1. 전안부 conference
Scleritis
R3 정현진/ Ap. 나경선

2. Case presentation
69/F
C.C :Red eye, lid swelling(OD) onset) 20days ago
ocular pain(+), bloody discharge(+)
Local에서 R/O episcleritis(OD)로 퀴노비드, 네 오덱스 안연고 점안하였으나 악화되어본원 전원 됨
DM(-), rheumatic disease Hx.(-)
ocular op./trauma Hx.(-/-)
scleral mass/sclerouevitis(OS)로 본원 systemic steroid Tx. 당시 공막염 원인에 대한evaluation(-)
10년전 아산병원에서 enucleation(OS)시행받음
안구적출후에도 좌안염증으로 고생, 의안도 못 함.
2.8
Arthralgia(-), oral & genital ulcer(-), back pain(-)
약물치료 일시적인 호전과 악화 반복

3. Ocular evaluation VA 0.08 ref. error IOP 15mmHg
lid mild swelling c mild erythema
Conj. mod. injected
scleral edema
thinning of defect at inf(x2)
(1.5mm, 1.0mm sized)
Cornea rl .clear
AC deep & cell(+)
Pupil round & nl sized, LR(+)
Lens mod cortical opacity/OD
박창현 charting mod. injected c melted necrotizing conj defect(x2)(1.5mm, 1.0mm sized) c Pus(+) at temporal side
B-scan: 안구안쪽으로는 염증소견 없음, scleral thickining, eyeball은 유지됨(T-sign(-))
여의도 post. Scleritis: O-LVF x 4, O-LON x q1hr, O-DCF x 4
Uveitis lab(-), CSF study(-) Add 1AT x 2, H-LON 40mg, IPF20(ibuprofen 200mg) 3T

4. Plan Impression Necrotizing scleritis(OD) R/O bacterial infection(OD)
Last eye(OD)
Plan
Culture(bacterial & fungus)
Immunology lab -> Rheumatology consultation
Admission
H-LON 60mg start
Ceftriaxone 1g iv qd
O-CS x q 1hr
O-MOX x q 1hr
O-1AT x 2(OD)
Scleral patch를 대는 것은 위험

5. 입원다음날, 내원 3일후 S: 좀 좋아진거 같아요 VA 0.16
Lid sl. erythematous swelling Conj. mild injected c thinning defect (x2)
at inf. & temp. side(dec.)
Cornea some SPE's(+) c microbullae
AC deep & cell(+)
Pupil post. synechiae at inf. Half
Lens mod cortical opacity c NS
Fd nl. Optic disc c flat post. Pole,
tigroid app.
/OD
Plan : keep eyedrops & H-LON 60mg, IV anti 유 지
2.10
H-LON 60mg for 3days

6. Lab finding Autoimmune study(-) by Rheumatology
(RA factor, SLE profile, ANA, ANCA, UA, CBC, BC, ACE)
Infection study(-)
(VZV, HSV, syphilis, Toxoplasma, Toxocariosis, CNV)
Culture :propionibacterium acnes(+) 5일
Rheumatology consultation
Rheumatic disease소견은 없으나 면역억제제 복용량 조절해주기로함
(METHOTREXATE 10MG Q WEEK + FOLIC ACID 1MG QD 추가, steroid는 tapering하거나 daily 5mg씩 줄이시되, maintanence dose는 유지)

7. 입원8일후, 내원 9일후 S: 분비물 많이 줄어 들었어요 VA 0.25
Conj. mild injected c thinning defect (x2) at inf. & temp. side(dec.)
Cornea some SPE's(+)
AC deep & cell(rare)
Pupil post. synechiae at inf. Half
Lens mod cortical opacity c nucleosclerosis
Fd nl. Optic disc c flat post. Pole, tigroid app./OD
2.17
Plan: 금일 퇴원
O-CS x 2, O-MOX x 4, O-1AT x 2, O-LON x 4, (O-CS, O-MOX 감량)
H-LON 60mg for 8days-> H-LON x 40mg for 2days,MTX 10mg q1wk, folic acid 1mg QD, ->H-LON x 30mg for 1wk예정

8. plan 금일 퇴원 MTX 10mg q 1wk H-LON 30mg for 1wk Folic acid 10mg qd
O-CS x 2
O-LON x 4
O-MOX x 4
O-1AT x2 (OD)
O-LON은 12일 부터 추가

9. 퇴원 12일후, 내원 21일후 S : 많이 좋아짐 VA OD 0.125 IOP OD 19mmHg
sclerae & Conj. mild injected c inf. thinning defect (x2) at inf. & temp. side(dec.)
Cornea some SPE's(+)
AC deep & cell(rare)
Pupil synechia at inf. Half
Lens mod cortical opacity
c nucleosclerosis, PSCO(+)
Fd nl optic disc c flat post pole, tigroid app.
/OD
2.29
H-LON 25mg for 1wk->H-LON 20mg for 1wk류마티스내과 조절예정
O-MOX x 4
O-LON x 4
백내장 수술고려

