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Review of Uveitis

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By Dr. Fritz Allen

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Review of Uveitis

  1. 1. Fritz Allen, MD Visionary Eye Doctors Review of Uveitis
  2. 2. • A generic term for intraocular inflammation. • Does not indicate site of inflammation • Does not indicate the cause: Autoimmune or Infectious Uveitis: Definition
  3. 3. How Common is Uveitis?  10-15% of severe visual handicap in the U.S.  3rd leading cause of blindness in the world  U.S. Incidence 52.4/100,000  U.S. Prevalence 115.3/100,000  3 times higher than previous estimate  Prevalence higher in women (1:1.4)  Common in older patients  Gritz and Wong. Ophthalmology 2004  Worldwide prevalence ~2.4 million  ~5-10% of cases in children <16 yrs  Mean age of onset is 37.2 years  Range 20-50 years 151,200 322,000 2,400,000 U.S. Prevalence WorldwideU.S. Incidence
  4. 4. IUSG Classification of Uveitis Anterior uveitis  iris and pars plicata (CB) Intermediate uveitis  pars plana and vitreous Posterior uveitis  retina + choroid Panuveitis “front” “back”
  5. 5. Cells per high-power field in 1x1 mm slit beam 0 = < 1 cell/hpf 0.5+ = 1 - 5 cells 1+ = 6 - 15 2+ = 16 - 25 3+ = 26 - 50 4+ = > 50 Flare 0 = none 1+ = faint 2+ = moderate, (iris/lens details clear) 3+ = marked (iris/lens hazy) 4+ = intense (fibrin or plastic aqueous) SUN Grading system for AC cell and flare
  6. 6. Number of Cells* Description Grade 0-1 clear 0 2-20 few opacities trace 21-50 scattered opacities 1+ 51-100 moderate opacities 2+ 101-250 many opacities 3+ >250 dense opacities 4+ *cells are counted using a Hruby, 90 or 78 diopter lens National Eye Institute Grading System for Vitreous Cell (No SUN Working Group Consensus)
  7. 7. 0 = Clear 0.5+/trace = Trace 1+ = Few opacities, mild blurring 2+ = Significant blurring but still visible 3+ = Optic nerve visible, no vessels seen 4+ = Dense opacity obscures optic nerve head National Eye Institute Grading System for Vitreous Haze (adopted by SUN Working Group)
  8. 8. Developing a Differential Diagnosis  Is the disease acute or chronic?  Where is the inflammation located in the eye?  Unilateral or bilateral?  Granulomatous or non-granulomatous?  What are the demographics of the patient?  Associated symptoms?  Associated signs on physical exam?  How did the disease respond to previous therapy?
  9. 9. Anterior Uveitis: ~60% of all uveitis Idiopathic HLA-B27 associated  Inflammatory bowel disease  Ankylosing spondylitis  Reiter’s syndrome  Psoriatic arthritis JIA (Juvenile Idiopathic Arthritis) associated Behçet’s disease Fuchs’ heterochromia Sarcoidosis Syphilis Glaucomatocyclitic crisis Masquerade syndromes
  10. 10. Anterior uveitis prevalence: 81/100,000 (Gritz et al)
  11. 11. Differential Diagnosis of Stellate Keratic Precipitates:  Fuchs heterochromia (rubella, herpes, toxoplasmosis)  Viral  Toxoplasmosis
  12. 12. Differential Diagnosis of hypopyon:  HLA-B27 associated  Behçet’s disease  Low back pain, ethnicity, GI symptoms, ulcers, joints
  13. 13. JIA-associated uveitis  <16 yo,>6 mo disease  Pauci-articular: 25%  Type 1=ANA+ young girls  Type 2=Older boys B27+  Poly-articular: ~15%  Systemic onset: 1-5% Most at risk: ANA+, RF-, pauci-articular girls Uveitis develops within 5-7 yrs No correlation betw joint and eye Frequently asymptomatic Uveitis before joint disease poor px BK/PS/cataract/ON hyperemia/CME common
  14. 14. Complications Treatment Posterior synechiae Cataract  Inflammation-related  Steroid-induced Secondary glaucoma  Steroid response  Angle closure Cystoid macular edema Band keratopathy  more common in children Topical corticosteroids Cycloplegics Glaucoma gtts NSAIDs (gtt or PO) Periocular steroids Systemic steroids Systemic immunosuppression Anterior Uveitis
