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Retinal Vasculitis

Retinal vasculitis is an inflammatory eye disease affecting the retinal vasculature. It can present as periphlebitis (vein involvement), periarteritis (artery involvement), or angiitis (combination). Symptoms include gradual vision loss, floaters, and photopsia. Signs include vascular sheathing, hemorrhages, edema, and neovascularization. It can be caused by infections, autoimmune diseases, malignancies, or be idiopathic. Treatment involves identifying and treating the underlying cause, along with corticosteroids, laser photocoagulation, and vitrectomy if needed. Intermediate uveitis is a chronic form characterized by vitritis, snowballs,

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1
2 RETINALRETINAL VASCULITISVASCULITIS 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 OCULAR CAUSES SECONDARY CAUSES
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 DISEASESDISEASES • 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 ETIOLOGYETIOLOGY A 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
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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
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33 NEOVASCULARIZATIONNEOVASCULARIZATION • IN 80% Pts • NVD • NVE • Recurrent vit. Hemorrhage • Pigmentation suggestive of healed chorioretinitis
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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
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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 regimen Rifampicin 450 mg 1 x OD x 9 months Isoniazid 300 mg 1 x OD x 9 months
47 RETINAL ABLATIONRETINAL ABLATION PHOTOCOAGULATION Juntional 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
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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
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80 PROGNOSISPROGNOSIS 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
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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. Idiopathic 2. Ocular associations Cytomegalovirus retinitis AIDS retinitis Toxoplasmic chorioretinitis 3. Systemic associations systemic 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
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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
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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 BIRDSHOTBIRDSHOT RETINOCHOROIDOPATHYRETINOCHOROIDOPATHY • 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 equator 1. oval & not well demarcated. Pale yellow or cream color spots easily seen with indirect ophthalmoscopy 2. atrophic, sharply demarcated, round “punched out” seen by both indirect ophthalmoscopy & 78 or 98 D lens
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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 VA color perception night vision adverse effect of medications RFTs, LFTs FFA, 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 drops b. Systemic steroids c. Non-steroidal anti-inflammatory drugs d. 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

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Retinal Vasculitis

  • 1.
  • 2.
    2 RETINALRETINAL VASCULITISVASCULITIS BYBY DR. YOUSAF JAMALDR.YOUSAF JAMAL FCPS TRAINEEFCPS TRAINEE KIOMS, HMC.KIOMS, HMC. 08/04/200908/04/2009
  • 3.
    3 CONTENTSCONTENTS • INTRODUCTION • CLINICALCHARACTERISTICS • PATHOGENESIS • ETIOLOGY • OCULAR CAUSES & MANAGEMENT • CAUSES IN PAKISTAN ( A STUDY ) • MCQs
  • 4.
    4 INTRODUCTIONINTRODUCTION • Retinal vasculitisis 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.
    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.
    6 SIGNSSIGNS • RAPD inM.S. • Visual field defects • Abnormal Amsler grid & color vision • Elevated IOP in ocular toxoplasmosis • A/C cells & Flare • Neovasularization • Vitrits • Vitreous hemorrhages
  • 7.
    7 SIGNS OF PERIARTERITISSIGNSOF PERIARTERITIS • Attenuation • Sheathing (diagnostic) • Cotton-wool spots • Opaque superficial retina due to occlusion
  • 8.
    8 SIGNS OF PERIPHLEBITISSIGNSOF PERIPHLEBITIS • Retinal hemorrhages • Edema • Telengectasias • Micro-aneurysms
  • 9.
    9 PATHOGENESISPATHOGENESIS • PRIMARY VASCULITIS •INFECTIOUS VASCULITIS • IMMUNE VASCULITIS
  • 10.
    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.
    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.
    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.
  • 14.
    14 OCULAR CAUSESOCULAR CAUSES •Eale’s Disease • IRVAN Syndrome (Idiopathic Retinal Vasculitis, Aneurysms & Neuroretinitis) • Intermediate uveitis (Parsplanitis) • Frosted branch angiitis • Birdshot retinochoroidopathy
  • 15.
    15 SECONDARY CAUSESSECONDARY CAUSES VASCULITIS •Giant cell arteritis • Takayasu arteritis • Polyarteritis nodosa • Wegener’s granulomatosis • Churg-strauss syndrome
  • 16.
    16 SYSTEMIC / INFLAMMATORYSYSTEMIC/ INFLAMMATORY DISEASESDISEASES • 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.
