Uveitis
Encyclopedia
Uveitis specifically refers to inflammation
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

 of the middle layer of the eye
Human eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...

, termed the "uvea
Uvea
The uvea , also called the uveal layer, uveal coat, uveal tract, or vascular tunic, is the pigmented middle of the three concentric layers that make up an eye. The name is possibly a reference to its reddish-blue or almost black colour, wrinkled appearance and grape-like size and shape when...

" 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. Uveitis requires an urgent referral and thorough examination by an optometrist or ophthalmologist along with urgent treatment to control the inflammation.

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
    Iridocyclitis
    Iridocyclitis, a type of anterior uveitis, is a condition in which the uvea of the eye is inflamed.Iridocyclitis isInflammation of the iris and the ciliary body.- Symptoms :Symptoms include:* Photophobia* Redness* Watering of the eyes* Lacrimation...

    ) is the inflammation of the iris
    Iris (anatomy)
    The iris is a thin, circular structure in the eye, responsible for controlling the diameter and size of the pupils and thus the amount of light reaching the retina. "Eye color" is the color of the iris, which can be green, blue, or brown. In some cases it can be hazel , grey, violet, or even pink...

     and anterior chamber
    Anterior chamber
    The anterior chamber is the fluid-filled space inside the eye between the iris and the cornea's innermost surface, the endothelium. Aqueous humor is the fluid that fills the anterior chamber. Hyphema and glaucoma are two main pathologies in this area. In hyphema, blood fills the 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
    Red eye (medicine)
    In medicine, red eye is a non-specific term to describe an eye that appears red due to illness, injury, or some other condition. Conjunctivitis and subconjunctival hemorrhage are two forms of a red eye....

    , injected conjunctiva
    Conjunctiva
    The conjunctiva covers the sclera and lines the inside of the eyelids. It is composed of rare stratified columnar epithelium.-Function:...

    , pain and decreased vision. Signs include dilated ciliary vessel
    Ciliary body
    The ciliary body is the circumferential tissue inside the eye composed of the ciliary muscle and ciliary processes. It is triangular in horizontal section and is coated by a double layer, the ciliary epithelium. This epithelium produces the aqueous humor. The inner layer is transparent and covers...

    s, presence of cells and flare in the anterior chamber, and keratic precipitate
    Keratic precipitate
    Keratic precipitate is inflammatory cellular deposit seen on corneal endothelium. Acute KPs is white and round in shape whereas old KPs r faded and irregular in shape. Mutton-fat KPs are large in shape and is greasy-white in color and is formed from macrophages and epithelioid cells....

    s ("KP") on the posterior surface of the cornea
    Cornea
    The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Together with the lens, the cornea refracts light, with the cornea accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is...

    .
  • "Intermediate uveitis
    Intermediate uveitis
    Intermediate uveitis refers to inflammation localized to the vitreous and peripheral retina. Primary sites of inflammation include the vitreous of which other such entities as pars planitis, posterior cyclitis, and hyalitis are encompassed...

    " (pars planitis) consists of vitritis - inflammatory cells in the vitreous cavity
    Vitreous humour
    The vitreous humour or vitreous humor is the clear gel that fills the space between the lens and the retina of the eyeball of humans and other vertebrates...

    , sometimes with snowbanking, or deposition of inflammatory material on the pars plana
    Pars plana
    The pars plana is part of the uvea, choroidea, one of the three layers that comprise the eye. As a part of the ciliary body, it is about 4 mm long and located near the point where the iris and sclera touch....

    .
  • "Posterior uveitis" (or chorioretinitis
    Chorioretinitis
    Chorioretinitis is an inflammation of the choroid and retina of the eye. It is also known as choroid retinitis.-Causes:...

    ) is the inflammation of the retina
    Retina
    The vertebrate retina is a light-sensitive tissue lining the inner surface of the eye. The optics of the eye create an image of the visual world on the retina, which serves much the same function as the film in a camera. Light striking the retina initiates a cascade of chemical and electrical...

     and choroid
    Choroid
    The choroid, also known as the choroidea or choroid coat, is the vascular layer of the eye, containing connective tissue, and lying between the retina and the sclera. The human choroid is thickest at the far extreme rear of the eye , while in the outlying areas it narrows to 0.1 mm...

    .
  • "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.

