Retinal vasculitis is an inflammatory eye disease affecting the retinal vasculature. It can present as periphlebitis (vein involvement), periarteritis (artery involvement), or angiitis (both). Symptoms include gradual vision loss, floaters, photopsia, and scotomata. Signs include vascular sheathing, attenuation, hemorrhages, cotton-wool spots, and neovascularization. Causes include infectious etiologies like tuberculosis and syphilis, autoimmune diseases like sarcoidosis and Behcet's disease, and primary vasculitis like Eales' disease. Treatment involves managing underlying causes with medications like steroids, immunosuppressants,

1. RETINAL VASCULITIS
DR SHRUTI LADDHA

2. INTRODUCTION
• Retinal vasculitis is a sight threatening inflammatory eye disease affecting the
retinal vasculature.
Presents
• Periphlebitis : veins are affected
• Periarteritis : arteries are affected or
• Angiitis : as a combination of both

3. CLINICAL CHARACTERISTICS
3
• 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
SYMPTOMS

4. SIGNS
4
• RAPD in M.S.
• Visual field defects
• Abnormal Amsler grid & color vision
• Elevated IOP in ocular toxoplasmosis
• A/C cells & Flare
• Neovasularisation
• Vitritis
• Vitreous hemorrhages

5. SIGNS OF PERIARTERITIS
5
• Attenuation
• Sheathing (diagnostic)
• Cotton-wool spots
• Opaque superficial retina due to occlusion

6. SIGNS OF PERIPHLEBITIS
6
• Retinal hemorrhages
• Edema
• Telengectasias
• Micro-aneurysms

7. PATHOGENESIS
7
• Primary vasculitis
• Infectious vasculitis
• Immune vasculitis

8. PRIMARY VASCULITIS
8
• Lymphopenia with normal helper T-cells to suppressor T-cells ratio
• Increased concentration 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

9. INFECTIOUS VASCULITIS
9
• 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

10. IMMUNE VASCULITIS
10
• May be T cell mediated as in graft rejection, giant cell arteritis & takayasu
disease
• Ag-Ab & immune complex deposition is main mechanism .
• Anti-endothelial cell antibody and anticardiolipin antibodies are also associated
with retinal vasculitis

11. ETIOLOGY
OCULAR
CAUSES
SECONDARY
CAUSES
VASCULITIS INFLAMMATORY
DISEASES
INFECTIOUS MASQUERADE

12. OCULARCAUSES
12
• Eale’s Disease
• IRVAN Syndrome (Idiopathic Retinal Vasculitis, Aneurysms & Neuroretinitis)
• Intermediate uveitis (Parsplanitis)
• Frosted branch angiitis
• Birdshot retinochoroidopathy

13. SECONDARYCAUSES - VASCULITIS
13
• Giant cell arteritis
• Takayasu arteritis
• Polyarteritis nodosa
• Wegener’s granulomatosis
• Churg-strauss syndrome

14. SYSTEMIC/INFLAMMATORY DISEASE
• 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

15. INFECTIOUS
15
BACTERIAL
• Tuberculosis
• Syphilis
• Lyme disease
• Bartonella henselae
• Whipple’s disease
• Rickettsial disease
VIRAL
• Acute Retinal necrosis
• Cytomegalovirus
• Human immunodeficiency virus
• HTLV vasculitis
• Hepatitis-related vasculitis
PARASITIC
• Toxoplasmosis
• Toxocariasis

16. MALIGNANCY/MASQUERADE
16
• Retinoblastoma
• Ocular lymphoma
• Metastasis
• Leukemia
• Melanoma

17. PHLEBITIS ARTERITIS BOTH
 Tuberculosis
 Sarcoidosis
 Multiple
Sclerosis
 Behcet’s Disease
 HIV
 Eale’s Disease
 Syphilis
 PAN
 ARN/PORN
SLE
 IRVAN
 Toxoplasma
 Wegner’s
granulomatosis
 Crohn’s Disease
 Relapsing
polychondritis
CLASSIFICATION ON BASIS OF VASCULAR
INVOLVEMENT

18. • Tuberculosis
• Sarcoidosis
• Behcet’sDisease
• Multiple Sclerosis
• HIV
• Eales’Disease
DISORDERS ASSOCIATED WITH PERIPHLEBITIS