10. plan MTX 10mg q 1wk H-LON 25mg for 1wk -> 20mg for 1wk로 tapering
Folic acid 10mg qd
O-MOX x 4
O-LON x 4(OD)

11. 퇴원 한달후, 내원 6주후 S : 많이 좋아짐 VA 0.1 IOP 20mmHg
퇴원 한달후, 내원 6주후
S : 많이 좋아짐
VA IOP 20mmHg
sclerae & Conj. not injected c inf. thinning defect (x2) at inf. & temp. side(dec.)
Cornea some SPE's(+)
AC deep & cell(rare)
Pupil synechia at inf. Half
Lens mod cortical opacity
c nucleosclerosis, PSCO(+)
Fd nl optic disc c flat post pole,
tigroid app.
/OD
여기까지는 좋아지고 있음
백내장 수술고려

12. plan
MTX 10mg q 1wk
H-LON 10mg for 1wk-> 7.5mg for 1wk -> 5mg for 1wk-> 2.5mg 1wk로 tapering
Folic acid 10mg qd
O-MOX x 2
O-LON x 2(OD)

13. 내원 2개월 후 S: ocular pain, tearing(OD) onset )5days ago
VA OD ref. error
sclerae & Conj. mild injected
c thinning defect (x2) at inf. & temp. side(dec.)
Cornea some SPE's(+)
AC deep & cell(rare)
Pupil synechia at inf. Half
Lens mod cortical opacity
c nucleosclerosis, PSCO(+) 악화
4.6
현재까지 약
2.8 H-LON 60mg for 8day -> H-LON 40mg for 2day(MTX 10mg q1wk) -> H-LON 30mg for 1wk(MTX 10mg q1wk) -> H-LON 25mg for 1wk(MTX)-> H-LON 20mg for 1wk(MTX)->H-LON x 15mg for 1wk(MTX)-> H-LON x 12.5mg for 1wk(MTX)-> H-LON x 10mg for 1wk(MTX)-> H-LON 7.5mg for 1wk(MTX)(이때부터악화)->H-LON 5.0mg for 2days(내원)-> H-LON 60mg으로 증량 -> mg감량, mg으로 감량

14. plan
Culture
H-LON 60mg 증량
O-MOX x 6
O-TOB x 6 (OS)
Adm. recommand

15. 1주일후 S: 많이 편해졌어요. 중간에 고름 안났어요. VA OD 0.125 ref. error
sclerae & Conj. mild injected c thinning defect (x2) at inf. & temp. side(dec.)
scleromalatic area at inferotemporal-stationarly
Cornea some SPE's(+)
AC deep & cell(trace)
Pupil synechia at inf. Half
Lens mod cortical opacity c
nucleosclerosis, PSCO(+)
Culture(-)
4.134.20마지막

16. Plan H-LON 60mg(for 1wk) O-MOX x 4 O-TOB x 4(OD)
FU 1wk(호전되면 H-LON 45mg으로 감량예정)

17. Review
Cornea Krachmer 3rd edition, Ch.104

18. Anatomy

19. Applied anatomy of vascular coats
상공막염 : 염증이 episclera와 tenon’s capsule에 구한
공막염 공막에 염증

20. Causes and systemic associations of scleritis
1. Rheumatoid artharitis
2. Connective tissue disease
Wegener granulomatosis
Polyarteritis nodosa
SLE
Miscellaneous
Relapsing polychondritis
Herpes zoster ophthalmicus
Surgically induced

21. Pathogenesis Immune-mediated leading to vessel and tissue damage
genetically controlled mechanisms
environmental factors(infectious or endogenous)
Type III hypersensitivity – immune complex inflammatory microangiopathy.
Type IV hypersensitivity – systemic autoimmune disease associated with circulating immune complexes(SLE, rheumatoid arthritis, and polyarteritis nodosa.)
Antigen – possibly scleral tissue(collagen), scleral blood vessel.
Posterior scleritis – CD4+ lymphocytes, intercellular adhesion molecule-1(ICAM-1) and LFA-1) on sclera and conjunctiva

22. Epidemiology Scleritis Episcleritis
more frequent in middle-aged and elderly
Peak in fifth decade
Diffuse, nodular > necrotizing
25~50%-associated systemic disease(RA>WG>relapsing polychondritis> spondylopathies, SLE.)
Episcleritis
young and middle-aged women
Simple>>nodular
26~36%-associated disease, 5~14% collagen vascular disease
No known geographic, racial, or genetic associations.

