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Uveitis Classification and external resources Hypopyon in anterior uveitis, seen as yellowish exudate in lower part of anterior chamber of eye ICD-10 H20. ICD-9 364 DiseasesDB 13676 eMedicine oph/580 emerg/284 MeSH D014605 Uveitis specifically refers to inflammation of the middle layer of the eye, termed the "uvea" but in common usage may refer to any inflammatory process involving the interior of the eye. Uveitis is estimated to be responsible for approximately 10% of the blindness in the United States.[1] Uveitis requires an urgent referral and thorough examination by an ophthalmologist along with urgent treatment to control the inflammation. Contents 1 Anatomical classification 2 Conditions associated with uveitis and uveitis syndromes 2.1 Systemic disorders associated with uveitis 2.2 Infectious causes 2.3 Uveitis Syndromes 2.4 Masquerade syndromes 3 Symptoms 4 Treatment 5 See also 6 Footnotes 7 External links Anatomical classification Uveitis may be classified anatomically into anterior, intermediate, posterior and panuveitic forms, based on which part of the eye is primarily affected by the inflammation. "Anterior uveitis" (or iridocyclitis) is the inflammation of the iris and anterior chamber. Anywhere from two-thirds to 90% of uveitis cases are anterior in location. This condition can occur as a single episode and subside with proper treatment or may take on a recurrent or chronic nature. Symptoms include red eye, injected conjunctiva, pain and decreased vision. Signs include dilated ciliary vessels, presence of cells and flare in the anterior chamber, and keratic precipitates ("KP") on the posterior surface of the cornea. "Intermediate uveitis" (pars planitis) consists of vitritis - inflammatory cells in the vitreous cavity, sometimes with snowbanking, or deposition of inflammatory material on the pars plana. "Posterior uveitis" (or chorioretinitis) is the inflammation of the retina and choroid. "Pan-uveitis" is the inflammation of all the layers of the uvea. In 2004, a group of international uveitis specialists convened in Baltimore, MD, to standardize the method of reporting data in uveitis clinical trials, including anatomical classification. The results of this meeting were published in the American Journal of Ophthalmology in 2005.[2] Conditions associated with uveitis and uveitis syndromes Myriad conditions can be associated with uveitis, including diseases with major extra-ocular involvement, as well as syndromes confined to the eye. In anterior uveitis, no associated condition or syndrome is found in approximately one-half of cases. However, anterior uveitis is often one of the syndromes associated with HLA-B27. Presence this type of HLA allele has a relative risk of evolving this disease by approximately 15%.[3] Systemic disorders associated with uveitis Systemic disorders that can be associated with uveitis include:[4] Ankylosing spondylitis Behçet's disease Chronic granulomatous disease Enthesitis Inflammatory bowel disease Juvenile rheumatoid arthritis Kawasaki's disease Multiple sclerosis Polyarteritis nodosa Psoriatic arthritis Reactive arthritis Sarcoidosis Systemic lupus erythematosus Vogt-Koyanagi-Harada syndrome Whipple's disease Lyme disease A wide range of autoimmune and autoinflammatory disease[5] Infectious causes Uveitis may be a (normal) immune response to fight an infection inside the eye. While representing the minority of patients with uveitis, such possible infections include: Brucellosis Herpes simplex Herpes zoster Leptospirosis Lyme disease Presumed ocular histoplasmosis syndrome Syphilis Toxocariasis Toxoplasmosis Tuberculosis Uveitis Syndromes In many cases, uveitis is not associated with a systemic (i.e. extraocular) condition: the inflammation is confined to the eye. In some of these cases, the presentation in the eye is characteristic of a described syndrome, and include the following diagnoses: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) Birdshot retinochoroidopathy Fuchs Heterochromic Iridocyclitis Multifocal Choroiditis and Panuveitis Syndrome Multiple Evanescent White Dot Syndrome (MEWDS) Punctate Inner Choroidopathy (PIC) Serpiginous Choroiditis Masquerade syndromes Masquerade syndromes are ophthalmic disorders that clinically present as either an anterior or posterior uveitis, but are not primarily inflammatory. The following are some of the most common: Anterior segment Intraocular foreign body Juvenile xanthogranuloma Leukemia Malignant melanoma Retinal detachment Retinoblastoma Posterior segment Lymphoma Malignant melanoma Multiple sclerosis Reticulum cell sarcoma Retinitis pigmentosa Retinoblastoma Symptoms Redness of the eye Blurred vision Sensitivity to light (photophobia) Dark, floating spots along the visual field Eye pain Treatment The prognosis is generally good for those who receive prompt diagnosis and treatment, but serious complication (including cataracts, glaucoma, band keratopathy, retinal edema and permanent vision loss) may result if left untreated. The type of uveitis, as well as its severity, duration, and responsiveness to treatment or any associated illnesses, all factor in to the outlook.[1] Uveitis is typically treated with glucocorticoid steroids, either as topical eye drops (prednisolone acetate) or oral therapy with corticosteroids. But before administration of corticosteroids, corneal ulcers are ruled out, typically by a Florescence Dye test. In addition to corticosteroids, topical cycloplegics, such as atropine or homatropine, may be used. In some cases an injection of PSTTA (posterior subtenon triamcinolone acetate) may also be given to reduce the swelling of the eye. [6] Antimetabolite medications, such as methotrexate are often used for recalcitrant or more aggressive cases of uveitis. Experimental treatments with Infliximab or other anti-TNFs' infusions may prove helpful. See also List of eye diseases and disorders List of systemic diseases with ocular manifestations Intermediate uveitis Footnotes ^ Sergio Schwartzman. Inflammatory eye disease: an expert interview with Sergio Schwartzman,MD Medscape Rheumatology 2007. ^ Jabs DA, Nussenblatt RB, Rosenbaum JT. Standardization of Uveitis Nomenclature (SUN) Working Group. Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol 2005;140:509-516. ^ Table 5-7 in: Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson. Robbins Basic Pathology. Philadelphia: Saunders. ISBN 1-4160-2973-7.  8th edition. ^ White G. "Uveitis." Retrieved August 20, 2006. ^ McGonagle D, McDermott MF (2006) A proposed classifi cation of the immunological diseases. PLoS Med 3(8): e297. DOI: 10.1371/journal. pmed.0030297 ^ BNF 45 March 2003 External links Uveitis Information Group, UK based information site and support group Uveitis at the Open Directory Project The Ocular Immunology and Uveitis Foundation v · d · eEye disease · pathology of the eye (H00–H59, 360–379) Adnexa eyelid: inflammation (Stye, Chalazion, Blepharitis) · Entropion · Ectropion · Lagophthalmos · Blepharochalasis · Ptosis · Blepharophimosis · Xanthelasma · eyelash (Trichiasis, Madarosis) lacrimal system: Dacryoadenitis · Epiphora · Dacryocystitis · Xerophthalmia orbit: Exophthalmos · Enophthalmos · Orbital cellulitis · Periorbital cellulitis conjunctiva: Conjunctivitis (Allergic conjunctivitis) · Pterygium · Pinguecula · Subconjunctival hemorrhage Globe Fibrous tunic sclera: Scleritis cornea: Keratitis (Herpetic keratitis, Acanthamoeba keratitis, Fungal keratitis) · Corneal ulcer · Photokeratitis · Thygeson's superficial punctate keratopathy · Corneal dystrophy (Fuchs', Meesmann) · Keratoconus · Keratoconjunctivitis sicca · Keratoconjunctivitis · Corneal neovascularization · Kayser-Fleischer ring · Arcus senilis · Band keratopathy Vascular tunic Iris and ciliary body Iritis · Uveitis (Intermediate uveitis) · Iridocyclitis · Hyphema · Rubeosis iridis · Persistent pupillary membrane · Iridodialysis · Synechia Choroid Choroideremia · Choroiditis (Chorioretinitis) Lens Cataract · Aphakia · Ectopia lentis Retina Retinitis (Chorioretinitis, Cytomegalovirus retinitis) · Retinal detachment · Retinoschisis · Ocular ischemic syndrome/Central retinal vein occlusion · Retinopathy (Bietti's crystalline dystrophy, Coats disease, Diabetic retinopathy, Hypertensive