    17 INFECTIOUSINFECTIOUS BACTERIAL • Tuberculosis • Syphilis •Lyme disease • Bartonella henselae • Whipple’s disease • Rickettsial disease
  • 18.
    18 VIRALVIRAL • Acute Retinalnecrosis • Cytomegalovirus • Human immunodeficiency virus • HTLV vasculitis • Hepatitis-related vasculitis PARASITIC • Toxoplasmosis • Toxocariasis
  • 19.
    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.
    20 MALIGNANCY/MASQUERADEMALIGNANCY/MASQUERADE • Retinoblastoma • Ocularlymphoma • Metastasis • Leukemia • Melanoma
  • 21.
    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.
    22 EPIDEMIOLOGYEPIDEMIOLOGY • Most commonin 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.
    23 ETIOLOGYETIOLOGY A diagnosis ofexclusionA 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.
    24 • Vestibuloauditory dysfunction • Wegener’s granulomatosis •Acute or sub acute mylopathy • Leprosy • Sarcoidosis • MS • Lyme disease • SLE
  • 25.
    25 PATHOLOGYPATHOLOGY • Chronic andincomplete vascular occlusion with tissue hypoxia • 3 phases Inflammatory Obliterative Proliferative
  • 26.
  • 27.
    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.
    31 NONPERFUSIONNONPERFUSION • Peripheral retinamore 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.
    33 NEOVASCULARIZATIONNEOVASCULARIZATION • IN 80%Pts • NVD • NVE • Recurrent vit. Hemorrhage • Pigmentation suggestive of healed chorioretinitis
  • 34.
  • 35.
    35 OTHER SIGNSOTHER SIGNS •BRVO • PVD in 27% • Macular edema • Ischemic maculopathy • Macular hole
  • 36.
    36 COURSECOURSE • Variable • Temporaryor 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.
    37 DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS •Proliferative diabetic retinopathy • CRVO, BRVO • Sickle cell hemoglobinopathies • Sarcoidosis • SLE
  • 38.
    38 COMPLICATIONSCOMPLICATIONS • Recurrent vit.Hemorrhage • Rubeosis iridis & Neovascular glaucoma • Complicated cataract • PVD • Tractional R/D
  • 39.
  • 40.
    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.
    41 IMAGINGIMAGING FFA • Micro vascularabnormalities • Neovasulariation & exudative sheathing will leak • Helps to localize the area for laser application
  • 42.
  • 43.
    43 IMAGINGIMAGING • Ultrasound helpsin showing Vitreous hemorrhage, R/D • CXR • MRI brain for white matter anomalies
  • 44.
    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.
    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.
    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 regimen Rifampicin 450 mg 1 x OD x 9 months Isoniazid 300 mg 1 x OD x 9 months
  • 47.
    47 RETINAL ABLATIONRETINAL ABLATION PHOTOCOAGULATION Juntionalarea 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.
    48 VITRECTOMYVITRECTOMY • PPV effectivein non clearing vit. Hemorrhage • In tractional R/D, PPV + membrane dissection
  • 49.
    49 IRVAN SYNDROMEIRVAN SYNDROME •Schatz & Kincaid • Uncommon • Healthy young with female more affected • Periarteritis • Not familial • Investigations negative
  • 50.
    50 PRESENTATIONPRESENTATION • Asymptomatic, despitefundus 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.
    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.
    56 OUTCOMEOUTCOME • Extensive retinopathy& non-perfusion threatens vision • Leads to neovascularization, vitreous hemorrhage & neovascular glaucoma
  • 57.
    57 TREATMENTTREATMENT • Symptomatic • Nobenefit from steroids despite inflammatory process • Pan retinal photocoagulation • Pars plana vitrectomy plus endolaser
  • 58.
    58 COMPARISON WITH EALESCOMPARISONWITH EALES EALES • Perphlebitis • Males more affected • tuberculin hypersensitivity • temporal retina • Steroids recommended IRVAN •Periarteritis •Female •No relation •Not specific •No use
  • 59.
    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.
    60 NOMENCLATURENOMENCLATURE Cyclitis Fuchs’ 1908 Peripheraluveitis 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.
    61 EPIDEMIOLOGYEPIDEMIOLOGY • PP morecommon in children • Other IU 25-35 yrs • IU is 15% of all uveitis cases & 20% pediatric uveitis cases • Bilateral 70-90% • Clinical diagnosis
  • 62.
    62 CLINICAL FEATURESCLINICAL FEATURES SYMPTOMS •Blurring of vision • Floaters • Mild photophobia • Rarely redness & pain in PP • Central vision decreased if CME develops
  • 63.
