Episcleritis

Scleritis&

Panit cherdchu, M.D.

上強膜炎

強膜炎

REFERENCE参照

CONTENT• Review anatomy• Episcleritis vs Scleritis• Episcleritis in depth• Scleritis in depth• Take home message

コンテンツ

ANATOM

Y解剖学 ( レビュー )

Sclera• Type1 collagen and proteoglycans (decorin, biglycan

and aggrecan)• Elastin and glycoproteins (fibronectin)• Fibroblast lie along collagen bundles• Supply by long posterior ciliary nerves at anterior sclera• More translucent when thinning occurs or the water

content changes, falling below 40% or rising above 80%• Thinnest at insertion of rectus muscles

強膜

• Sensory innervation from the ciliary nerves – short posterior ciliary

nerves the posterior portions

– two long posterior ciliary nerves the anterior region.

• Extraocular muscles are inserted into the sclera

• Dull ache of scleral inflammation is made worse by ocular movement

• The sclera itself is avascular• Anteriorly– the anterior ciliary arteries

• Posteriorly– four vortex veins drain the choroidal circulation

Episcleritis

ScleritisVS

Panit cherdchu, M.D.

上強膜炎

強膜炎

Classificationepiscleritis scleritisSimple or diffuse Anterior scleritis

-nodular type-diffuse type-necrotizing with inflammation-necrotizing without inflammation (Scleromalacia Perforan)

Nodular Posterior scleritis

backgroundEpiscleritis scleritis

• Not very common condition

• Self-limiting• asymptomatic• No predominance of

male or female in adult• Predominance of male

in children

• Various presentation• 40-60 years of age• 25-50% of scleritis are

from systemic diseases• Affects more in women

than men (1.6:1)• May follows ocular

surgery

Pathogenesis and risk factorsEpiscleritis Scleritis

•Idiopathic, collagen vascular disease •Self-limited benign inflammation without destructive (not sight-threatening)

• Disordered immune response

• Immune complex deposition (type III hypersensitivity)

• Post surgery scleritis has 96% chance of presented necrotizing scleritis (cataract,pterygium Sx)

• Possibly from infection,trauma,endogenous substance

Episcleritis Scleritis•Abrupt -Days to weeks•Redness without irritation•Mild ocular discomfort

-Burning, FB sensation, irritation

•Localized

• Insidious – over several days•Pain, tenderness to palpation, radiate•Worse at night+awake

Clinical

Episcleritis上強膜炎

• Benign • Self-limiting condition• Simple or nodular• Not a mild version of scleritis• Adult (men=women)• Children (men>women) (1.6:1)

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• Acute onset in general• Nodular episcleritis is more insidious • Not much pain at all• May present as hot,prickly and FB like

sensation

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Physical examination• VA is usually not affected• Lid edema• Chemosis• Brick red injection, not violaceous• Normal vessels architecture• Topical phenylephrine blanch only

conjunctival vasculature and superficial episcleral capillary plexus

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Red brick

Chemosis

injection

Vessel engorgement

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SNodular

InjectionFaint brick red color

Displacing of reflected light

Clinical Course• Simple episcleritis is bilateral in 40% of cases• Nodular episcleritis is bilateral 13% of cases• Two classic presentation

– Peaks 24 after the onset of symptoms and slow improvement develops in 5-10 days, with complete resolution 2-3weeks

– Milder, with more prolonged attacks and no regularity to the intervals between attacks (associated with systemic diseases)

• Nodular episcleritis is more uncomfortable, solitary or multiple lesions which persists for 2 months

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Differential diagnosisEP

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Phlyctenular conjunctivitis

Conjunctivitis

Scleritis

pathology• Non-granulomatous inflammation with

vascular dilation and perivascular infiltration of lymphocytes and plasma cells

• Biopsy of episcleral nodules in RA is similar to subcutaneous rheumatoid nodules

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complication• Complications are uncommon• If high recurrent rate may lead to

Anterior Uveitis, Intermediate Uveitis, corneal change but these are rare

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etiology• Most are idiopathic• 26-36% have an associated disorder• Wegener’s granulomatosis ,Cogan’s

syndrome• Gout is certainly associated with

episcleritis

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Laboratory EvaluationEP

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Rheumatoid FactorAntinuclear AntibodySerum uric acidErythrocyte sedimentation rateComplete Blood count with differentialVDRL-FTAUrinalysisPPDChest X-ray film

treatment• May be not necessary• Episcleritis doesn’t progress to scleritis• Treat underlying diseases• Supportive therapy (cold compress, iced artificial tears)• Topical NSAIDs is effective• Amino-Thiazole hydrochloride 0.1% is effective• Topical steroids is dilemma

– Increased risk of recurrent episode– Rebound effect make episcleritis even worse– Start with FML qid is acceptable in just short course

• Oral NSAIDs should be kept for the last

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Scleritis強膜炎

• Divided into two major categories

– Non-infectious scleritis (immune-mediated disorders, vasculitides)

– Infectious scleritis (surgery, adjacent organ)

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Immune-mediated scleritis• Most common in 40-60 group of age• Women>men 1.6:1• Bilateral 52%• Scleritis have 25-50% chance of harboring a

systemic disease involved– Relapsing polychondritis has 14% chance of

acquired scleritis,– Wegener’s granulomatosis has 10%– IBS has 10%– Polyarteritis nodosa, RA have 6 %SCLE

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Pathogenesis and risk factors• Exact mechanisms are unknown • disordered immune response might play crucial roles

of scleritis• Interaction of genetically controlled mechanisms with

environmental factors or endogenous substances give rise to an autoimmune process that damages the episcleral and scleral perforating capillary and post capillary venules causing inflammatory microangiopathy

• Histopathologic studies suggest that immune complex deposition (type III hypersensitivity) SCLE

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• Scleritis, especially necrotizing scleritis, is more common in those with an underlying systemic vasculitis.