  15. 15. Intermediate Uveitis: ~15% of all uveitis Most common causes: Sarcoidosis Pars planitis syndrome (idiopathic) Multiple sclerosis Masquerade Syndromes Infection  Toxoplasma, Lyme, Toxocara, Syphilis, TB
  16. 16. Intermediate Uveitis  Vitritis +/- periphlebitis  Snowballs, snowbanking (more severe disease process)  Pars planitis: PP exudates (HLA-DR15)  ~15% of patients with pars planitis will develop MS  CME is the main vision threatening complication
  17. 17. Posterior & Panuveitis: 10-15% of all uveitis Focal choroiditis/retinitis: Toxocariasis Tuberculosis Nocardiosis Masquerade syndrome Multifocal Retinitis: Syphilis Herpes simplex virus, CMV Sarcoidosis Masquerade syndromes Candidiasis Meningococcus Multifocal Choroiditis: SO VKH Sarcoidosis Serpiginous Birdshot Wegener’s, SLE Histoplasmosis/TB Masquerade syndrome PANuveitis: Syphilis Sarcoidosis VKH Behçet’s disease Sympathetic Ophthalmia Infectious endophthalmitis
  18. 18. Posterior (Pan) Uveitis Inflammation involving retina/choroid  Optic nerve:  ON Edema, papillitis, granuloma  FA features—hot?  Retinal vasculature:  Staining, leakage, capillary dropout  Involves mainly veins vs arteries  Peripheral vs central  Chorioretinal lesions:  Dalen-fuchs nodules  Size, age of lesion (old atrophic vs new elevated with substance to it)
  19. 19. Sarcoidosis  Sarcoidosis is a multisystem granulomatous disorder  Lungs (90-95%), lymph nodes, skin, eyes, CNS  Typically affects young adults  More commonly seen in African Americans and Caucasians of Northern European descent  In US 8-10x more common in AA  AA: 35 to 82/100,000 Caucasians: 8 to 11/100,000  Etiology unknown but believed to be immune mediated:  Genetic predisposition (familial aggregation, monozygotic twins, HLA-B8, HLA-DRB1) and environmental factors (environmental allergens and infectious agents) have been suggested.  Ocular disease most common extra-pulmonary presentation  Uveitis occurs in 25-50% of pts  20-50 yrs, typically bilateral (98%)
  20. 20. Sarcoidosis: Dalen-Fuch’s Nodules
  21. 21. 30 yo AAM: Referred for endogenous candida endophthalmitis Also has recent onset of headache, mood changes, gait abnormalities Slit-like third ventricle Enlarged lateral ventricles Transependymal CSF flow Diagnosis: Biopsy-Proven Neurosarcoidosis
  22. 22. 75 yo WF with recent onset blurry vision Carried dx of SLE for >20 yrs CBC: slightly elevated WBC Neg or wnl: Lyme, RPR, FTA–ABS, PPD HLA B27 neg, UA & Chem 20 wnl Diagnostic vitrectomy: • Nests of macrophages & giant cells • Small and reactive lymphocytes • Further work-up: hilar LAD on CT and PET scan Diagnosis: Presumed Ocular Sarcoidosis
  23. 23. Behçet’s Disease  Modified Japanese Criteria:  Major criteria (skin, oral, genital, eye)  Minor criteria (arthritis, GI, epididymitis, neuropsychiatric etc)  Classification  Complete (4 major), Incomplete (3 major OR ocular disease+1 major), Suspect (2 major nonocular), Possible (1 major)  International Study Group for BD recurrent oral ulcers is a must (+2 other criteria)
  24. 24. Behçet’s retinitis
  25. 25. VKH: Common in pigmented ethnic groups •Bilateral panuveitis •Vitiligo, alopecia, poliosis, (10-60%) •Dysacusia, tinnitus (75% auditory problems) •Meningitis (80% have CSF lymphocytic pleocytosis) •ON edema & hyperemia, Serous RD •Dalen-Fuchs nodules •Sunset-glow fundus •Sigiura sign (perilimbal vitiligo) •HLA DR4 (esp Japanese), DR1
  26. 26. 24 yo Latino male with VKH: •Sudden onset blurred vision •Headache •Tinnitus & hearing loss One month after presentation Ten months after presentation
  27. 27. End-stage VKH with diffuse RPE loss and subretinal fibrosis