retinopathy, Retinopathy of prematurity) · Macular degeneration · Retinitis pigmentosa · Retinal haemorrhage · Central serous retinopathy · Macular edema · Epiretinal membrane · Macular pucker · Vitelliform macular dystrophy · Leber's congenital amaurosis · Birdshot chorioretinopathy Other Glaucoma/Ocular hypertension · Floater · Leber's hereditary optic neuropathy · Red eye · Keratomycosis · Phthisis bulbi Pathways Optic nerve and optic disc Optic neuritis · Papilledema (Foster Kennedy syndrome) · Optic atrophy/Optic neuropathy (Leber's hereditary optic neuropathy, Kjer's optic neuropathy) · Optic disc drusen · Toxic and nutritional optic neuropathy · Ischemic optic neuropathy (AION, PION) Ocular muscles, binocular movement, accommodation Paralytic strabismus Ophthalmoparesis · Progressive external ophthalmoplegia · Palsy (III, IV, VI) · Kearns-Sayre syndrome Other strabismus Esotropia/Exotropia · Hypertropia · Heterophoria (Esophoria, Exophoria) · Brown's syndrome · Duane syndrome Other binocular Conjugate gaze palsy · Convergence insufficiency · Internuclear ophthalmoplegia · One and a half syndrome Refraction Refractive error: Hyperopia/Myopia · Astigmatism · Anisometropia/Aniseikonia · Presbyopia Visual disturbances and blindness Amblyopia · Leber's congenital amaurosis · Subjective (Asthenopia, Hemeralopia, Photophobia, Scintillating scotoma) · Diplopia · Scotoma · Anopsia (Binasal hemianopsia, Bitemporal hemianopsia, Homonymous hemianopsia, Quadrantanopia) · Color blindness (Achromatopsia, Dichromacy, Monochromacy) · Nyctalopia (Oguchi disease) · Blindness/Low vision Pupil Anisocoria · Argyll Robertson pupil · Marcus Gunn pupil · Adie syndrome · Miosis · Mydriasis · Cycloplegia · Parinaud's syndrome Other Nystagmus Eye infections Trachoma · Onchocerciasis M: EYE anat(g/a/p)/phys/devp/prot noco/cong/tumr, epon proc, drug(S1A/1E/1F/1L) v · d · eInflammation Acute Plasma derived mediators Bradykinin · complement (C3, C5a, MAC) · coagulation (Factor XII, Plasmin, Thrombin) Cell derived mediators preformed: Lysosome granules · vasoactive amines (Histamine, Serotonin) synthesized on demand: cytokines (IFN-γ, IL-8, TNF-α, IL-1) · eicosanoids (Leukotriene B4, Prostaglandins) · Nitric oxide · Kinins Chronic Macrophage · Epithelioid cell · Giant cell · Granuloma Processes Traditional: Rubor · Calor · Tumor · Dolor (pain) · Functio laesa Modern: Acute-phase reaction/Fever · Vasodilation · Increased vascular permeability · Exudate · Leukocyte extravasation · Chemotaxis Specific types Nervous CNS (Encephalitis, Myelitis) · Meningitis (Arachnoiditis) · PNS (Neuritis) · eye (Dacryoadenitis, Scleritis, Keratitis, Choroiditis, Retinitis, Chorioretinitis, Blepharitis, Conjunctivitis, Iritis, Uveitis) · ear (Otitis, Labyrinthitis, Mastoiditis) Cardiovascular Carditis (Endocarditis, Myocarditis, Pericarditis) · Vasculitis (Arteritis, Phlebitis, Capillaritis) Respiratory upper (Sinusitis, Rhinitis, Pharyngitis, Laryngitis) · lower (Tracheitis, Bronchitis, Bronchiolitis, Pneumonitis, Pleuritis) · Mediastinitis Digestive mouth (Stomatitis, Gingivitis, Gingivostomatitis, Glossitis, Tonsillitis, Sialadenitis/Parotitis, Cheilitis, Pulpitis, Gnathitis) · tract (Esophagitis, Gastritis, Gastroenteritis, Enteritis, Colitis, Enterocolitis, Duodenitis, Ileitis, Caecitis, Appendicitis, Proctitis) · accessory (Hepatitis, Cholangitis, Cholecystitis, Pancreatitis) · Peritonitis Integumentary Dermatitis (Folliculitis) · Cellulitis · Hidradenitis Musculoskeletal Arthritis · Dermatomyositis · soft tissue (Myositis, Synovitis/Tenosynovitis, Bursitis, Enthesitis, Fasciitis, Capsulitis, Epicondylitis, Tendinitis, Panniculitis) Osteochondritis: Osteitis (Spondylitis, Periostitis) · Chondritis Urinary Nephritis (Glomerulonephritis, Pyelonephritis) · Ureteritis · Cystitis · Urethritis Reproductive female: Oophoritis · Salpingitis · Endometritis · Parametritis · Cervicitis · Vaginitis · Vulvitis · Mastitis male: Orchitis · Epididymitis · Prostatitis · Balanitis · Balanoposthitis pregnancy/newborn: Chorioamnionitis · Omphalitis Endocrine Insulitis · Hypophysitis · Thyroiditis · Parathyroiditis · Adrenalitis Lymphatic Lymphangitis · Lymphadenitis