23. Classsification * Immune mediated * infectious
Surv Ophthalmol 50 (4) July–August 2005
* infectious

24. Episcleritis gradually subsiding over a period usually 5~10days
rapid onset, reaching peak in a few hours
Start upper temporal or upper nasal quadrants
gradually subsiding over a period usually
5~10days
Frequently asymptomatic
Mild ocular discomforts, FB sensation, mild photophobia
Redness
mild pinkish to fiery red (dilatation of episcleral blood vessels)
May be bilateral (not frequently simultaneous)
Recurrence (1 / 3, not necessarily in the same location)
fluid extravasuclar space
Tx: Topical steroids
Systemic NSAID : flurbiprofen (100 mg tid if unresponsive or recurrent)
그림 1 smple diffuse episcleritis

25. Nodular episcleritis Less common inflammation is confined
well-circumscribed, raised, red-pink, mobile nodule
D/Dx with conjunctival phlyctenula
- freely movable overlying conjunctiva
그림 1Localized nodule which can be moved over sclera
그림 2 Deep scleral part of slit-beam
not displaced
Nodular episcleritis

26. Scleritis Insidious Moderate~severe penetrating pain
refered to forehead, jaw, sinuses
Tearing, mild to moderate photophobia
globe tenderness
Necrotising scleritis-Intense, chronic, debilitating pain
Bilateral: 34~50% (simultaneous or sequential)
Recurrence
Anterior scleritis
Scleral edema, dilatation of deep episcleral vasculatures
Injection with bluish to violaceous tinge
Scleromalaciaperforans
no pain, redness

27. Diffuse anterior scleritis
Most benign and common
Comprising btw 40 % ~ 64 % of ant. Scleritis
12 of 30, progression to nodular type
3 of 30, progression to necrotizing type.
Btw 45 % ~ 62 % - associated systemic disease, typically RA
Insidious onset of generalized inflammation,
Last several months
Superficial and deep vessel distorted and tortuous,
Losing the radial pattern
Relatively benign - does not progress to necrosis
Widespread scleral and episcleral injection
Tx : oral NSAIDs
oral steroid if unresponsive

28. Nodular anterior scleritis
Tender and immobile, firm to touch, yellow deep red or violaceous scleral nodule.
Usually localized to the interpalpebral zone, approximately 3 ~ 4 mm from the limbus.
Progression to necrotizing scleritis – 20 %
45 % - associated systemmic disease, typically RA
그림 1 On cursory examination resembles nodular episcleritis
그림 2 Scleral nodule cannot be moved over underlying tissue
Treatment - similar to diffuse non-necrotizing scleritis

29. Anterior necrotizing scleritis with inflammation
Most severe form of scleritis
Sign of systemic vascular diseases (50~81% )
Intense pain, worsen at night
White avascular area surrounded by the sw elling of sclera
Become thinned sclera or choroid bare, st aphyloma may form
FAG : venular obstruction
Necrotizing scleritis – proteoglycans is stripped from the collagens by MMP from fibroblast – Antigenicity
Most severe form of scleritis and ominous
sign of systemic vascular diseases (50~81%)
Intense pain, worsen at night
White avascular area surrounded by the swelling of sclera
Become thinned sclera or choroid bare, staphyloma may form
FAG : venular obstruction
Painful and most severe type
Complications - uveitis, keratitis, cataract and glaucoma
Tx.
Oral steroids
Immunosuppressive agents (cyclophosphamide, azathioprine, cyclosporin)
Combined intravenous steroids and cyclophosphamide if unresponsive
그림 1 : avascular patchs
그림 2 scleral necrosis and visiblity of uvea
그림 3: Spread and coalescence
Most destructive form of scleritis
Extension of the inflammatory process led to – anterior uveitis (69 %), peripheral ulcerative keratitis (41 %), glaucoma (23 %), significant loss of visual acuity (82 %).
50 % ~ 81 % - associated with systemic connective tissue disease and/or potentially lethal systemic vasculitic disease.
of necrosis

30. Ocular complication Corneal Involvement Stromal keratitis
Sclerosing keratitis
Corneal thinning –peripheral
Peripheral ulcerative keratitis
Posterior Segment Involvement
Uveitis
Optic disc edema
Glaucoma
Hypotonia
Cataract
Orbital involvement

31. Anterior scleritis without inflammation (scleromalacia perforans)
Insidious onset, slow progression and lack of symptom
Thinned, avascular area of sclera
Ass with longstanding rheumatoid arthritis
FAG : arteriolar obstruction
Insidious onset, slow progression and lack of symptom
Thinned, avascular area of sclera
Ass with longstanding rheumatoid arthritis
FAG : arteriolar obstruction
Asymptomatic and untreatable
그림들 Progressive scleral thinning with exposure of underlying uvea
Rare, bilateral condition, occurring predominantly among elderly females with a history of severe, progressive, long-standing rheumatoid arthritis with extra-articular manifestations.
Caused by arteriolar obliteration as evidenced by FA, as opposed to venular non-perfusion, which is more prominent in necrotizing scleritis.

