Retinal Vasculitis

1

2

RETINAL VASCULITISRETINAL VASCULITIS

BYBY

DR. YOUSAF JAMALDR. YOUSAF JAMAL

FCPS TRAINEEFCPS TRAINEE

KIOMS, HMC.KIOMS, HMC.

08/04/200908/04/2009

3

CONTENTSCONTENTS

• INTRODUCTION

• CLINICAL CHARACTERISTICS

• PATHOGENESIS

• ETIOLOGY

• OCULAR CAUSES & MANAGEMENT

• CAUSES IN PAKISTAN ( A STUDY )

• MCQs

4

INTRODUCTIONINTRODUCTION

• Retinal vasculitis is a sight threatening inflammatory eye disease affecting the retinal vasculature.

• Presents as:

a. Periphlebitis: veins are affected

b. Periarteritis: arteries are affected or

c. Angiitis: as a combination of both

5

CLINICAL CHARACTERISTICSCLINICAL CHARACTERISTICS

SYMPTOMS• Asymptomatic if restricted to peripheral fundus• Gradual, painless loss of vision (most common)

• Floaters (indicates significant migration of leukocytes to vitreous)

• Photopsia & reduced color vision (less common but present in vasculitis surrounding macula)

• Central or Para central scotomata

6

SIGNSSIGNS

• RAPD in M.S.• Visual field defects• Abnormal Amsler grid & color vision• Elevated IOP in ocular toxoplasmosis• A/C cells & Flare• Neovasularization• Vitrits• Vitreous hemorrhages

7

SIGNS OF PERIARTERITISSIGNS OF PERIARTERITIS

• Attenuation

• Sheathing (diagnostic)

• Cotton-wool spots

• Opaque superficial retina due to occlusion

8

SIGNS OF PERIPHLEBITISSIGNS OF PERIPHLEBITIS

• Retinal hemorrhages

• Edema

• Telengectasias

• Micro-aneurysms

9

PATHOGENESISPATHOGENESIS

• PRIMARY VASCULITIS

• INFECTIOUS VASCULITIS

• IMMUNE VASCULITIS

10

PRIMARY VASCULITISPRIMARY VASCULITIS

• Lymphopenia with normal helper T-cells to suppressor T-cells ratio

• Increased conc. Of immune complexes• Anticardiolipin antibodies• Reduced antibody affinity to retinal-S antigen• Increased expression of IL-2 surface markers

But their significance still remains to be seen

11

INFECTIOUS VASCULITISINFECTIOUS VASCULITIS

• Vascular endothelium invaded by microorganisms result in cell injury & death

• Immune complexes form with antigenic components of microorganisms, activates complement system, attract leukocytes & induce inflammation

12

IMMUNE VASCULITISIMMUNE VASCULITIS

• May be T cell mediated as in graft rejection, giant cell arteritis & takayasu disease

• Ag-Ab & immune complex deposition is main mechanism including eye

• Anti-endothelial cell antibody and anticardiolipin antibodies are also associated with retinal vasculitis

13

ETIOLOGYETIOLOGY

OCULARCAUSES

SECONDARYCAUSES

14

OCULAR CAUSESOCULAR CAUSES

• Eale’s Disease • IRVAN Syndrome (Idiopathic Retinal Vasculitis,

Aneurysms & Neuroretinitis)

• Intermediate uveitis (Parsplanitis)

• Frosted branch angiitis

• Birdshot retinochoroidopathy

15

SECONDARY CAUSESSECONDARY CAUSES

VASCULITIS

• Giant cell arteritis• Takayasu arteritis• Polyarteritis nodosa• Wegener’s granulomatosis• Churg-strauss syndrome

16

SYSTEMIC / INFLAMMATORYSYSTEMIC / INFLAMMATORY DISEASES DISEASES

• Multiple sclerosis• Behcet’s syndrome• Sarcoidosis• SLE• Inflammatory bowel

disease• Rheumatoid arthritis• Vogt-Koyanagi-

Harada disease

• Relapsing polychondritis

• Susac syndrome• Sjogren syndrome

(rare)• Juvenile idiopathic

arthritis

17

INFECTIOUSINFECTIOUS

BACTERIAL• Tuberculosis• Syphilis• Lyme disease• Bartonella henselae• Whipple’s disease• Rickettsial disease