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
HLA-B27
Human Leukocyte Antigen B27 is a class I surface antigen encoded by the B locus in the major histocompatibility complex on chromosome 6 and presents antigenic peptides to T cells...

. Presence this type of HLA
Human leukocyte antigen
The human leukocyte antigen system is the name of the major histocompatibility complex in humans. The super locus contains a large number of genes related to immune system function in humans. This group of genes resides on chromosome 6, and encodes cell-surface antigen-presenting proteins and...

 allele has a relative risk
Relative risk
In statistics and mathematical epidemiology, relative risk is the risk of an event relative to exposure. Relative risk is a ratio of the probability of the event occurring in the exposed group versus a non-exposed group....

 of evolving this disease by approximately 15%.

Systemic disorders associated with uveitis

Systemic disorders that can be associated with uveitis include:
  • Ankylosing spondylitis
    Ankylosing spondylitis
    Ankylosing spondylitis , previously known as Bekhterev's disease, Bekhterev syndrome, and Marie-Strümpell disease is a chronic inflammatory disease of the axial skeleton with variable involvement of peripheral joints and nonarticular structures...

  • Behçet's disease
    Behçet's disease
    Behçet's disease is a rare immune-mediated systemic vasculitis that often presents with mucous membrane ulceration and ocular involvements...

  • Chronic granulomatous disease
    Chronic granulomatous disease
    Chronic granulomatous disease is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds used to kill certain ingested pathogens...

  • Enthesitis
    Enthesitis
    Enthesitis is inflammation of the entheses, the sites where tendons or ligaments insert into the bone. It is also called enthesopathy, or any pathologic condition involving the entheses. The entheses are any point of attachment of skeletal muscles to the bone, where recurring stress or...

  • Inflammatory bowel disease
    Inflammatory bowel disease
    In medicine, inflammatory bowel disease is a group of inflammatory conditions of the colon and small intestine. The major types of IBD are Crohn's disease and ulcerative colitis.-Classification:...

  • Juvenile rheumatoid arthritis
  • Kawasaki's disease
  • Multiple sclerosis
    Multiple sclerosis
    Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...

  • Polyarteritis nodosa
    Polyarteritis nodosa
    Polyarteritis nodosa is a vasculitis of medium & small-sized arteries, which become swollen and damaged from attack by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease...

  • Psoriatic arthritis
    Psoriatic arthritis
    Psoriatic arthritis is a type of inflammatory arthritis that, according to the National Psoriasis Foundation, will develop in up to 30 percent of people who have the chronic skin condition psoriasis...

  • Reactive arthritis
    Reactive arthritis
    Reactive arthritis , is classified as an autoimmune condition that develops in response to an infection in another part of the body. Coming into contact with bacteria and developing an infection can trigger the disease. Reiter's syndrome has symptoms similar to various other conditions collectively...

  • Sarcoidosis
    Sarcoidosis
    Sarcoidosis , also called sarcoid, Besnier-Boeck disease or Besnier-Boeck-Schaumann disease, is a disease in which abnormal collections of chronic inflammatory cells form as nodules in multiple organs. The cause of sarcoidosis is unknown...

  • Systemic lupus erythematosus
    Systemic lupus erythematosus
    Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...

  • Vogt-Koyanagi-Harada syndrome
    Vogt-Koyanagi-Harada syndrome
    Vogt–Koyanagi–Harada syndrome is a condition seen in humans and dogs involving various melanocyte-containing organs, characterized by uveitis , poliosis , vitiligo , and meningitis, although dogs with this syndrome rarely develop meningitis...

  • Whipple's disease
    Whipple's disease
    Whipple's disease is a rare, systemic infectious disease caused by the bacterium Tropheryma whipplei. First described by George Hoyt Whipple in 1907 and commonly considered a gastrointestinal disorder, Whipple's disease primarily causes malabsorption but may affect any part of the body including...

  • Lyme disease
    Lyme disease
    Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...

  • HLA-B27
    HLA-B27
    Human Leukocyte Antigen B27 is a class I surface antigen encoded by the B locus in the major histocompatibility complex on chromosome 6 and presents antigenic peptides to T cells...

  • A wide range of autoimmune and autoinflammatory disease

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
    Brucellosis
    Brucellosis, also called Bang's disease, Crimean fever, Gibraltar fever, Malta fever, Maltese fever, Mediterranean fever, rock fever, or undulant fever, is a highly contagious zoonosis caused by ingestion of unsterilized milk or meat from infected animals or close contact with their secretions...