    63 SIGNSSIGNS • KP • Cornealedema • Vitreous cells • Snowballs most numerous inferiorly • Peripheral periphlebitis, perivascular sheathing • Snow banking • Neovascularization on snow bank • Disc edema
  • 64.
  • 65.
    65 Mild peripheral periphlebitisMildperipheral periphlebitis
  • 66.
  • 67.
    67 ASSOCIATIONSASSOCIATIONS • Idiopathic/PP 85-90% •MS • Sarcoidosis • IBD • CNS/intraocular lymphoma • Toxocara • Lyme disease • HTLV-1
  • 68.
    68 PATHOGENESISPATHOGENESIS • Yet tobe 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.
    69 PATHOLOGYPATHOLOGY Snowball opacities • Epitheliodcell granulomas Snow bank • Condensed vitreous, spindle cells, blood vessels, & hyper plastic non-pigmented epithelium of Pars plana with few lymphocytes
  • 70.
    70 INVESTIGATIONSINVESTIGATIONS • FBC, U&E,ESR • Urinalysis • TPHA, FTA-ABS • Lyme serology • ACE levels • CXR • MRI if neurological signs
  • 71.
    71 INVESTIGATIONSINVESTIGATIONS • Vitreous cytologyfor lymphoma • OCT & FFA for CME • Ultrasound biomicroscope 50MHz in case of small pupil or dense cataract • ERG may show dysfunction of retina
  • 72.
    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.
  • 74.
    74 TREATMENTTREATMENT • Treat thecause 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.
    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.
    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.
    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.
    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.
    80 PROGNOSISPROGNOSIS 3 categories 1. 10%self-limiting 2. 30% with remissions & exacerbations 3. 60% prolonged course without exacerbations
  • 81.
    81 FROSTED BRANCH ANGIITISFROSTEDBRANCH 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.
    83 EPIDEMIOLOGYEPIDEMIOLOGY • Rare, describedin 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.
    84 CLASSIFICATIONCLASSIFICATION 1. Idiopathic 2. Ocularassociations Cytomegalovirus retinitis AIDS retinitis Toxoplasmic chorioretinitis 3. Systemic associations systemic lupus erythematosus Crohn’s disease large cell lymphoma acute lymphoblastic leukemia
  • 85.
    85 PRESENTATIONPRESENTATION SYMPTOMS • acute visualloss • floaters • flashing lights Associated systemic symptoms • flu-like syndrome • sore throat, fever and malaise
  • 86.
    86 SIGNSSIGNS • Acute visualloss • Severe vascular sheathing • retinal edema • Vitritis and iridocyclitis • Otherwise healthy patients
  • 87.
    87 • Papillitis, hardexudates, 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.
    88 INVESTIGATIONSINVESTIGATIONS • 1. Ophthalmoscopy •2. Fluorescein angiogram • 3. Visual field test • 4. Electrophysiological tests: ERG/VECPs
  • 89.
    89 FFAFFA • In earlyphase: 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.
    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.
    92 DDxDDx • Sarcoidosis • Syphilis •Tuberculosis • Multiple sclerosis • Systemic lupus erythematosus • Pars planitis • Eales’disease • Viral • Lymphoma/Leukemia
  • 93.
    93 TREATMENTTREATMENT • Treat thecause • 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.
    96 COMPLICATIONSCOMPLICATIONS • Neovascularization • Neovascularglaucoma • Macular scarring • Retinal detachment
  • 97.
    97 References for FBAReferencesfor 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.
    98 BIRDSHOTBIRDSHOT RETINOCHOROIDOPATHYRETINOCHOROIDOPATHY • 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.
    99 • Deep, oval,creamy, indistinct spots • Radiate from disc towards equator • Moderate vitritis
  • 100.
    100 PATHOPHYSIOLOGYPATHOPHYSIOLOGY • Cause isunknown • 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.
    101 EPIDEMIOLOGYEPIDEMIOLOGY • Rare • Middleage (35-70yrs), average age 50yrs • Caucasians • Female more affected
  • 102.
    102 PRESENTATIONPRESENTATION SYMPTOMS • Decreased vision- 68% • Floaters - 29% • Nyctalopia - 15% • Dyschromatopsia - 12% • Glare - 19% • Photopsia - 17%
  • 103.
    103 • Fluctuating vision- 7% • Pain - 7% • Decreased depth of perception - 5% • Shimmering vision - 3% • Metamorphopsia - 3% • Decreased peripheral vision - 3%
  • 104.