19. • Tuberculosis affects the lungs in 80% of patients,while in the remaining 20% the
disease may affect other organs,including the eye.
• Posterior uveitis most common presentation
• focal,multifocal or serpiginoid choroiditis,
• solitary or multiple choroidal nodules (tubercles)
• choroidal granuloma (tuberculoma)
• neuroretinitis
• subretinal abscess
• endophthalmitis
• panophthalmitis
• retinal vasculitis, which is frequently ischemic in nature and may lead to
proliferative vascular retinopathy with recurrent vitreous hemorrhage,rubeosis
iridis,and neovascular glaucoma.
TUBERCULOSIS

20. Fundus photograph of the right eye of a 44-year old man with
strongly positive tuberculin skin test demonstrating thick
perivenous sheathing with perivascular exudates along with
superficial hemorrhages suggestive of active vasculitis .

21. Fundus photograph of the left eye of a 36-year old man with
strongly positive tuberculin skin test demonstrating choroid
tubercles, choroid granuloma and areas of active inflammation.

22.  The diagnosis ofocular TB is often problematic
 The absence of clinically evident pulmonary TB does not rule out the possibility
of ocular TB
 In most studies,the diagnostic criteria for presumed tuberculous uveitis were:
 residence or migration from areas endemic in TB,
 previous history of contact withTB-infected patients,
 presence of suggestive ocularfindings,
 exclusion of other known causes of uveitis,
 corroborative evidence such as a positive TST,positive interferon-gamma release
assays (IGRAs),and a positive response toconventional ATT without recurrence.
DIAGANOSIS

23. • Oral prednisone is used in thetreatmentofocularTB,in order to control thecoexisting
inflammatory reaction,and reduce macular edema.
• Proliferativestage of neovascularization - laser photocoagulation.
• Patients withnon-resolving vitreous hemorrhage and withTRD - pars plana vitrectomy
and adequate endolaser photocoagulation.
TREATMENT

24. • Can beassociated with
• uveitis,
• episcleritis/scleritis,
• eyelid abnormalities,conjunctival granuloma,
• optic neuropathy,
• lacrimal gland enlargement
• orbital inflammation.
• Intermediate uveitis
• Young adults affects females more commonly with
bilateral hilar lymphadenopathy,ocular and skin
lesions.
• Ocular involvement in 60% of systemic cases,
predominantly presenting as anterior granulomatous
uveitis.
• Retinal periphlebitis - non occlusive associated
segmental cuffing or“candle wax drippings’’
SARCOIDOSIS

25. Ocular signs suggestive for ocular
sarcoidosis
1. Mutton-flat keraticprecipitates
and/or irisnodules
2.Trabecular meshwork nodules
and/or tent-shaped peripheral
anterior synechiae
3.Snowballs or string of pearls in the
vitreous
4. Active or inactive multiple
chorioretinal peripherallesions
5. Nodular and/or segmental
periphlebitis and/ormacroaneurysms in
an inflamed eye
6.Optic disc nodule(s)/granuloma(s)
and/or solitary choroidalnodule
7. Bilateral involvement
Laboratory investigations in patients suspected for
ocular sarcoidosis
1. Negative tuberculin test
2.Elevated serum level of angiotensin converting enzyme
(ACE) and/orlysozyme
3.Chest x-ray for bilateral symmetric hilar adenopathy
4.Abnormal liver enzyme tests (any 2 of ALT,LDH,AST,GGT)
5.Chest computerized tomography in patients with negative chest
x-ray
DIAGNOSIS

26. Fundus photograph of a patient with documented sarcoidosis
demonstrating segmental perivenous sheathing with hemorrhage.

27. • The treatmentis oral steroids in theactive stage ofinflammation.
• Immunosuppressive agents like Methotrexate, Azathioprine and Cyclosporine.
• Laser treatment in proliferative stage.
• PPV in patients ofnon-clearing vitreous hemorrhage and tractional RD
TREATMENT

28. • Multisystem inflammatory vasculitis with periodic
recurrences causing obliteration, necrosis, and
fibrosis of vascular system where itextends.
• Recurrent occlusive vasculopathy affecting both
(predominantly) veinsand arterioles and
spontaneous remission.
• Oral and genital mucosa,skin,and eyes involved.
• b/Lnon-granulomatous panuveitis and retinal
vasculitis – most common.
BEHCHETS DISEASE