18

VIRALVIRAL

• Acute Retinal necrosis• Cytomegalovirus• Human immunodeficiency virus• HTLV vasculitis• Hepatitis-related vasculitis

PARASITIC• Toxoplasmosis• Toxocariasis

19

DRUG-INDUCEDDRUG-INDUCED * *

• Sulfonamides• Propythiouracil• Penicillin• NSAIDS• Anticonvulsants

* Rahi AH, Tabbara KF. Retinal vasculitis: Pathogenesis and laboratory investigations. Int Ophthalmol Clin 1995;35:93-105

20

MALIGNANCY/MASQUERADEMALIGNANCY/MASQUERADE

• Retinoblastoma • Ocular lymphoma • Metastasis • Leukemia • Melanoma

21

EALES’ DISEASEEALES’ DISEASE

• An idiopathic obliterative vasculopathy affecting peripheral retina of young males characterized by recurrent vitreous hemorrhages

• 1st described by Henry Eales in 1880 & 1882 in men with Hx of recurrent headache, epistaxis, dyspepsia & ch. Constipation

• Eales thought it to be due to vasomotor necrosis but found it to be of venous inflammation (periphlebitis)

22

EPIDEMIOLOGYEPIDEMIOLOGY

• Most common in Indian sub. & middle east• 1 in 200 or 250 ophthalmic pts in India• Peak age = 20-35 yrs, although 13-63 yrs

reported too• Males affected more• Bilateral involvement in 50 – 90% pts

23

ETIOLOGYETIOLOGYA diagnosis of exclusionA diagnosis of exclusion

Idiopathic but associations….Idiopathic but associations….

• Tuberculosis

• Focal sepsis

• Thromboangitis obliterans

• Brucellosis

• Syphilis• Behcet’s disease• Para nasal sinus

disease

• Infectious mononucleosis

24

• Vestibuloauditory dysfunction

• Wegener’s granulomatosis

• Acute or sub acute mylopathy

• Leprosy

• Sarcoidosis

• MS• Lyme disease• SLE

25

PATHOLOGYPATHOLOGY

• Chronic and incomplete vascular occlusion with tissue hypoxia

• 3 phases

Inflammatory

Obliterative

Proliferative

26

PRESENTATIONPRESENTATION

SYMPTOMS

• Decreased vision

• Floaters

• Specks

• Cobwebs

27

SIGNSSIGNS

INFLAMMATION

• A/C cells, flare & KPs

• Vitreous debris & cells

• Tortuous & dilated veins

• Perivascular exudates

• Vascular sheathing

• Superficial retinal hemorrhages

28

29

30

31

NONPERFUSIONNONPERFUSION

• Peripheral retina more affected (temporal)• Solid white lines of obliterated vessels• Well demarcated area at perfused-

nonperfused retina• Collaterals, micro aneurysms, AV-shunts• Venous beading• Hard exudates• Cotton-wool spots

32

33

NEOVASCULARIZATIONNEOVASCULARIZATION

• IN 80% Pts

• NVD

• NVE

• Recurrent vit. Hemorrhage

• Pigmentation suggestive of healed chorioretinitis

34

35

OTHER SIGNSOTHER SIGNS

• BRVO

• PVD in 27%

• Macular edema

• Ischemic maculopathy

• Macular hole

36

COURSECOURSE

• Variable• Temporary or permanent remission• Progressive blindness in others• Charmis classification

Stage I: mild periphlebitis of small capillaries

Stage II: perivasulitis larger veins

Stage III: Neovessels with hemorrhage

Stage IV: recurrent vit. Hemorrhage, Trac. R/D

37

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

• Proliferative diabetic retinopathy

• CRVO, BRVO

• Sickle cell hemoglobinopathies

• Sarcoidosis

• SLE

38

COMPLICATIONSCOMPLICATIONS

• Recurrent vit. Hemorrhage

• Rubeosis iridis & Neovascular glaucoma

• Complicated cataract

• PVD

• Tractional R/D

39

INVESTIGATIONSINVESTIGATIONS

• Laboratory tests

• Imaging

• Other tests

40

LAB. TESTSLAB. TESTS

• CBC• Blood glucose levels• Sickle cell preparation & Hb electrophoresis• ACE & Lysozyme levels• ANA, RA factor, ESR• Mantoux test• PCR of M. tuberculosis in vitreous

