6. OCULAR VASCULITIS
VERSUS RETINAL VASCULITIS
more global concept of ocular vasculitis including retinal vasculitis is
useful
encompass episcleritis, scleritis, (PUK), retinal vasculitis, choroidal
vasculitis, optic nerve vasculitis
7. Systemic vasculitis
Vasculitis – histologically proven inflammation of vessel wall
Primary
Large vessel
Medium
small
secondary vessel changes without histologic evidence
Secondary vasculitis / vasculopathy
8. Ocular vasculitis
Primary vasculitis limited to the eye
Secondary vasculitis limited to eye
Systemic primary vasculitis involving the eye
Systemic secondary vasculitis involving the eye
9. PRIMARY VASCULITIS LIMITED TO
THE EYE
Episcleritis without any systemic involvement
Scleritis and peripheral ulcerative keratitis (PUK) without systemic involvement
Retinal vasculitis
Idiopathic retinal vasculitis – Eale’s
pars planitis
Frosted branch angiitis
IRVAN
Acute multifocal haemorrhagic retinal vasculitis
Choroidal vasculitis
MEWDS
APMPPE
MFC
Serpiginous choroiditis
10. Eale’s
Obliterative periphlebitis
venous sheathing are the commonest clinical presentation
Starts at or around equator and
progresses posteriorly
compensatory phenomena like collaterals, microaneurysms, capillary
telangiectasia, corkscrew vessels, and venous beading
11.
12. can lead to neovascularization - peri[heral
recurrent vitreous haemorrhage
Traction retinal detachment
affect healthy young adults in the third and fourth decades
Men> women
Etiopathogenesis – type III hypersensitivity reaction ?hypersensitivity to
tuberculoprotein
(HLA) B5 (B51), DR1, and DR4
13. systemic steroids
panretinal photocoagulation
vitrectomy – endolaser in non-resolving VH with FVP
14. Secondary inflammatory vasculopathy
limited to eye
Episcleritis, Scleritis and PUK secondary to local infection
Retinal inflammatory vasculopathy
Immune mediated
–Birdshot chorioretinopathy retinal vasculitis
– Ocular sarcoidosis
Infectious or para-infectious
– Necrotic herpetic retinopathies (herpes simplex virus, varicella-zoster virus)
– Toxoplasma
– – DUSN (Diffuse unilateral subacute neuroretinitis, due to parasites, Toxocara canis)
Neoplasms
– Primary ocular lymphoma
15. Choroidal inflammatory vasculopathy or vasculitis
secondary choriocapillaropathy
adjacent to retinitis or choroiditis)
Secondary stromal vasculitis
-Immune mediated
– Birdshot choroiditis (choroidal disease of birdshot chorioretinopathy)
– Sympathetic ophthalmia
– Toxoplasmic retinochoroiditis
– Vogt-Koyanagi-Harada disease
– ocular Sarcoidosis
-Infectious
17. Giant cell arteritis
affects large and medium-sized arteries
50 years or older
headache and tenderness of the temporal artery or scalp
Jaw or tongue claudication
malaise, anorexia and weight loss, fever, neck pain, joint and muscle pain,
and ear pain
Visual symptoms may include transient or permanent visual loss, diplopia,
and eye pain
18. (AAION) is the most common cause of vision loss but
central retinal artery occlusion, cilioretinal artery occlusion
posterior ischemic optic neuropathy
ocular ischemic syndrome also occur
posterior ciliary arteries, choroidal ischaemia
anterior segment ischaemia (uveitis and episcleritis
extraocular muscle palsies
19. Westergren ESR (mean 70 mm/hr; often >100 mm/hr) - may be normal in
up to 16% of cases
CRP level
Temporal artery biopsy – CONFIRMATORY (false –ve 3 – 9 %)
IV MP (1 g/day for the first 3–5 days)
suspected GCA without loss of vision, oral prednisone
continue therapy for at least 1–2 years
20. Polyarteritis nodosa
medium-sized and small muscular arteries
40 and 60 years and affects men 3 times more
hepatitis B ?
