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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