41

IMAGINGIMAGING

FFA

• Micro vascular abnormalities

• Neovasulariation & exudative sheathing will leak

• Helps to localize the area for laser application

42

43

IMAGINGIMAGING

• Ultrasound helps in showing Vitreous hemorrhage, R/D

• CXR

• MRI brain for white matter anomalies

44

OTHER TESTSOTHER TESTS

• Increased conc. Of oxidation & per oxidation products in vitreous *

• Decreased levels of antioxidant enzymes in vitreous i.e. reduced glutathione, super oxide dismutase, and glutathione peroxidase *

• Hearing & balance testing***Sulochana KN, Biswas J, Ramakrishnan S. Eales' disease: increased

oxidation and peroxidation products of membrane constituents chiefly lipids and decreased antioxidant enzymes and reduced glutathione in vitreous. Curr Eye Res. Sep 1999;19(3):254-9.

**Renie WA, Murphy RP, Anderson KC, et al. The evaluation of patients with Eales' disease. Retina 3:243 248, 1983

45

TREATMENTTREATMENT

MEDICAL• Antioxidants

Vit. A, C & E• Corticosteroids

systemic, subconjuntival, periocular• Intravitreal Triamcinolone acetonide

for CME, Dose = 2-4mg• Anti-VEGF (in studies showing good results)

46

ANTI-TUBECULUOS TxANTI-TUBECULUOS Tx

• Presence of old tuberculosis lesion or positive mantoux test may show some relation

• Reserved for those with acute phlebitis, massive infiltration, nodule formation, obliteration of vessels

• ATT regimenRifampicin 450 mg 1 x OD x 9 monthsIsoniazid 300 mg 1 x OD x 9 months

47

RETINAL ABLATIONRETINAL ABLATION

PHOTOCOAGULATIONJuntional area between perfused & non-perfused

area to be treated• Argon green laser (514nm)• Xenon arc photocoagulation• Diode laser (810nm)• Frequency doubled Nd:YAG laser (532nm)ANTERIOR RETINAL CRYOTHERAPY• Convert hypoxic areas to anoxic arc, stops

proliferative Eales’ retinopathy

48

VITRECTOMYVITRECTOMY

• PPV effective in non clearing vit. Hemorrhage

• In tractional R/D, PPV + membrane dissection

49

IRVAN SYNDROMEIRVAN SYNDROME

• Schatz & Kincaid

• Uncommon

• Healthy young with female more affected

• Periarteritis

• Not familial

• Investigations negative

50

PRESENTATIONPRESENTATION

• Asymptomatic, despite fundus involvement• Multiple, leaking, tied-knot-like aneurysmal

dilatations of retinal arteriolar tree & optic nerve head

• Neuroretinitis with exudates extending to macula

• Extensive peripheral capillary non-perfusion• Aneurysms may increase in no. or regress

51

52

53

54

55

FFA FINDINGSFFA FINDINGS• Prominent vascular dilatation• Late staining of macro aneurysms & Retinal

arteriolar wall• Peripheral extensive capillary non-perfusion• Hyper fluorescent optic nerve head with

extensive leakage in late angiogram

56

OUTCOMEOUTCOME

• Extensive retinopathy & non-perfusion threatens vision

• Leads to neovascularization, vitreous hemorrhage & neovascular glaucoma

57

TREATMENTTREATMENT

• Symptomatic

• No benefit from steroids despite inflammatory process

• Pan retinal photocoagulation

• Pars plana vitrectomy plus endolaser

58

COMPARISON WITH EALESCOMPARISON WITH EALES

EALES• Perphlebitis• Males more affected• tuberculin

hypersensitivity• temporal retina• Steroids

recommended

IRVAN•Periarteritis•Female•No relation

•Not specific•No use

59

INTERMEDIATE UVEITISINTERMEDIATE UVEITIS

• An insidious, chronic, relapsing disease with vitreous major site of inflammation

