal
inflammation epithelial defect Stromal infiltrate Progressive stromal melting
If untreated necrosis of entire cornea
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WHY IN PERIPHERAL CORNEA ?
Peripheral cornea - Unique anatomical & immunological features
Close to sclera / episclera / conjunctiva Limbal capillary Arcade Avascular central cornea Associated with sub conjunctival
lymphaticsafferent arm IgM in periphery large Langerhans cells Reservoir of inflammatory cells More susceptible to immunological damage
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PATHOPHYSIOLOGY OF DAMAGE IN PUKAny inflammatory stimulus in peripheral cornea local cellular & humoral response
complement activation vascular permeability
chemotactic factors for neutrophils (C3a , C5a) neutrophil invasion Inflamm Of Conj proteolytic ,collagenolytic enzymes,leucotrienes
destruction of collagen Collagenase
Corneal thinning
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CLINICAL PRESENTATION & DIAGNOSIS
50% Of non infectious PUK due to collagen vascular disease (SLE, RP, PSS, RA,WG, PAN, GCA)
34% of non infectious PUK caused by RA (Tauber et al)
PUK may be initial manifestation of WG & PAN
Moorens ulcer – local autoimmune disease with PUK
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CLINICAL PRESENTATION &DIAGNOSIS - II
PUK due to CVD more in females PUK due to Moorens more in males Other causes include
Neoplasia Rosaceae Surgical trauma Blepharitis Inflammatory bowel disease
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EXAMINATION IN PUK
Ocular
Systemic
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OCULAR EXAMINATION
Symptoms Pain, epiphora, photophobia pain if scleritis (RA, WG, PAN, RP) pain without scleritis ( Mooren’s) Decreased VA
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EXAMINATION
Examination of lids Blepharitis Telengiectasis (rosaecae)
Posterior segment examination Posterior scleritis Vasculitis of CVD
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SLIT LAMP EXAMINATION-CORNEA
Crescent Juxta limbal Epithelial defect Stromal yellow white
infiltrates Stromal thinning Circumferential /central
spread Adjacent scleral /
conjunctival inflammation
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SLIT- LAMP EXAMINATION SCLERA
Associated necrotising scleritis systemic disease
In advanced cases- corneal/scleral melt
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SYSTEMIC EXAMINATION Thorough systemic history & examination mandatory Important Questionnaire?
Weight loss, fatigue Skin – facial rashes, ulcers, periungual infarcts(SLE) Respiratory symptoms ( WG, SLE) GI symptoms- pain diarrhoea ( SLE, WG) Musculoskeletal symptoms- joint pain ( RA, SLE) Neurological – seizures, Raynauds (WG, RP, SLE)
Genitourinary- hematuria ( PAN, SLE) Swollen ear lobes (RP, SLE) Deafness (WG) Nasal ulcers/ bleeds ( WG) Saddle nose ( WG, RP)
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Differential Diagnosis Of PUK
Other Non Inflammatory Progressive Peripheral Thinning: Terriens marginal degeneration Pellucid marginal degeneration
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TERRIENS MARGINAL DEGENERATION
Progressive , non inflam. thinning
No symptoms, V/A Painless Corneal epith intact Begins superiorly No stromal infiltration Lipid deposition Occasional adjacent
conjunctival or scleral inflammation present
Can perforate
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Bilateral,painless Inferior corneal crescent
thinning Progressive Clear zone of cornea Epithelium intact Adjacent conjunctiva no
inflammation Corneal ectasia above thinning High against the rule
astigmatism Corneal topography
PELLUCID MARGINAL DEGENERATION
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Laboratory Investigations For Non Infectious PUK
CBC ESR CRP URINE ANALYSIS RF ANA (SLE/RA) C ANCA (96% WG) ANTI-ds DNA(SLE) C3/C4 LEVELS CIRCULATING IMMUNE
COMPLEXES
CHEST X-RAY
SINUSES (X-RAY / CT SCAN)
HEPATITIS B,C Ag
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LOCAL INVESTIGATIONS
Corneal scraping/culture Conjunctival biopsy
Removes source of collagen Diagnosis of CVD fibrinoid
necrosis,granulomas,vasculitis Diagnosis of Moorens justifies immune
suppression in occult systemic disease
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Treatment of Non Infectious PUK
THERAPY
MEDICAL SURGICAL
LOCAL SYSTEMIC
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LOCAL THERAPY Goals
Promote epithelial healing– stromal thinning