32. Ocular exam Level of inflammation
Examine under daylight, slit lamp(diffuse, narrow, red-free)
Under daylight
Pink~red - epislceritis
Deep-bluish red or violaceous - scleritis
If scleral necrotitis, blue-gray to dark-brown areas of underlying uvea

33. Diagnostic Evaluation
Comprehensive medical, ophthalmic history, review of system
Ocular and physical exam
Chest X-ray, blood test, serum chemistries, urine analysis
ANCAs for systemic vasculitides
c-ANCA: sensitive and specific for Wegener’s granulomatosis
p-ANCA: microscopic polyarteritis nodosa, relapsing polychondritis, renal vasculitis
HLA-B27, RA
FTA-abs (syphilis)
Scleral biopsy
Infectious scleritis, foreign body, masquerade syndrome

34. Surgically induced scleritis
Uninfectious
- underlying 전신질환이 있는 경우가 많다
Infectious
- Rare
-Exogenous
- 외상, 수술후, 각막염에서 침범(m/c)
- Endogenous
- 전신 감염와 연관

35. Infectious scleritis Pseudomonas - m/c cause
- scleritis without keratitis
도 유발 가능
익상편 수술시 MMC 혹은
irradiation과 관련하여 잘 발생
m/c cause : Pseudomonas,
Pneumococcus

36. Posterior scleritis
Inflammation of sclera posterior of the ora serrata
Frequently extends to choroid, retina, optic nerve, extraocular muscles, orbital tissues
Up to 60%, present with anterior inflammation diseases
In about 29% , systemic associations

37. Symptoms & signs Pain Visual loss Redness
severe, radiating
Visual loss
Mild & correctable with convex lens
transient hyperopia d/t reduction in AL
Moderate to uncorrectable(17~84%)
Choroidal folds or retinal striae
Annular cilioretinal detachment
Disc and macular edema
Fundus or orbit mass lesions
Reversible c good prognosis,
if early Dx. & appropriate Tx. provided
Redness
Proptosis, lid swelling, painful LOM
MRI Posterior scleral thickening
a - Thickening of posterior sclera
b -Fluid in Tenon space (‘T’ sign)
ERD
Ring choroidal detachment
Subretinal mass

38. Ocular Exam Fundoscopic findings Ultrasonographic findings
Common findings
Optic disc swelling
Exudative retinal detachment
Choroidal folds
Less common
Focal subretinal mass-like lesions
Ultrasonographic findings
“T” sign
Scleral & choroidal thickening
Edema in Tenon’s space
Computed tomography
Essential in excluding orbital inflammatory diseases, tumors, thyroid eye disease, sinus diseases
Extension into adjacent structures

40. Treatment Episcleritis Scleritis
Simple – observation or topical NSAIDS.
Episcleritis with connective tissue disease. – oral NSAIDs
Scleritis
30.4 % - required oral NSAIDs,
60 % - oral corticosteroids or immunosuppressive drugs
(90 ~ 100 % - necrotizing or posterior scleritis)
Diffuse or nodular scleritis not associated with an underlying systemic vasculitis.
oral NSAIDs, c/s topical corticosteroids
 Failure – systemic corticosteroids(1 to 1.5 mg/kg per day)
Oral NSAIDS 반응 없으면 우선 다른종류 NSAIDs써본다. -> NON necro
Oral steroid : severe NON-ne
Necrotizing
Systemic immunosuppressant” severe necro/ c Wegeners/ c pollyarterytis nodosa
Topiac cyclosporine: effective

41. Treatment Oral NSAIDs Systemic corticosteroids
1st line if not associated with systemic vasculitis
Treatment response within 2~3 weaks
For a minimum of 1 year
Systemic corticosteroids
Treatment failure with oral NSAIDs or severe scleritis
Prednisone 1~1.5mg/kg/day with tapering
High-dose methylprednisolone(1g/day x3) with tapering
Safe and effective in the induction
Immunosuppressive therapy
Failed systemic corticosteroids or for“steroid-sparing”
Underlying systemic vasculitis
Cyclophosphamide-1st line for scleritis with Wegener’s granulomatosis or polyarteritis nodosa
Specific antimicrobial therapy
For infectious scleritis

44. Findings epi Red Superficial episcleral vs. engorgement
scl. Violet, blue (translucent sclera)
Superficial and deep episcleral vascular engorgement
Avascular area with vascular occlusion
Peripheral corneal ulceration
Tx : topica (oral NSAIDs).oral
전신질환 관련(-) (+)

45. Thank you for your attention