• May be idiopathic or associated• Pars planitis is idiopathic IU (85-90%) with

snowballs & snow banking• Mild periphlebitis & nongranulomatous ‘spill over’

anterior uveitis

60

NOMENCLATURENOMENCLATURE

Cyclitis Fuchs’ 1908

Peripheral uveitis Schepens 1950

Pars planitis Welch et al 1960

Chronic cyclitis Smith et al 1973

Basal uveoretinitis Bec et al 1977

Intermediate uveitis international uveitis 1987

study group

61

EPIDEMIOLOGYEPIDEMIOLOGY

• PP more common in children

• Other IU 25-35 yrs

• IU is 15% of all uveitis cases & 20% pediatric uveitis cases

• Bilateral 70-90%

• Clinical diagnosis

62

CLINICAL FEATURESCLINICAL FEATURES

SYMPTOMS

• Blurring of vision• Floaters• Mild photophobia• Rarely redness & pain in PP• Central vision decreased if CME develops

63

SIGNSSIGNS

• KP• Corneal edema• Vitreous cells• Snowballs most numerous inferiorly• Peripheral periphlebitis, perivascular sheathing• Snow banking• Neovascularization on snow bank• Disc edema

64

vitritisvitritis

65

Mild peripheral periphlebitisMild peripheral periphlebitis

66

snow bankingsnow banking

67

ASSOCIATIONSASSOCIATIONS

• Idiopathic/PP 85-90%• MS• Sarcoidosis• IBD• CNS/intraocular lymphoma• Toxocara• Lyme disease• HTLV-1

68

PATHOGENESISPATHOGENESIS

• Yet to be ascertained• Cell breakdown products in vitreous base may

act as antigen• Relative ischemia leads to inflammation• Ormerod et al* isolated Propionibacterium acnes

from vitroeus of pt with persistent PP

*ormerod LD, Puklin JE, Giles CL. Chronic propionibacterium acnes endophthalmitis as a cause of intermediate uveitis, Ocular immunology and inflammation 1997;4:67-68

69

PATHOLOGYPATHOLOGY

Snowball opacities• Epitheliod cell granulomas

Snow bank• Condensed vitreous, spindle cells, blood

vessels, & hyper plastic non-pigmented epithelium of Pars plana with few lymphocytes

70

INVESTIGATIONSINVESTIGATIONS

• FBC, U&E, ESR• Urinalysis• TPHA, FTA-ABS• Lyme serology • ACE levels• CXR• MRI if neurological signs

71

INVESTIGATIONSINVESTIGATIONS

• Vitreous cytology for lymphoma• OCT & FFA for CME• Ultrasound biomicroscope 50MHz in case of

small pupil or dense cataract• ERG may show dysfunction of retina

72

COMPLICATIONSCOMPLICATIONS

1. CME (30%)…. Major cause of visual loss

2. Macular epiretinal formation

3. Posterior sub capsular cataract

4. Band shape keratopathy

5. Glaucoma

6. Retinal detachment

7. Vitreous hemorrhage

73

Cystoid macular edemaCystoid macular edema

74

TREATMENTTREATMENT

• Treat the cause in IU• In PP treat the inflammatory process• Tx only indicated if

Vision 6/12 or less or Evidence of CME

• Four step approach by Kaplan*

*Kaplan HJ. Intermediate uveitis a four step approach to treatment. In Saari KM (Ed): Uveitis update Amsterdam: Excerpta Medica 1984;169-72

75

STEP 1STEP 1

• Posterior sub-tenon inj. Of depot steroids

methyl prednisolone 40 mg

triamcinolone acetonide 40 mg• Can be repeated for 2-3 times with three weeks

interval• Systemic steroid 60-80 mg/day alone or in

combo with periocular injections in severe cases• I/vitreal triamcinolone

76

STEP 2STEP 2

• If step 1 fails• Cryopexy done to destroy hyperemic

vascular component• Done by double freeze & thaw technique• Laser photocoagulation for neovessels

77

STEP 3STEP 3

• If step 2 fails & immunomodulatory agents not indicated

• PPV with posterior hyaloid separation and peripheral laser photocoagulation to pars plana snow bank