• 96% of patients with surgically-induced scleritis had necrotizing scleritis

• Scleritis has been reported as an adverse side effect of medications, primarily bisphosphonates

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Clinical finding• Watson and Hayreh classification

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• Diffuse anterior scleritis is the most common clinical presentation and is the least severe type of scleritis

• The sclera often takes on a violet, blue, or salmon color

• After resolution translucent or bluish-gray color

• An associated systemic disease may be found in 25-45% of patients with diffuse anterior scleritis, most commonly RASCLE

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• Nodular anterior scleritis presents with a nodule that is firm, immobile and tender to palpation

• Yellow or deep red

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• Of all, necrotizing anterior scleritis with inflammation (necrotizing scleritis) is the most severe and is associated with the greatest potential for visual loss

• Present in extreme discomfort and may be in severe distress

• Examination reveals white, avascular areas of sclera and conjunctiva, with surrounding scleral edema and congestion

• Uveal tissue may appear SCLE

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• Rapidly progressive necrotizing scleritis is often bilateral and associated with an acute exacerbation of systemic vasculitis

• Visual loss is common in necrotizing scleritis and probably more severe with underlying connective tissue disease or vasculitis (WG,RA,Relapsing Polychondritis)

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SCLE

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IS Necrotizing scleritisavascular areas

surrounding scleral edema and congestion

Markedly injected surrouding area

• Necrotizing scleritis without inflammation, also termed

scleromalacia perforans, is almost total lack of symptoms

• Patient may present with blurred vision because of astigmatism or may notice a discoloration of the sclera

• In necrotizing scleritis , only conjunctiva may cover prolapsed uvea, but perforation is uncommon without trauma

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Necrotizing scleritis without inflammation is most common in elderly women with

long-standing RA

Posterior scleritis• may be difficult to diagnose unless accompanied by anterior scleritis • The condition is under-diagnosed and frequently leads to sight-

threatening complications• About 20% of all cases of scleritis• About 30% of patients have systemic disease

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SCLE

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IS• Patients may complain of loss of vision, pain, or diplopia

and findings may include hyperopia, conjunctival chemosis, proptosis, lid edema or retraction, and ophthalmoplegia

• Posterior segments=optic nerve edema, optic neuritis, serous and exudative RD, ME, annular ciliochoroidal detachment and choroidal thickening, mass and folds, all commonly associated with visual loss

Associated systemic diseases

• Most commonly seen in patients with necrotizing scleritis and scleromalacia perforans

• The most common associated disease is RA, WG and relapsing polychondritis respectively

• Necrotizing scleritis in WG is a severe disease that can lead to permanent visual loss

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Rheumatoid ArthritisWegener’s granulomatosis

Relapsing Polychondritis

complications• Visual loss• Uveitis

– AU in necrotizing scleritis, PU in posterior scleritis• Keratitis, interstitial keratitis, PUK

– Adjacent peripheral cornea thinning• Glaucoma

– Transient elevated IOP during inflammatory episodes• Cataract

– PSC from inflammation and steroid Rx• Fundus abnormalities

– CME,optic nerve edema, RD, Choroidal foldsSCLE

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• Imaging study– Chest x-ray (TB,sarcoidosis)– Sacroiliac joints film – B-scan ultrasonography (posterior

scleritis)• Thickening of posterior sclera• “T” sign • Fluid in sub-Tenon space at the

junction of the optic nerve and globe

Diagnostic EvaluationSC

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“T” signSC

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SCLE

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ISdiffuse thickening of the choroid, sclera and episcleral tissues

The optic disc was swollen

fluid in the subtenon space producing a prominent T-sign

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Posterior scleral thickening with proptosis

CT orbit and brain

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therapy• NSAIDs oral• Topical steroids and topical NSAIDs• Systemic steroids (1mg/kg/day)• Subconjunctival injections of TA• Intraorbital injection of steroids• MP 2.5-3.0mg/kg IM• Systemic immunosuppressive agents• New biologic agents SCLE

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Infectious scleritis• Classified into two group

– Exogenous is most common (post-trauma,post-surgery) acute,suppurative, and destructive

– Endogenous is less common (syphilis, TB) resemble noninfectious diffuse, nodular, or necrotizing type

• Most commonly, the sclera is infected by migration of organisms from the adjacent cornea in cases of keratitis. But infections of the conjunctiva or orbital tissues almost never involve the scleraSCLE

