211 Vasculitis Final.docx

Introduction   Vasculitis Definition Vasculitis is the inflammatory destruction of blood vessels. Leukocyte migration leads to vessel destruction, arteritis, and phlebitis (veins). Vasculitis can be classified by affected organs and vessels (~20 types).   Primary Vasculitis Most common (81% of...

Introduction   Vasculitis Definition Vasculitis is the inflammatory destruction of blood vessels. Leukocyte migration leads to vessel destruction, arteritis, and phlebitis (veins). Vasculitis can be classified by affected organs and vessels (~20 types).   Primary Vasculitis Most common (81% of cases) Secondary Vasculitis Less common (19% of cases) No known cause (Idiopathic) Autoimmune or infectious etiology Giant cell arteritis (41%) Polyarteritis nodosa (5%) Granulomatosis w/ polyangiitis (3.4%) Churg-Strauss Syndrome (<1%) Connective tissue diseases (11.6%) Rheumatoid Arthritis (4.5%) Systemic Lupus Erythematosus (3.7%) Mixed/Other (3..4%) Malignancy (3%) Behcet disease (<1%) Sarcoidosis(<1%) Sjogren's Syndrome (<1%)       Vasculitis MOA Weakening of blood vessels from inflammation leads to thrombosis and/or aneurysm formation. This can then cause ischemia or hemorrhaging.   Complications S&S Labs/Testing Tx Stroke Most common comp. for large vessel vasculitis Renal failure Most common comp. for small vessel vasculitis Glomerulonephritis HTN MI Respiratory disorders Dermatological disorders Infections Gangrene Palpable purpura Palpable raised lesions; Rash Reduced VA Acute visual loss Fever Weight loss HA Myalgia Arthralgia Bloody cough Hematuria Bloody stool Abdominal pain Nose bleeds Anti-neutrophil cytoplasmic antibodies (ANCA) Sedimentation rate (ESR) & C-reactive protein (CRP) Nonspecific markers of inflammation Anti-nuclear antibody (ANA) CBC w/ differential Biopsy Angiography Urinalysis   Address underlying illness NSAIDs/Steroids Other immunosuppressants   Gangrene Palpable Purpura   ESR ANA ANCA   Important Vasculitides to Know (5)   1) Giant Cell Arteritis (GCA) (Temporal Arteritis) (41%; Most common vasculitis) One of the most important medical emergencies in ophthalmology (high risk of vision loss) Vision loss is preventable with early dx and tx (one of the 5 A's!)   Prevalence Affected Vessels Symptoms 1/10k people >50 annually ~228k people in the US 70-80 mean age of onset >70 yo → 80% of cases >60 yo → 90% of cases F>M (3:1) Whites >> AA & Asian Large and Medium vessels Head, arms, neck Aorta Ophthalmic artery →blindness Posterior ciliary arteries →ION HA (56-72%) Jaw claudication (40-48%) Ischemia to masseter muscle Scalp tenderness (40-43%) TA tenderness (20-23%) Polymyalgia rheumatica (52%) Low-grade joint stiffness Shoulders and hips Ocular findings (50-70%)   Etiology Complications Dx Tx Idiopathic Irreversible blindness MI (10%) Stroke (2-7%) Death possible 35% 5yr survival rate Duplex sonography of TA (+)ESR & (+)CRP tests 97% specificity! TA biopsy (definitive Dx) Characteristic symptoms Immediate oral steroids 60-100 mg prednisone Up to 1-2 yrs Actemra New from 2017   Temporal Artery Biopsy (+ vs. -) Duplex Sonography of TA (+) = narrowed lumen (-) = normal lumen size Dark halo = thickened vessel wall Lumen can narrow   Ocular Manifestations     Some form of vision loss (up to 92%) ION (ischemic optic neuropathy) (82%) AION (76%) (anterior) PION (6%) (posterior) Cotton wool spots (33%) Amaurosis fugax (26%) Retinal artery occlusions (13-25%) Diplopia / CN palsies (7-11%) Cotton wool spots Anterior ION   21% of (+)TAB GCA pts have vision loss WITHOUT systemic symptoms!   