78

STEP 4STEP 4

• If all fails then systemic immunomodulatory agents

Methotrexate

Cyclophosphamide

Cyclosporine

Azathioprine 50 mg x TDS….2 months

50 mg x BD……1 month

50 mg x OD……1 month

79

80

PROGNOSIS PROGNOSIS

3 categories

1. 10% self-limiting

2. 30% with remissions & exacerbations

3. 60% prolonged course without exacerbations

81

FROSTED BRANCH ANGIITISFROSTED BRANCH ANGIITIS

• An acute panuveitis with severe vasculitis of whole retina

• Veins more involved• Also called diffuse retinal periphlebitis• First described by ITO in Japanese literature in

1976, in 6 yrs old child with severe sheathing of all retinal vessels appearing as frosted branches of tree

82

83

EPIDEMIOLOGYEPIDEMIOLOGY

• Rare, described in only 58 cases in literature• Mostly in Japan…. Also north America, India &

turkey• Typically bilateral but unilateral (28%)• Males : females (52% : 48%)• 6-16 yrs in Japan• 23-29 yrs in other countries

84

CLASSIFICATIONCLASSIFICATION

1. Idiopathic2. Ocular associations

Cytomegalovirus retinitis AIDS retinitis Toxoplasmic chorioretinitis

3. Systemic associationssystemic lupus erythematosus Crohn’s disease large cell lymphoma acute lymphoblastic leukemia

85

PRESENTATIONPRESENTATION

SYMPTOMS• acute visual loss • floaters • flashing lights

Associated systemic symptoms • flu-like syndrome • sore throat, fever and malaise

86

SIGNSSIGNS

• Acute visual loss

• Severe vascular sheathing

• retinal edema

• Vitritis and iridocyclitis

• Otherwise healthy patients

87

• Papillitis, hard exudates, retinal hemorrhages, venous occlusion are uncommon

• Dye leakage from sheathed vessels in the late phase of fluorescein angiogram and no signs of stasis or occlusion.

88

INVESTIGATIONSINVESTIGATIONS

• 1. Ophthalmoscopy

• 2. Fluorescein angiogram

• 3. Visual field test

• 4. Electrophysiological tests: ERG/VECPs

89

FFAFFA

• In early phase: normal venous flow & delayed filling of arteries

• In late stages: leakage from vessels (veins more) +  hyper fluorescence of optic disc are characteristic

• Vessels narrowing but no occlusion• Areas of non-perfusion• A-V anastomosis

90

91

VISUAL FIELDVISUAL FIELD

• Blind spot enlargement• Central scotoma within 30 degrees due to

macular edema & exudates

ERG• Reduction in amplitude of a- & b- waves

VECPs• Pattern VECPs also reduced

92

DDxDDx

• Sarcoidosis • Syphilis • Tuberculosis • Multiple sclerosis • Systemic lupus erythematosus • Pars planitis • Eales’disease • Viral • Lymphoma/Leukemia

93

TREATMENTTREATMENT

• Treat the cause

• Systemic steroids

initial dose 80 to 100mg oral prednisone for 10 days

• Prognosis is usually very good with steroid Tx

visual acuity, field recovery in 2-3 months

94

95

96

COMPLICATIONSCOMPLICATIONS

• Neovascularization

• Neovascular glaucoma

• Macular scarring

• Retinal detachment

97

References for FBAReferences for FBA

• 1. Kleiner RC, Kaplan HJ, Shakin JL et al. Acute frosted retinal periphlebitis. AJO 1988; 106: 27-34.

• 2. Sugin S, Henderly DE, Friedman SM et al. Unilateral frosted branch angiitis. AJO 1991; 11: 682-85.

• 3. Hamed LM, Fang EN, Fanous MM, et al. Frosted branch angiitis: the role of systemic corticosteroids. J Ped. Ophth. Strab. 1992; 29: 312-13.

• 4. Kleiner RC. Frosted branch angiitis: clinical syndrome or clinical sign? Retina 1997; 17(5): 370-71.

• 5. Quillen DA, Stathopulos NA, Blankenship GW, et al. Lupus associated frosted branch periphlebitis and exudative maculopathy. Retina 1997; 17(5): 449-51.