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• Trauma can introduce organisms directly into the sclera in penetrating injuries

• Foreign Body that pass through the sclera may harbor organisms

• Systemic immunosuppression is a risk factor for infectious scleritisSCLE

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Clinical finding• Keratoscleritis• Panopthalmitis• Scleritis after scleral buckling surgery• Post-pterygium excision scleritis• Other postsurgical infectious scleritis• Herpetic scleritis• Syphilitic scleritis• Mycobacterium tuberculosis scleritisSCLE

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• Keratoscleritis– Most cases of infectious scleritis result from

severe bacterial infections of the cornea– Most are gram negative bacteria

esp.Pseudomonas aeruginosa– Staphylococcus aureus, Sterptococcus

pneumoniae, Mycobacterium chelonei, herpes simplex, herpes zoster, Aspergillus, Acremonium and Acanthamoeba

– Can be more severe in immunosuppressed patientsSC

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• Panophthalmitis– May result from progression of severe

endopthalmitis of any cause.– Most cases occur after trauma or surgery– First sign of scleral infection may be scleral

thinning, uveal prolapse, or scleral perforation.

– Poor visual prognosis

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• Scleritis after scleral buckling surgery– Pain and FB sensation– May develop immediately after surgery, or

years later– Causative genera include staphylococcus,

Pseudomonas, Proteus, and fungi

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• Post-pterygium excision scleritis– Rare– Usually associated with the use of

mitomycin-C or beta-irradiation at the excision site.

– Interestingly, infectious scleritis after pterygium surgery can have a long latency period (1-36 years)

– Pain, redness, scleral necrosis and purulent discharge

– P.aeruginosa is the most common organism in postsurgical infectious scleritisSC

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• Other postsurgical infectious scleritis– Cataract surgery– Strabismus surgery– Glaucoma filtering surgery– vitrectomy

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• Herpetic scleritis– Scleral involement in primary varicella

zoster can occur and may be associated with stromal keratitis

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• Syphilitic scleritis– Less than 5% of cases of scleritis in a

referral practice– Has been reported with the secondary,

tertiary, and congenital forms of the disease

– Usually have positive serologic tests for tremonema pallidum and usually respond to systemic penicillin or other appropriate antibiotics

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• Mycobacterium tuberculosis scleritis– May be asymptomatic or may have pain,

redness, and a mucopurulent discharge.

– Uveitis with corneal opacification and vascularization will often accompany tubercular scleritis

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Sign aiding in differential diagnosis

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investigation• C/S cornea • History taking • C/S of infected tissues should be performed• G/S, Giemsa stains should be obtained with placement

of scrapings on blood, chocolate and sabourad agar.• If TB is supectedAFB and cultures on Lowenstein-

Jensen Medium• If Acanthamoeba is suspected staining with calcofluor

white or specific Antibodies, and plating on non-nutrient agar over laid with E.coli

• Bx of inflammed sclera is not instantly suggestSCLE

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Therapy• Effective treatment of infectious scleritis usually requires

culture or pathologic evidence of a causative organism• TB IRZE (or streptomycin instead of ethambutol)• Herpes zoster scleritistopical or systemic

corticosteroids, Acyclovir • Syphilitic scleritissystemic penicillin therapy, systemic

or topical steroids• Fungal scleritisprolonged systemic and topial antifungal

therapy• Aspergillus flavus scleritis combination of voriconazole

and caspofunginSCLE

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• Bacterial scleritisbased on G/S,C/Sparenteral therapy is generally accepted

• Pseudomonas scleritis or sclerokeratitiscombination of IV caftazidime and aminoglycoside (tobramycin or gentamicin) + topical Fortified ATB can be effective

• Cryotherapy

• Aggressive surgical debridement of infected tissue with appropiate ATB therapy may further improve response

• Severe destruction of the cornea or scleral perforation may require lamellar or full-thickness grafting.SCLE

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TAKE HOME MESSAGE

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Classificationepiscleritis scleritisSimple Anterior scleritis

-nodular type-diffuse type-necrotizing with inflammation-necrotizing without inflammation (Scleromalacia Perforan)

Nodular Posterior scleritis

Episcleritis Scleritis•Abrupt -Days to weeks•Redness without irritation•Mild ocular discomfort

-Burning, FB sensation, irritation

•Localized

• Insidious – over several days•Pain, tenderness to palpation, radiate•Worse at night+awake

Clinical

EPIS

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S

Clinical finding• Watson and Hayreh classification

SCLE

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Infectious scleritis• Classified into two group

– Exogenous is most common (post-trauma,post-surgery) acute,suppurative, and destructive

– Endogenous is less common (syphilis, TB) resemble noninfectious diffuse, nodular, or necrotizing type

• Most commonly, the sclera is infected by migration of organisms from the adjacent cornea in cases of keratitis. But infections of the conjunctiva or orbital tissues almost never involve the scleraSCLE

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IS

• Diffuse anterior scleritis is the most common clinical presentation and is the least severe type of scleritis

• An associated systemic disease may be found in 25-45% of patients with diffuse anterior scleritis, most commonly RA

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“T” signSC

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Posterior scleritis

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