30. • Corticosteroids are themainstay oftreatment.
• Combination with cyclosporine has synergistic effect.
• Other immunosuppressive agents like tacrolimus and azathioprine can be used.
• Interferon-alphahas role in treatingmild or moderate exacerbations of Behcet’sdisease
• Infliximab is used in limited tovision-threatening uveitis .
TREATMENT

31. • Itis a chronic disease thatcauses demyelination and
sclerosis in CNS.
• Ageof onset 20-40 years.
• More common in females.
• 95 % cases are bilateral.
• Retinalperiphlebitis may occur in 5-10% ofcases.
• Active lesion:perivenular infiltratesare present which can
progress to occlusive peripheral vasculitis leading to
neovascularization,vitreous haemorrhage & tractional
retinal detachment.
• Intermediate uveitis and panuveitis are themost common
categories ofMS associated uveitis.
MULTIPLE SCLEROSIS

32. • Mostly vasculitis in HIV patientsis associated with C M V retinitis.
• Acquisition ofthevirus occurs through placental transfer, breast feeding, saliva,sexual
contact,blood transfusions,and organ or bone marrow transplants.
• Infection ofCMV leads tolife-long persistence,thevirus becomes dormant and
remains in latency.
• Activation occurs in patients with immature or compromised immune systems, leading
tosystemic infection of lungs (pneumonitis),gastrointestinal tract(colitis),CNS
(encephalitis), and retina(retinitis).
HIV

33. Fulminant CMV retinitis large posterior retinal tear with shallow
localized detachment – there is vascular
sheathing reminiscent of frosted branch angiitis

34. Acute retinal necrosis -advanced
disease reaching the posterior
pole
Progressive retinal necrosis-established
disease resulting from confluence of multiple
foci – there is little or no haemorrhage

35. • Inflammation can lead tofullthickness retinal necrosis and eventually
development ofretinal holes and tears.
• Area ofretinal necrosis may spread atthe rateof24 μ/day in untreated patients.
• Large size and anterior location oflesions raise therisk of developing RD.
• Treatment modalities IV ganciclovir or foscarnet,oral valganciclovir or
cidofovir
• Intravitreal implant ofganciclovir active for 6-8 months

36. • Described in 1880 by Henle Eale, as an idiopathic
inflammatory venous occlusive disease of young
adult males with recurrent vitreous hemorrhage and
tractional retinal detachment.
• Prevalence in India ,1 % ofadult population.
• Three hallmark signs ofEales’disease:
• Retinalphlebitis
• Peripheral nonperfusion
• Retinalneovascularization.
• Features ofvitritis ,uveitis are absent
• Diagnosis ofexclusion
EALES DISEASE

37. • Hypersensitivity totuberculo-protein, tuberculosis, immune mediated
mechanisms, raised peptide growth factors like PDGF, IGF, EGF, TGF alpha &
beta, VEGF, oxidative stress and hyper-homocystinemia are thelikely causes put
forthfor thisdisease.
• The condition is treated in active stage by steroids, in stage of neovascularization
by photocoagulation & non- clearing vitreous hemorrhage & TRD by PPV.

38. • Syphilis
• SLE
• Acute Retinal Necrosis(ARN)
• PAN
DISORDERS ASSOCIATED WITH PERIARTERITIS

39. • Syphilis needs tobe ruled out in any case of retinal vasculitis as its a great
imitator.
• Wide variety of lesions:
 focal or multifocalchorioretinitis
 acute posterior placoid chorioretinitis
 necrotizing retinitis
 retinal vasculitis
 intermediate uveitis,and panuveitis
 neuroretinitis
 opticneuritis
• More commonly arterial but isolated periphlebitis -also noted.
• Treatment includes I/V penicillin G 12-24 million units daily for 10-15 days.
• Or I/M procaine penicillin 2.4 million units daily,with oral probenecid.
SYPHILIS

40. a- hyperemic disc (red arrow) with posterior placoid retinochoroiditis (yellow
arrow)
b- hyperemic disc (red arrow) and superficial retinal precipitates (blue arrow)

41. • Retinalvascular lesions are themost common ophthalmic manifestations inSLE.
• Patientmay present with cotton wool spots,with or without retinal hemorrhages.
• Diffuse arteriolar occlusion withextensive capillary non perfusion.
• Patients with raised anti-phospholipid antibody have a higher risk of occlusive
retinal vasculitis.
• Exacerbations ofdisease activity may present only in theretina as retinal vascular
occlusions.
SLE