2) Polyarteritis Nodosa (PAN) (5%)   Prevalence Affected Vessels Etiology Rare M>F Onset 40-60 yo Small & Medium Arteries Full thickness affected Necrotizing inflammation Idiopathic Subset associated with Hep B   Complications Dx Tx Peripheral neuropathy (50-70%) GI disorder / infarct (15-65%) Renal failure (~33%) MI (12%) Stroke (8%) Leg rashes and livedo reticularis Lungs are SPARED Angiography ANCA (-) ESR elevation possible Tissue biopsy Immunosuppressants Fatal if un-tx (2-5 years) Renal failure and GI infarcts     Kidney Aneurysm Aneurysm (Angiography)   Gangrene Leg Rash Livedo Reticularis   Ocular Complications (10-20%) Scleritis/Episcleritis Peripheral Ulcerative Keratitis Retinal Vasculitis &/or Artery occlusion Orbital pseudotumor   3) Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) (3-4%)   Prevalence Affected Vessels Etiology Onset 40-65 yo White >> Others (90%) Small & Medium Arteries Upper respiratory tract (70%) Nose, trachea, sinuses Chronic rhinitis and nosebleeds Lower respiratory tract (85%) Lungs Kidneys (77-85%) Idiopathic   Dx Tx ANCA (+) (90%) Biopsy (+) (75-85%) Orbit MRI/CT Orbital pseudotumor Immunosuppressants 80-90% survive with tx >8 yrs Fatal if un-tx (2 years)   Saddle-Nose Chest CT (Lung granulomas) X-ray (Lung Granulomas)   Ocular Manifestations (50-60%)     Orbital Pseudotumor (30%) Diplopia and Reduced VA Ulcerative keratitis Episcleritis Scleritis Uveitis GPA-associated Pseudotumor GPA-associated Sclerokeratitis   4) Systemic Lupus Erythematosus (SLE) (Secondary vasculitis found in 3.7% of cases) Chronic, systemic autoimmune connective tissue disorder. Type III hypersensitivity (immune complex deposition) Systemic inflammation (joints, kidneys, lungs, heart, brain) but primarily affects skin and joints   Prevalence Etiology Dx Tx 20-150 / 100K F>>M (10:1) Onset 15-40 yo AA>Asian>White Idiopathic ANA (+) (95%) Clinical exam findings Immunosuppressants   Malar Butterfly Facial Rash Discoid Rash on Hands   Ocular Manifestations (33%)   Dry Eye Syndrome (33%) Diplopia and Reduced VA Diplopia / CN palsies (29%) Lupus retinopathy (10%) Retinal vasculitis Retinal artery occlusion Cotton wool spots Episcleritis / Scleritis (2%) Optic neuritis (1%) Ulcerative keratitis (rare) Uveitis (rare) Orbital pseudotumor (rare) Dry Eye Syndrome Lupus Retinopathy     5) Behcet's Disease / Syndrome (<1%; Preference for veins)   Prevalence Complications Etiology Tx Middle eastern, Turkish, Asian 20-40 yo.s M>F Rare 20% mortality rate over 7 yrs Mouth sores (canker sores) (90-100%) Pain, swelling, stiffness of joints (72%) Genital sores / ulcerations (62%) Skin sores (acne-like) (55%) CNS (stroke, HAs) (16%) GI ulcerations (13%) Autoimmune Immunosuppressants     Ocular Manifestations Inflammation Inside the Eye (50-75%) S&S Dx Retinal phlebitis (82%) Preference for veins! Uveitis / Iritis (63%) Optic neuritis (21%) Glaucoma (17%) Blurry vision Pain Redness Photophobia HLA-B51 (72-79%) Behcetine pathergy test (79%)     Ocular Triad = Behcet's AKA "oculo-oro-genital syndrome"   Iritis with hypopyon Mouth sores Genital sores   Retinal Phlebitis Behcetine Pathergy Test Iritis + Hypopyon     Summary Tables   Vasculitides Most Common Ocular Clinical Findings Most Common Lab Test Results Giant Cell Arteritis ION (+)ESR, (+)CRP Polyarteritis Nodosa Mixed (+)ESR??, (-)ANCA Granulomatosis w/ Polyangiitis Orbital pseudotumor (+)ANCA, (-)ANA Systemic Lupus Erythematosus Dry eye syndrome (-)ANCA, (+)ANA Behcet’s Disease Retinal phlebitis (+)HLA-B51, (-)ANCA, (-)ANA   Large Vessel Medium Vessels Small Vessels Behcet’s Disease Polyarteritis Nodosa Granulomatosis w/ Polyangiitis Giant Cell Arteritis   SLE Vasculitis   ***Ocular findings, systemic findings, and lab tests are used in conjunction with one another to come up with the diagnosis…look at the overall clinical picture!