Ocular involvement is present in up to 20%
fatigue, fever, weight loss, and arthralgia
mononeuritis multiplex is the most common
Renal involvement – HT - may manifest as hypertensive retinopathy
small-bowel ischemia and infarction
21. Scleritis, PUK, episcleritis, conjunctivitis and conjunctival vasculitis
choroidal vasculitis most common ocular involvement (posterior ciliary
arteries, large and small choroidal vessels → choroidal ischaemia
Cranial nerve palsies, amaurosis fugax, homonymous hemianopia, Horner
syndrome
The 5- year mortality rate of untreated PAN is 90%
Combinaton steroid + IMT – 80%
22. Wegener’s granulomatosis
Granulomatosis with polyangiitis
classic triad
Involvement of the paranasal sinuses is the most characteristic
followed by pulmonary and renal disease
Dermatologic involvement - one-half of patients, purpura (lower
extremities) ulcers and subcutaneous nodules
Nervous system - one-third of patients - peripheral neuropathies;
mononeuritis multiplex and less frequently cranial neuropathies, seizures,
stroke syndromes, and cerebral vasculitis
23. Ocular involvement in 50 %
Orbit most common - contiguous extension
Dacryocystitis
Scleritis of any type – 40%
Ant, int., post. Uveitis
Retinal involvement 10% - cotton-wool spots, intraretinal hemorrhages,
branch or central retinal artery or vein occlusion
Retinitis - 20% of patients; may accompany retinal vasculitis - retinal
neovascularization, vitreous hemorrhage, neovascular glaucoma
24. Tissue biopsy
chest x-ray - nodular, diffuse, or cavitary lesions
proteinuria or hematuria
elevated (ESR) and CRP level
ANCA - GPA, MPA, eosinophilic granulomatosis with polyangiitis (Churg-
Strauss syndrome), renal limited vasculitis, and pauci-
immunoglomerulonephritis
25. Immunofluorescence pattern of ANCA
c-ANCA (PR3 - ANCA)
present in up to 95% of patients of GPA
p-ANCA (Myeloperoxidase) - MPA, renal limited vasculitis, and pauci-
immunoglomerulonephritis
Without therapy, the 1-year mortality rate is 80%.
cyclophosphamide and corticosteroids 93% successfully achieve remission
with resolution of ocular manifestations
29. Ankylosing spondylitis
Asymptomatic
lower back pain and morning stiffness, (Often, persons with anterior uveitis
lack symptoms of back disease)
90% - HLA B27 +ve
Sacroiliac imaging
Pulmonary apical fibrosis and cardiovascular disease (aortic valvular
insufficiency) may also develop
NSAIDs
Sulfasalazine
anti-TNF drugs
exercise, physical therapy, and smoking cessation
30. Reactive arthritis syndrome
Reiter syndrome
triad of nonspecific urethritis, polyarthritis, and conjunctival inflammation,
often accompanied by nongranulomatous anterior uveitis
keratoderma blennorrhagicum:
circinate balanitis
HLA-B27 – 95%
triggered by episodes of diarrhea or dysentery
Ureaplasma urealyticum, Chlamydia, Shigella, Salmonella, and Yersinia
Arthritis begins within 30 days of infection in 80% of patients
31. knees, ankles, feet, and wrists
Asymmetrical Oligoarticular
Eye involvement - 20%. Conjunctivitis is the most common
Punctate and subepithelial keratitis
Acute nongranulomatous anterior uveitis occurs in up to 10% of patients
and may become bilateral and chronic
32. Juvenile idiopathic arthritis
Pedia ant uveitis – most common syst. Assoc. is JIA
arthritis begins before age 16 and lasts for at least 6 weeks
Risk factors for chronic uveitis in JIA patients - female sex, oligoarticular
onset, and the presence of ANA
Most patients test negative for rheumatoid factor
Ocular involvement in JIA JIA can be classified into 3 types
Systemic onset (Still disease) - 10 – 15%
under age 5 years,
fever, rash, lymphadenopathy, and hepatosplenomegaly
Joint involvement may be minimal
fewer than 6% of patients have uveitis
33. Polyarticular onset - 40%
involvement of > 4 joints in the first 6 months of the disease
Oligoarticular onset - 40%–50%
(80%–90%) of patients with JIA-associated uveitis
34. Multiple sclerosis
Uveitis is 10 times more common in MS
Bilateral intermediate uveitis is the most common manifestation
cross-reactivity between myelin-associated glycoprotein and Müller cells
35. SLE
connective tissue disorder
women of childbearing age
polyclonal B-lymphocyte activation, hypergammaglobulinemia, immune
complex deposition - end-organ damage
ANA - anti-ssDNA and anti-dsDNA
antibodies to cytoplasmic components (anti- Sm, anti-Ro, and anti-La)
antiphospholipid antibodies
36. Ocular manifestations - in 50%
cutaneous lesions on the eyelids (discoid lupus erythematosus),
Scleritis, episcleritis
secondary Sjögren syndrome in 20% of patients
neuro-ophthalmic lesions (cranial nerve palsies, optic neuropathy, and
retrochiasmal and cerebral visual disorders)
retinal vasculopathy
in rare cases, uveitis
37. Lupus retinopathy
Cotton-wool spots with or without intraretinal hemorrhages occur
independently of hypertension and are due to the microangiopathy
arterial and venous thrombosis - related to anti phospholipild
autoantibodies induced hypercoagulable state
Lupus choroidopathy - choroidal infarction and choroidal
neovascularization
NSAIDs, corticosteroids, IMT, plasmapheresis, and systemic
antihypertensive
antiplatelet therapy or systemic anticoagulation