42. Fundus
showing
arrowmarked
CWS, vessel
attenuation
and
hemorrhage
FFA showing
occlusion.

43. DIAGNAOSIS
• The clinical manifestations+
• Higher titres of anti-double stranded DNA antibody
• Raised anti nuclear antibody,
• Positive lupus erythematous cell phenomenon
• Hypergammaglobulinemia
• Raised circulating immune complexes
• Reduced serum complement

44. • In 1971,Akira Urayama described acuteretinal necrosis (ARN).
• Occur in otherwise healthy patients.
• VZV less often by HSV 1 &2
• Clinical examination shows significant anterior uveitis, corneal edema,keratic
precipitates,and posterior synechiae,raised IOP.
• The typical fundus picture is thatof vitritis with confluent areas of mid peripheral,
deep retinal whitening with associated intra-retinal hemorrhages.
ARN

45. • The earliest retinal lesions are subtle,isolated retinal opacities that may assume a patchy,
granular or nummular configuration, depending on theirstage ofevolution.
• Although usually seen in the mid-periphery and pre-equatorial regions, small
nummular lesions may also be seen in the posterior retina, generally sparing themacula.
• With progression ofthe syndrome, the granular and nummular lesions increase in size
and coalesce toform confluentzones of full-thickness retinalnecrosis.
• These lesions,which are typically situated in theretinal periphery, may occupy as little as
1–2 clock hours or may extend to completely encircle theretina over 360°.
• Diagnosis is mainly clinical,PCR may be done in uncertainty.
• Response toantiviral treatmentis confirmatory.

46. • In 1990,Forster and colleagues identified a ARN like syndrome affecting
immunocompromised individuals and coined theterm "progressive outer retinal
necrosis," or PORN.
• The retinitis start in the macula or in the periphery with patchy, multifocalouter
retinal lesions coalescing rapidly throughout the retina.
• reactivation of herpes zoster virus, although herpes simplex virus (HSV-1) has also
been reported to cause PORN.
• Severe visual loss from thediffuse retinal necrosis,optic atrophy,and retinal detachment
occurs in up to7 0 % ofpatients.
• Progression is extremely rapid,occurring over days or even hours.
PORN

47. .

48. Acute retinal necrosis -advanced
disease reaching the posterior
pole
Progressive retinal necrosis-established
disease resulting from confluence of multiple
foci – there is little or no haemorrhage

49. • The goals oftreatmentin acute viral retinitisare:
 Arresting active viral infectionin theretina.
 Preventing contralateralspread ofthedisease.
 Minimizing secondary,inflammatory intraocular damage.
 Preventing andtreating retinal detachment.
• Systemic treatment is started with intravenous acyclovir, 10 mg/kg 3 times daily for 5 to
10 days,followed by oral acyclovir 800mg 5/d for three months atleast.
TREATMENT OF NECROTIZING RETINITIS

50. • Unlike ARN,PORN does notrespond very well tosystemic antiviral therapy.
• The prognosis for vision remains guarded,with two-thirds ofeyes having finalvisual
acuity ofno light perception.
• Ideally,thePORN patientwith AIDS should be started on HAART.
• While intravenous antiviral therapy is usually employed,thedisease may not respond
well toIV acyclovir alone. For this reason, some physicians initiate treatment of PORN
with intravitreal injections of ganciclovir or foscarnet in addition toIV antiviral therapy.
• Combination systemic therapy with ganciclovir plus foscarnet is often used for
induction and maintenance ofPORN.
TREATMENT OF PORN

51. • Itis necrotizing vasculitis ofsmall and medium sized arteries in all organs.
• Vasculitis commonly involves theheart,kidneys,liver, GIT,CNS.
• Ocular involvement is present in 10-20 % ofpatients.
• Periarteritis consist ofcotton wool spots,haemorrhages,oedema & central retinal artery
occlusion.
• Other ocular manifestations include peripheral ulcerative keratitis, necrotizing scleritis,
non granulomatous iritis, vitritis, papilitis & ischaemic opticneuropathy.
POLYARTERITIS NODOSA

53. • Toxoplasmosis
• Wegner’sGranulomatosis
• Frosted BranchAngiitis
DISORDERS ASSOCIATED WITH BOTH
POLYARETERITIS AND POLYPHLEBITIS