  28. 28.  Systemic Lupus Erythematosus  Retinopathy is an important marker of systemic activity esp CNS vasculitis-75%  Polyarteritis Nodosa (PAN)  M>F; HBs+, polyneuropathy, Raynaud’s, coronary arteritis  Untreated: 90% mortality  Wegener granulomatosis  Necrotizing granulomatosis of upper & lower resp tract -esp paranasal sinuses  Glomerulonephritis (85%), peripheral neuropathy  Untreated: 80% mortality  Behçet’s Disease Retinal Vasculitis
  29. 29. 52 yo M Acute onset of blurred vision & photophobia OS Non-granulomatous anterior uveitis OS > OD Vitritis OS > OD BRAO and retinitis OD HIV+ not on HAART RPR+ 1:2048, Syphilis IgG+ Syphilis-related panuveitis Responded to IV Penicillin x 4 wks
  30. 30.  Serpiginous choroidopathy  Relationship w/TB?  Treated with immunosuppressives  HLA-B07  >30%VA <20/200
  31. 31. APMPPE: Acute posterior multifocal placoid pigment epitheliopathy •Bitten by a lab animal •Preceding flu-like symptoms •Early hypo, late hyper on FA (White Dot Syndromes) •Hypofluorescent spots on ICG •CNS vasculitis •Benign course •20% Visual Sequelae 24 yo WM with “flashes of light” and blurry vision that developed overnight
  32. 32. Posterior/Panuveitis complications Cataract Epiretinal membrane Secondary glaucoma Hypotony Chronic cystoid macular edema Subretinal fibrosis Atrophy of retina/RPE Choroidal neovascularization Retinal ischemia Retinal neovascularization Optic nerve atrophy Retinal detachment Phthisis bulbi
  33. 33. Work-up  CBC with diff,Chem 20, UA, ESR, CRP  TB (PPD+anergy panel)+Chest X-ray  Syphilis (both RPR and Sy IgM, IgG)  HIV  Additional:  ACE, lysozyme, Ca  sarcoidosis  UA-> TINU, Wegener, SLE  ANA, anti-DNA, RF, anti-CCP, ENA panel connective tissue disorders  ANCAs (c-ANCA=PR3; p-ANCA=MPO)  Wegener, PAN  Hypercoagulability panel (ACA, LAC, Factor V Leiden mut) occlusive vasculitis  High Resolution Chest CT  TB, sarcoidosis  PFT/pulm consult  sarcoidosis  Hearing test VKH, sarcoidosis  LP MS, VKH, PIOL/CNSL  HLA panel Birdshot, HLA-B27, Behçet, MS, sarcoid  Sinus CT  Wegener’s  Lumbosacral XR/MRIHLAB27 associated uveitides  Colonoscopy  IBD, Behçet, malignancy work-up  Anterior chamber and/or vitreous tap for PCR, cultures, cytokines Despite a million dollar work-up-> 40% still idiopathic
  34. 34. Treatment: Corticosteroids have been the mainstay since 1970s Neutrophils Inhibit neutrophil migration neutrophil adherence to vascular endothelium bactericidal activity of neutrophils Local effects on the endothelium Mononuclear phagocytes Chemotaxis Clearance of antibody coated particles Production of Il-1 and TNFα Lymphocytes Redistribution of T lymphocytes(CD4 > CD8) Inhibit T lymphocyte activation proliferation and lymphokine production Inhibit Ig production by B cells (high dose)
  35. 35. Immunosuppressive Therapy Antimetabolites:  Methotrexate (anti-folate), Azathioprine (purine inhibitor), Mycophenolate Mofetil (pu) (Cellcept), Leflunomide (pyrim inh) T-cell Inhibitors:  Cyclosporine, Tacrolimus (cacineurin), Sirolimus(mtor) Alkylating agents:  Cyclophosphamide, Chlorambucil Biologics:  Anti-TNF( *infliximab, etanercept, adalimumab, golimumab, certolizumab)  Anti-IL2R (*daclizumab, basiliximab)  Anti-IL1 (anakinra)  Anti-B cell (*Rituximab, Ocralizumab) million dollar treatment  ?effect on outcome
  36. 36. Summary Diagnosis: what, where, when, who Differential: use to guide testing  Rule out etiologies that must be treated before immunosuppression (infections!) Treatment: don’t wait too long to move beyond corticosteroid treatments (Please refer!)  If not responding to treatment, consider another diagnosis Goals: Prevent complications, minimize side effects of treatment, PRESERVE VISION
  37. 37. Thank you!

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