Control of inflammation Collagenase inhibition– stromal thinning
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Promote epithelial healing
• Lubricating drops, gels Avoid epitheliotoxic drugs ( aminoglycosides – tobra, genta;
fluroquinolones—ciprofloxacin) No role of topical antibiotics /
antifungals unless secondary infection
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No role of topical steroids/ NSAIDS ( inhibits collagen synthesis—increases melt) Use topical 1% medroxy progesterone (good anti inflammatory, no collagen synthesis
inhibition) Can use topical cyclosporine 0.5- 1% ( local T cell immune modulation) Low dose topical steroids Lid hygeine only in
marginal infiltrates with blepharitis ( staph antigen)
Control of inflammation
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Other local medical treatment Blepharitis: lid hygeine Rosaecae: erythromycin ointment;
metronidazole
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SYSTEMIC THERAPY
Systemic collagenase inhibitors Tetracycline 250 mg QID Doxycycline 100 mg OD Systemic steroids + cytotoxic
immunosuppressives
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INDICATIONS FOR IMMUNE SUPPRESSION
PUK associated with proven CVD like RA, PAN, RP, WG, PSS, GCA, Churg-strauss angitis
If PUK associated with necrotising scleritis
If PUK unresponsive to aggressive conventional medical or surgical therapy
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DRUGS USED
High dose oral prednisolone 1- 1.5 mg / kg BW or
Pulsed IV methyl prednisolone ( 0.5- 1g) started first as cytotoxic immunosuppression takes 4 – 6 weeks for action
Drug of choice– oral cyclophosphamide ( 2 mg/ kg / day) adjust to clinical response, adverse effects
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DRUGS USED
Methotrexate, azathioprine, cyclosporine-A
Methotrexate : DOC in RA ( 7.5- 12.5 mg / wk)
Azathioprine: 1.3 mg/kg/day Cyclosporine-A:2.5-5mg/kg/day Monitor CBC,LFT,renal function tests Role of immunologist important Good patient education: long term follow
up systemic nature of disease
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Surgical treatment
Tissue adhesives ( cyano acrylate glue ) + BSL
Impending perforation / large thinning/ perforation size < 1-2mm
Delays disease process while patient is on immunosuppressives
infiltration of inflammatory cells
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Surgical treatment
Conjunctival resection + superficial keratectomy + glue + BCL
Removes source of cytokines / inflammatory cells
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Tectonic lamellar / full thickness corneal / scleral graft – Large Perforation w/ Uveal Prolapse
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Simultaneous systemic immunosuppression very important or graft will also melt
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LONG TERM MANAGEMENT
Local disease healed• No inflammation• Epithelium intact• Vascularised Corneal
pannus
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LONG TERM MANAGEMENT
Long term follow up as relapses Prolonged systemic immune suppression till
underlying disease controlled even if EQ Residual astigmatic correction– increases VA Combination of LK +PK for visual
rehabilitation done with full immunosuppression as surgery can trigger relapse
Cataract surgery when systemic disease under control & under systemic steroids
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MOORENS ULCER
Distinct clinical entity in PUK
PUK not associated with CVD
? Local auto immune disorder (altered corneal Ag)
? Role of hepatitis C Ag
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Distinguishing features
PUK unilateral / bilateral Pain out of proportion No scleritis Typical overhanging
central edge More aggressive and early
conjunctival resection and keratectomy advisable
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Glue + BCL – if impending perforation & increased thinning
Systemic steroids and immuno suppressives only if b/l moorens nonresponsive to local
therapy Cyclophosphamide,
methotrexate : DOC If Hep C Ag + :
interferon alpha 2b ( 3 million units tri weekly SC inj – for 6 months
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Conclusion..
Non Infectious PUK is a potentially devastating disorder, can be the initial presentation of a serious collagen vascular disorder. Hence proper diagnosis and aggressive therapy could improve local and systemic morbidity.
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THANK YOU!