Episcleritis and scleritis

update in Episcleritis and scleritis
ophthalmology department of Phramongkutklao Hospital

1. E piscleritis S cleritis & Panit cherdchu , M.D . 上強膜炎 強膜炎

2. R EFERENCE 参照

3. CONTENT R eview anatomy E piscleritis vs Scleritis E piscleritis in depth S cleritis in depth T ake home message コンテンツ

4. ANATOMY 解剖 学 ( レビュ ー )

5. 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

6. 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

7. The sclera itself is avascular Anteriorly the anterior ciliary arteries Posteriorly four vortex veins drain the choroidal circulation

8. E piscleritis S cleritis VS Panit cherdchu , M.D . 上強膜炎 強膜炎

9. Classification episcleritis scleritis Simple or diffuse Anterior scleritis -nodular type -diffuse type -necrotizing with inflammation -necrotizing without inflammation ( Scleromalacia Perforan ) Nodular Posterior scleritis

10. background Episcleritis 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

11. Pathogenesis and risk factors Episcleritis 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

12. 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

13. E piscleritis 上強膜炎

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

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

16. 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 EPISCLERITIS

17. EPISCLERITIS Red brick Chemosis injection Vessel engorgement

18. EPISCLERITIS Nodular Injection Faint brick red color Displacing of reflected light

19. 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 EPISCLERITIS

20. Differential diagnosis EPISCLERITIS

21. 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 EPISCLERITIS

22. complication Complications are uncommon If high recurrent rate may lead to Anterior Uveitis, Intermediate Uveitis, corneal change but these are rare EPISCLERITIS

23. etiology Most are idiopathic 26-36% have an associated disorder Wegener’s granulomatosis ,Cogan’s syndrome Gout is certainly associated with episcleritis EPISCLERITIS

24. EPISCLERITIS

25. EPISCLERITIS

26. EPISCLERITIS

27. Laboratory Evaluation EPISCLERITIS Rheumatoid Factor Antinuclear Antibody Serum uric acid Erythrocyte sedimentation rate Complete Blood count with differential VDRL-FTA Urinalysis PPD Chest X-ray film

28. 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 EPISCLERITIS

29. S cleritis 強膜炎

30. Divided into two major categories Non-infectious scleritis (immune-mediated disorders, vasculitides ) Infectious scleritis (surgery, adjacent organ) SCLERITIS

31. 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 % SCLERITIS

32. 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) SCLERITIS

33. 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 SCLERITIS

34. Clinical finding Watson and Hayreh classification SCLERITIS

35. SCLERITIS

36. 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 RA SCLERITIS

37. Nodular anterior scleritis presents with a nodule that is firm, immobile and tender to palpation Yellow or deep red SCLERITIS

38. 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 SCLERITIS

39. 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 ) SCLERITIS

40. SCLERITIS Necrotizing scleritis avascular areas surrounding scleral edema and congestion Markedly injected surrouding area

41. 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 SCLERITIS

42. SCLERITIS Necrotizing scleritis without inflammation is most common in elderly women with long-standing RA

43. 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 SCLERITIS

44. SCLERITIS 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

45. 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 SCLERITIS

46. SCLERITIS Rheumatoid Arthritis Wegener’s granulomatosis Relapsing Polychondritis

47. 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 folds SCLERITIS

48. 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 Evaluation SCLERITIS

49. “T” sign SCLERITIS

50. SCLERITIS diffuse thickening of the choroid, sclera and episcleral tissues The optic disc was swollen fluid in the subtenon space producing a prominent T-sign

51. SCLERITIS Posterior scleral thickening with proptosis CT orbit and brain

52. SCLERITIS

53. 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 SCLERITIS

54. SCLERITIS

55. 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 sclera SCLERITIS

56. SCLERITIS

57. 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 scleritis SCLERITIS

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

59. 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 patients SCLERITIS

60. SCLERITIS

61. 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 SCLERITIS

62. 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 SCLERITIS

63. 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 scleritis SCLERITIS

64. Other postsurgical infectious scleritis Cataract surgery Strabismus surgery Glaucoma filtering surgery vitrectomy SCLERITIS

65. Herpetic scleritis Scleral involement in primary varicella zoster can occur and may be associated with stromal keratitis SCLERITIS

66. 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 SCLERITIS

67. 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 SCLERITIS

68. Sign aiding in differential diagnosis SCLERITIS

69. 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 supected AFB 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 suggest SCLERITIS

70. 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 scleritistopical or systemic corticosteroids, Acyclovir Syphilitic scleritissystemic penicillin therapy, systemic or topical steroids Fungal scleritisprolonged systemic and topial antifungal therapy Aspergillus flavus scleritis  combination of voriconazole and caspofungin SCLERITIS

71. SCLERITIS

72. Bacterial scleritis based on G/S,C/ S parenteral therapy is generally accepted Pseudomonas scleritis or sclerokeratitiscombination 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. SCLERITIS

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74. Classification episcleritis scleritis Simple Anterior scleritis -nodular type -diffuse type -necrotizing with inflammation -necrotizing without inflammation ( Scleromalacia Perforan ) Nodular Posterior scleritis

75. 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

76. EPISCLERITIS

77. Clinical finding Watson and Hayreh classification SCLERITIS

78. 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 sclera SCLERITIS

79. 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 SCLERITIS

80. “T” sign SCLERITIS Posterior scleritis

81. SCLERITIS