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Vasculitis
- 2. presentation Asymptomatic decrease in vision / floaters scotomata
- 3. signs Sheathing or cuffing of blood vessels vitreous cells macular oedema cotton-wool spots retinal oedema Haemorrhages telangiectasis, microaneurysms, and neovascularization - late
- 4. Oct / ffa FFA frequently show that the vasculitis is more extensive leakage - breakdown of the inner BRB staining of the vessel wall
- 5. oct Cme Reproducible In micrometres Treatment response
- 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
- 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
- 16. Systemic primary vasculitis involving the eye Giant cell arteritis Takayasu arteritis Polyarteritis nodosa Kawasaki disease Wegener’s granulomatosis Churg-Strauss syndrome Henoch-Schönlein purpura Cutaneous leucocytoclastic angiitis Essential cryoglobulinaemic vasculitis
- 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
- 26. Systemic secondary vasculitis involving the eye Immune mediated HLA B27-associated uveitis Rheumatoid arthritis , JIA Multiple sclerosis Sarcoidosis Systemic lupus erythematosus Behçet’s disease Susac’s syndrome
- 27. Infectious Mycobacteria Spirochaetes Herpes zoster HTLV vasculitis toxocara Lyme disease Bartonella henselae Whipple’ disease Rickettsial diseases
- 28. HLA-B27–related diseases Ankylosing spondylitis Reactive arthritis syndrome Inflammatory bowel disease Psoriatic arthritis
- 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