98

BIRDSHOT BIRDSHOT RETINOCHOROIDOPATHY RETINOCHOROIDOPATHY

• Uncommon, idiopathic, chronic, recurrent, bilateral posterior uveitis

• First described by Franceschetti and Bable in 1949

• In 1980, Ryan and Maumenee coined the term birdshot*

• Gass called it  vitiliginous choroiditis bcz of similarity to cutaneous vitiligo

*Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthalmol. Jan 1980;89(1):31-45

99

• Deep, oval, creamy, indistinct spots• Radiate from disc towards equator• Moderate vitritis

100

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

• Cause is unknown• 80-95% are HLA-A29 positive• LeHoang and coauthors reported all pts of

BSRC positive for HLA-A29 type 2 subtype*• Nussenblatt and colleagues found relation of

BSRC with HLA-B12**

*LeHoang P, Ozdemir N, Benhamou A, et al. HLA-A29.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol. Jan 15 1992;113(1):33-5.

**Nussenblatt RB, Mittal KK, Ryan S, et al. Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. Aug 1982;94(2):147-58

101

EPIDEMIOLOGYEPIDEMIOLOGY

• Rare• Middle age (35-70yrs), average age 50yrs• Caucasians• Female more affected

102

PRESENTATIONPRESENTATION

SYMPTOMS

• Decreased vision - 68% • Floaters - 29% • Nyctalopia - 15% • Dyschromatopsia - 12% • Glare - 19% • Photopsia - 17%

103

• Fluctuating vision - 7%• Pain - 7%• Decreased depth of perception - 5% • Shimmering vision - 3% • Metamorphopsia - 3% • Decreased peripheral vision - 3% 

104

SIGNSSIGNS

• Decreased VA• A/C cells • KPs are rare• Post. Synechiae• Vitritis but no snowballs• Retinal vasculitis involving large and small

vessels• Optic disc edema & CME

105

FUNDUSFUNDUS

• Small lesions with ¼- ½ DD, may be confluent• Two types of lesions, present at posterior pole &

extend till equator1. oval & not well demarcated. Pale yellow or cream color spots easily seen with indirect ophthalmoscopy2. atrophic, sharply demarcated, round “punched out” seen by both indirect ophthalmoscopy & 78 or 98 D lens

106

107

Residual punched-out, non-pigmented scars

108

INVESTIGATIONSINVESTIGATIONS

LABORATORY WORK-UP

• Blood testing for HLA-A29 helps to support the diagnosis

• Baseline renal function for those who need cyclosporine therapy

109

IMAGING STUDYIMAGING STUDY

FA• Early hypo-fluorescence & late mild hyper-

fluorescence

ICG• Well-defined hypo-fluorescent spots in early

phases, becomes hyper-fluorescent later • Many more spots can be seen by ICG than FA

110

FA of birdshot retinochoroidopathy

• Extensive late intraretinal and disc leakage

• Venous hyperfluorescence

111

ELECROPHYSIOLOGYELECROPHYSIOLOGY

ERG• Normal in early disease, but then decreased b-

wave amplitude & then oscillatory potential• Delay in implicit time of 30 Hz flicker ERG is

most sensitive change• ERG findings suggest intraretinal edema, so

correlate with retinal vasculopathy rather than choroidal

112

HISTOPATHOLOGYHISTOPATHOLOGY

Only 2 histopathological studies (on phthisical eyes)• *Nussenblatt and coauthors described mild lymphocytic

response, retina involved with granulomatous inflammation

• **Gaudio and coauthors described aggregation of the lymphocytes with foci in choroid, optic nerve and retinal vessels

*Nussenblatt RB, Mittal KK, Ryan S, et al. Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. Aug 1982;94(2):147-58

**Gaudio PA, Kaye DB, Crawford JB. Histopathology of birdshot retinochoroidopathy. Br J Ophthalmol. Dec 2002;86(12):1439-41

113

TREATMENTTREATMENT

• Steroids

• Immunosuppressive agent (Cyclosporin)

• Antifungal (Ketoconazole)• Other immunomodulators• Intravenous immunoglobulin

114

STEROIDSSTEROIDS

• Conflicting results• Some respond to local injectables, others

respond to systemic• Some with low dose, other with high dose• Topical steroids of no use• Periocular steroids for CME