    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.
    105 FUNDUSFUNDUS • Small lesionswith ¼- ½ DD, may be confluent • Two types of lesions, present at posterior pole & extend till equator 1. oval & not well demarcated. Pale yellow or cream color spots easily seen with indirect ophthalmoscopy 2. atrophic, sharply demarcated, round “punched out” seen by both indirect ophthalmoscopy & 78 or 98 D lens
  • 106.
  • 107.
  • 108.
    108 INVESTIGATIONSINVESTIGATIONS LABORATORY WORK-UP • Bloodtesting for HLA-A29 helps to support the diagnosis • Baseline renal function for those who need cyclosporine therapy
  • 109.
    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.
    110 FA of birdshotretinochoroidopathy • Extensive late intraretinal and disc leakage • Venous hyperfluorescence
  • 111.
    111 ELECROPHYSIOLOGYELECROPHYSIOLOGY ERG • Normal inearly 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.
    112 HISTOPATHOLOGYHISTOPATHOLOGY Only 2 histopathologicalstudies (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.
    113 TREATMENTTREATMENT • Steroids • Immunosuppressiveagent (Cyclosporin) • Antifungal (Ketoconazole) • Other immunomodulators • Intravenous immunoglobulin
  • 114.
    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.
    115 CYCLOSPORINCYCLOSPORIN • Very effectivein 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.
    116 KETOCONAZOLEKETOCONAZOLE • Can beused 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.
    117 IMMUNOMODULATORSIMMUNOMODULATORS • Kiss andcolleagues * 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.
    118 FOLLOW-UPFOLLOW-UP • Every 4-6weeks • Query VA color perception night vision adverse effect of medications RFTs, LFTs FFA, ICG
  • 119.
    119 COMPLICATIONSCOMPLICATIONS • Chronic cystoidmacular edema – 50%; the most common cause of reduced central visual acuity • Epiretinal membrane - 10% • Macular pucker • Choroidal neovascularization • Peripapillary sub retinal neovascularization - 6%
  • 120.
    120 • Retinal neovascularizationlocated on the optic disc • Peripheral retinal neovascularization with capillary nonperfusion • Optic nerve atrophy • Other complications, such as cataract, glaucoma, and rhegmatogenous retinal detachment
  • 121.
    121 PROGNOSISPROGNOSIS • Guarded • potentiallyblinding • multiple exacerbations and remissions
  • 122.
    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.
    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.
    124 TAKE HOME MESSAGETAKEHOME MESSAGE • RV can be insidious, sight threatening • Thorough examination • Systemic associations • Treat the cause • Immunosuppressive in systemic diseases
  • 125.
  • 126.
    126 MCQsMCQs 1. Common causeof intermediate uveitis a. multiple sclerosis b. idiopathic c. lyme disease d. syphilis Ans: b, (85-90%)
  • 127.
    127 2. Major causeof visual loss in pars planitis a. band keratopahty b. PSC c. epiretinal membrane d. CME Ans: d
  • 128.
    128 3. The treatmentof choice for BSRC is : a. Topical steroids b. Systemic steroids c. Cyclosporin A d. Oral tolerization of retinal S- antigen Ans: c
  • 129.
    129 4. Which therapeuticalintervention for pars planitis is recommended as first choice? a. Steroid drops b. Systemic steroids c. Non-steroidal anti-inflammatory drugs d. Periocular steroid injections Ans: d
  • 130.
    130 5. What arethe 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.
    131 NEXT LECTURENEXT LECTURE PROPTOSISIN CHILDREN BY DR. MUSHTAQ

Editor's Notes

  • #5 Confirm def. of angiitis
  • #6 most often manifests as a gradual, painless loss of vision , Floaters , Photopsia is a less common presenting feature, as is reduced color perception, although the latter may indicate vasculitis surrounding the macula , central or Para central scotomata  , corresponding to areas of retinal ischemia induced by vascular inflammation and subsequent occlusion , Vasculitis restricted to the peripheral fundus may be entirely asymptomatic ,
  • #22 Elliot called it “Periphlebitis retinae” but later it was caller “Retinal vasculitis”
  • #23 90% according to duke-elder
  • #29 Eales disease. Fundus photo of the peripheral retina, revealing vascular tortuosity and peripheral retinal neovascularization.
  • #43 Fluorescein angiogram of late leakage from peripheral retinal neovascularization in same patient
  • #46 symptomatic
  • #58 For neovesels n vit hemorrhage
  • #62 Just exclude systemic association