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UVEITIS 成大眼科 楊勝吉 Definition  An inflammation of the uveal tract  Uveal tract: iris, ciliary body, choroid  Intraocular inflammation, not only theuvea, but also adjacent structures Classification Aetiological: exogenous, endogenous Pathological: granulomatous, nongranulomatous Medical history Systemic Evaluation  Skin  Joints  Lung  Neurologic  GI  GU  etc Symptoms and Signs Symptoms  Redness  Pain  Photophobia Signs  Injection  Keratic precipitate (KP)  Iris nodule  Koeppe, Busacca  Lacrimation  Cells (AC or Vitreous)  Floaters  Flares  Decreased vision  Posterior synechiae (PS)  Choroiditis  Retinitis  Vasculitis Fig. 11.2 Signs of acute anterior uveitis. (A) Ciliary injection; (B) miosis; (C) endothelial dusting by cells; (D) aqueous flare and cells; (E) fibrinous exudate; (F) hypopyon Posterior synechia Keratic precipitates. (A) Aggregate of inflammatory cells on the corneal endothelium; (B) large ‘mutton-fat’ keratic precipitates; (C) ‘ghost’ keratic precipitates; (D) old pigmented keratic precipitates Signs of posterior uveitis. (A) Retinitis; (B) Choroiditis; (C) vasculitis Iris nodules in granulomatous anterior uveitis. (A) Koeppe nodules; (B) Busacca nodules Cells & Flare Cells: active inflammation Flare: leakage of protein Special investigations  Indications  Granulomatous inflammation.  Recurrent uveitis.  Bilateral disease.  Systemic manifestations without a specific diagnosis.  Confirmation of a suspected ocular picture which depends on the test result as part of the criteria for diagnosis such as HLA-A29 testing for birdshot chorioretinopathy. Skin test (A) Positive tuberculin skin reaction; (B) strongly positive tuberculin skin reaction; (C) positive pathergy test in Behçet syndrome Other tests  Imaging:  Fluorescein angiography (FA), Indocyanine green angiography (ICGA), Optical coherence tomography (OCT)  Radiology  Chest X-ray, Sacroiliac joint X-ray, CT and MR  Biopsy  HLA type Treatment  Mydriatics  Steroids  topical, periocular injection, systemic  Calcineurin inhibitors:  Cyclosporin, Tacrolimus  Antimetabolites:  Azathioprine, MTX, Anterior uveitis  Acute  HLA-B27 related diseases  Behcet syndrome  Glaucomatocyclitic crisis  Lens-associated uveitis HLA-B27 Related Diseases  Seronegative spondyloarthropathies  Ankylosing spondylitis  Reiter syndrome  Inflammatory bowel disease  (enteropathic arthritis)  Psoriatic arthritis Ankylosing Spondylitis  Acute iritis, Sacroiliitis and spondylitis  HLA-B27  General population: 5%  Acute iritis: 45%  AS: 90%  Both AS & acute iritis: 95% c. Circinate balanitis d. Keratoderma blennorrhagicum Reiter Syndrome  Young adult male  Classic triad  Urethritis  Polyarthritis  Conjunctivitis (the most common) or acute iritis Behcet Syndrome (1)  Generalized occlusive vasculitis  Posterior involvement > Anterior  retinal vasculitis, hemorrhage, necrosis, macular edema, ischemic optic neuropathy (A) Major aphthous ulceration; (B) genital ulceration; (C) superficial thrombophlebitis; (D) dermatographia Behcet Syndrome (2)  Skin lesion: erythema nodosum  HLA-B51  Young adults  Recurrence  Poor prognosis Glaucomatocyclitic Crisis (Posner- Schlossman Syndrome)  Unilateral mild iritis: (low grade cells, flare)  Markedly elevated IOP  Recurrence are common  A diagnosis of exclusion Lens-Associated Uveitis  Phacoantigenic glaucoma  Lens capsule injury (immune response to lens protein)  Mutton-fat KP, dense cells & flare, PS  Phacolytic glaucoma  Intact capsule, hypermature cataract  Acute increase of IOP (clogging of TM by lens protein & engorged macrophages)  Lack of KP, PS Chronic Iridocyclitis  Juvenile rheumatoid arthritis  Most common systemic disorder associated with iridocyclitis in the pediatric  Female/Male: 3/2  Risk factors for iridocyclitis:  female, pauciarticular onset, ANA (+)  Often white & uninflamed in appearance  Complications:  cataract (84%), band keratopathy (70%), macular  edema, vitreous debris, glaucoma, phthisis Intermediate Uveitis  Anterior vitreous cells:  snowballs, snowbanking  Retinal phlebitis  Associated with sarcoidosis, multiple sclerosis, Lyme disease, toxocariasis, syphilis, tuberculosis, connective tissue disease Pars Planitis  Most common form of intermediate uveitis  < 40 y/o  Bilateral (80%)  Vitreous condensation, cellular  infiltration in vitreous base  Fluorescein angiography: diffuse peripheral venular leaking  Major cause of visual loss: cystoid macular edema Posterior segment signs in intermediate uveitis. (A) Peripheral periphlebitis and a few snowballs inferiorly; (B) inferior snowbanking and snowballs; (C) severe snowbanking, neovascularization and inferior retinal detachment Posterior Uveitis  Infectious  Viral: Herpes, CMV, Rubella, Measles, HIV…..  Fungal: Histoplasmosis, Candidiasis  Protozoal: Toxoplasmosis  Helminthic: Toxocariasis, Cysticercosis  Immunologic  Collagen vascular dz: SLE, PAN, Wegener granulomatosis  Retinochoroidopathies  Masquerade conditions  Endophthalmitis: Nocadia asteroids Cytomegalovirus retinitis Active toxoplasma retinitis. Toxocara granuloma Acute retinal necrosis (ARN)  Classic triad  Vitritis  Occlusive retinal arteriolitis & periphlebitis  Multifocal yellow-white peripheral retinitis  Pts: typically healthy & not debilitated  Varicella-zoster, herpes simplex, CMV  Posterior pole tends to be spared  Multiple posterior retinal breaks & traction/RRD  Ischemic optic neuropathy  Poor prognosis Ocular Complications in AIDS  Up to 70% of individuals with AIDS  May be the first sign of disseminated systemic inf.  Microangiopathy of retina  Various opportunistic infections  Kaposi sarcoma  Lymphoma  Squamous cell carcinoma of conjunctiva  HIV retinopathy: most common  Retinal hemorrhage, microaneurysm, cotton-wool spots  CMV retinitis: most common opportunistic ocular inf. Panuveitis  Infectious  Bacterial: Syphilis, Tuberculosis, Lyme dz,  Helminthic: Onchocerciasis  Immunologic & granulomatous  Sarcoidosis  Sympathetic opthalmia  Vogt-Koyanagi-Harada (VKH) syndrome  Masquerade syndrome  Neoplasms Sympathetic Ophthalmia  Bilateral granulomatous panuveitis occurring after     penetrating trauma The traumatized :exciting eye and the fellow eye: sympathizing eye Diffuse and massive lymphocytic infiltration of the choroid 65% of cases is between 2 weeks and 3 months after initial injury and 90% of all cases occur within the first year. Treatment:  Enucleation  Topical treatment of the anterior uveitis with steroids and cycloplegics resistant to this form of therapy (diagnostic clue)  Systemic steroids, immunomodulatory therapy Sympathetic Ophthalmia