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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
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