115

CYCLOSPORINCYCLOSPORIN

• Very effective in BSRC with improved VA, dec. vitritis, stable eye

• Vitale & Foster showed cyclosporin treatment in low doses (2.5-5 mg/kg )*

• Maximum dose = 5 mg/kg with 4-6 weeks review of renal function

• Used for a year at least, then tapered *vitale AT, Rodriguez A, Foster CS. Low-dose cyclosporine therapy in the

treatment of birdshot retinochoroidopathy. Ophthalmology. May 1994;101(5):822-31

116

KETOCONAZOLEKETOCONAZOLE

• Can be used as an adjunct• Ketoconazole delays metabolism of cyclosporin• Silverstein and Wong demonstrate

cyclosporin 0.75mg/kg + ketoconazole 200mg/d• However not very useful bcz of potential risks of

ketoconazole like hepatitis

117

IMMUNOMODULATORSIMMUNOMODULATORS

• Kiss and colleagues *mycophenolate mofetil, azathioprine, methotrexate, and daclizumab but study was of small size

• LeHoang and colleagues ** intravenous Immunoglobulin with stable vision of

33-36 eyes for 39 months*Kiss S, Ahmed M, Letko E, et al. Long-term follow-up of patients with birdshot

retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy

**LeHoang P, Cassoux N, George F, et al. Intravenous immunoglobulin (IVIg) for the treatment of birdshot retinochoroidopathy. Ocul Immunol Inflamm. Mar 2000;8(1):49-57

118

FOLLOW-UPFOLLOW-UP

• Every 4-6 weeks• Query

VAcolor perceptionnight visionadverse effect of medicationsRFTs, LFTsFFA, ICG

119

COMPLICATIONSCOMPLICATIONS

• Chronic cystoid macular edema – 50%; the most common cause of reduced central visual acuity 

• Epiretinal membrane - 10% • Macular pucker • Choroidal neovascularization • Peripapillary sub retinal neovascularization - 6% 

120

• Retinal neovascularization located on the optic disc 

• Peripheral retinal neovascularization with capillary nonperfusion 

• Optic nerve atrophy • Other complications, such as cataract,

glaucoma, and rhegmatogenous retinal detachment

121

PROGNOSISPROGNOSIS

• Guarded

• potentially blinding

• multiple exacerbations and remissions

122

CAUSES OF R.V. IN PAKISTAN*CAUSES OF R.V. IN PAKISTAN*

• Departments of Ophthalmology, Jinnah Postgraduate Medical Center, Karachi and Chandka Medical college, Larkana

• April 1996 to December 2002 • 102 pts of R.V.

*Pak J Ophthalmol Apr 2004;20(2):53-6

123

RESULTSRESULTS

• 50 pts(49%) diagnosed as Eales’ disease • 28 pts(27.4%) had inactive tuberculosis• 10 pts(9.8%) had raised serum ACE &

diagnosed as sarcoidosis• 08 pts(7.8%) had active tuberculosis• 04 pts(3.9%) had syphilis• 02 pts(1.9%) had ocular toxoplasmosis

124

TAKE HOME MESSAGETAKE HOME MESSAGE

• RV can be insidious, sight threatening

• Thorough examination

• Systemic associations

• Treat the cause

• Immunosuppressive in systemic diseases

125

THANK YOU

126

MCQsMCQs

1. Common cause of intermediate uveitis

a. multiple sclerosis

b. idiopathic

c. lyme disease

d. syphilis

Ans: b, (85-90%)

127

2. Major cause of visual loss in pars planitis

a. band keratopahty

b. PSC

c. epiretinal membrane

d. CME

Ans: d

128

3. The treatment of choice for BSRC is :

a. Topical steroids b. Systemic steroids c. Cyclosporin A d. Oral tolerization of retinal S- antigen

Ans: c

129

4. Which therapeutical intervention for pars planitis is recommended as first choice?a. Steroid dropsb. Systemic steroidsc. Non-steroidal anti-inflammatory drugsd. Periocular steroid injections

Ans: d

130

5. What are the most important differential diagnoses to consider in case of frosted branch angiitis?a. Sarcoidosis and tuberculosis.b. Multifocal choroiditis and panuveitis.c. SLE and polyarteritis nodosa.d. Wegeners’ granulomatosis.e. All ocular and systemic diseases presenting with retinal periphlebitis.

Ans: e

131

NEXT LECTURENEXT LECTURE

PROPTOSIS IN CHILDREN

BY

DR. MUSHTAQ