54. • Caused by obligate intracellular parasite Toxoplasma gondii.
• Hallmark is focal necrotizing retinitis causing characteristic atrophic scar.
• Severe vitritis greatly impair visualization ofthefundus ,although the inflammatory
focus may still be discernible known as ‘headlight in fog appearance.’
• Reactivation is near old scar.
• Vasculitis can be near to or distanttotheretinochoroiditis lesion.
• More severe in immunocompromised patientsand atypical features may occur i.e.
bilateral, confluent areas of retinitis, no pre-existing scar.
TOXOPLASMOSIS

55. Head light in fog Toxoplasma scar

56. • Clinical history andfundus examination is supported by serologic evidence of
T.gondii exposure.
• The high incidence of IgG antibodies in the population is due topast infections.
• Demonstration of the local synthesis ofToxoplasma antibodies in the eye by
intraocular fluid analysis is a valuable diagnostic tool.
• Cytologic identification of T.gondii from vitreous specimens has been described.
• The diagnosis of congenital disease in newborns is established by the detection of
specific IgM or IgA antibodies or the demonstration of stable or rising titers of IgG
antibodies for a period of several months afterbirth.
• Intraocular fluid samples may be assessed by the polymerase chain reaction for
Toxoplasma DNA.
• Rarely is a chorioretinal biopsy performed toshow T.gondii organism.
DIAGNOSIS

57. AIM:
• To reduce the
duration and severity
ofacute inflammation
• To lessen therisk of
permanent visual loss
• To reduce the risk of
recurrence
TREATMENT

58. • Necrotizing granulomatous vasculitis typically ofupper and lower airways and
kidneys.
• Ocular involvement occurs in 28- 5 8 % cases and may even precede other organ
involvement.
• Ocular manifestations,include orbital involvement secondary to invasion by paranasal
granulomata, nasolacrimal duct obstruction, episcleritis,scleritis,corneal ulceration,
optic nerve vasculitis,retinal artery occlusion,choroidal arterial occlusion,and retinal
vasculitis.
• The diagnosis ofWegener’s granulomatosis is based on typical clinical findings and
supporting histologic data.
• The classic‘cytoplasmic’staining pattern (cANCA) is seen in Wegener’s
granulomatosis.
WEGNERS GRANULOMATOSIS

60. • Frosted branch angiitis is a rare vasculitis where thick inflammatory infiltrates
surround the retinal arterioles and venules creating an appearance of frosted tree
branches.
• In addition patient has retinal haemorrhages,hard exudates &serous retinal
detachments of macula & periphery.
• Fundus fluorescein angiography demonstrates leakage ofdye but no evidence of
decreased blood flowor occlusion.
• Various suspectedetiologies are infiltration with malignant cells (lymphoma or
leukemia), SLE, Crohn’s disease, toxoplasmic retinochoroiditis, human T-cell lymphoma
virus type 1 infection, HIV infection,herpes simplex virus infection,Epstein–Barr virus
infection.
FROSTED BRANCH ANGIITIS

62. • Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare clinical entity
characterized by bilateral retinal arteritis, numerous aneurysmal dilatations of the retinal
and optic nerve head arterioles, peripheral retinal vascular occlusion,neuroretinitis,
and uveitis.
• Young females.
• Visual loss is due toexudative maculopathy and neovascular sequelae of retinal
ischemia.
• The resolution ofaneurysmal dilatations ofthe retinal arterioles in patients with IRVAN
treated with systemic steroids and peripheral retinalphotocoagulation.
IRVAN

63. A,B-Extensive deposition of hard
exudates in the posterior pole and
peripapillary location, along with
aneurysmal dilatation of arteries
C,D- after treatment with laser
photocoagulation and multiple
dexamethasone injections 7 years
later, there was a decrease in hard
exudates, along with a decrease in
arteriolar aneurysms

64. Disease History
Behcet’s disease Orogenital ulcers, arthralgia, skin rash-
Sarcoidosis Weight loss, cough, skin lesions, hilar lymphadenopathy
Tuberculosis Night fever,sweats, cough with expectoration, weight loss, loss of
appetite
Seronegative arthropathy Joint pains, backache
Multiple sclerosis Neurological symptoms
APLA Thromboembolic episodes
ROLE OF PATIENT HISTORY

65. Step ladder
approach to treat
Retinal Vasculitis.
TREATMENT