  • Herpes simplex
    Herpes simplex
    Herpes simplex is a viral disease caused by both Herpes simplex virus type 1 and type 2 . Infection with the herpes virus is categorized into one of several distinct disorders based on the site of infection. Oral herpes, the visible symptoms of which are colloquially called cold sores or fever...

  • Herpes zoster
    Herpes zoster
    Herpes zoster , commonly known as shingles and also known as zona, is a viral disease characterized by a painful skin rash with blisters in a limited area on one side of the body, often in a stripe...

  • Leptospirosis
    Leptospirosis
    Leptospirosis is caused by infection with bacteria of the genus Leptospira, and affects humans as well as other mammals, birds, amphibians, and reptiles.The...

  • Lyme disease
    Lyme disease
    Lyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...

  • Presumed ocular histoplasmosis syndrome
    Presumed ocular histoplasmosis syndrome
    Presumed ocular histoplasmosis syndrome is a disease syndrome affecting the eye that is characterized by peripheral atrophic chorioretinal scars, atrophy or scarring adjacent to the optic disc and maculopathy....

  • Syphilis
    Syphilis
    Syphilis is a sexually transmitted infection caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The primary route of transmission is through sexual contact; however, it may also be transmitted from mother to fetus during pregnancy or at birth, resulting in congenital syphilis...

  • Toxocariasis
    Toxocariasis
    -History of discovery:Werner described a parasitic nematode in dogs in 1782 which he named Ascaris canis. Johnston determined that what Werner had described was actually a member of the genus Toxocara established by Stiles in 1905. Fữlleborn speculated that T canis larvae might cause granulomatous...

  • Toxoplasmosis
    Toxoplasmosis
    Toxoplasmosis is a parasitic disease caused by the protozoan Toxoplasma gondii. The parasite infects most genera of warm-blooded animals, including humans, but the primary host is the felid family. Animals are infected by eating infected meat, by ingestion of feces of a cat that has itself...

  • Tuberculosis
    Tuberculosis
    Tuberculosis, MTB, or TB is a common, and in many cases lethal, infectious disease caused by various strains of mycobacteria, usually Mycobacterium tuberculosis. Tuberculosis usually attacks the lungs but can also affect other parts of the body...


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
    Birdshot retinochoroidopathy
    Birdshot chorioretinopathy, also called birdshot retinochoroidopathy, is a rare form of bilateral posterior uveitis affecting the eye. It causes severe, progressive inflammation of both the choroid and retina....

  • 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
    Foreign body
    A foreign body is any object originating outside the body. In machinery, it can mean any unwanted intruding object.Most references to foreign bodies involve propulsion through natural orifices into hollow organs....

  • Juvenile xanthogranuloma
    Juvenile xanthogranuloma
    Juvenile xanthogranuloma is a form of histiocytosis, classified as "non-Langerhans cell histiocytosis", or more specifically, "type 2"....

  • Leukemia
    Leukemia
    Leukemia or leukaemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts". Leukemia is a broad term covering a spectrum of diseases...

  • Malignant melanoma
  • Retinal detachment
    Retinal detachment
    Retinal detachment is a disorder of the eye in which the retina peels away from its underlying layer of support tissue. Initial detachment may be localized, but without rapid treatment the entire retina may detach, leading to vision loss and blindness. It is a medical emergency.The retina is a...

  • Retinoblastoma
    Retinoblastoma
    Retinoblastoma is a rapidly developing cancer that develops in the cells of retina, the light-detecting tissue of the eye. In the developed world, Rb has one of the best cure rates of all childhood cancers , with more than nine out of every ten sufferers surviving into...

  • Posterior segment
  • Lymphoma
    Lymphoma
    Lymphoma is a cancer in the lymphatic cells of the immune system. Typically, lymphomas present as a solid tumor of lymphoid cells. Treatment might involve chemotherapy and in some cases radiotherapy and/or bone marrow transplantation, and can be curable depending on the histology, type, and stage...

  • Malignant melanoma
  • Multiple sclerosis
    Multiple sclerosis
    Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...

  • Reticulum cell sarcoma
  • Retinitis pigmentosa
    Retinitis pigmentosa
    Retinitis pigmentosa is a group of genetic eye conditions that leads to incurable blindness. In the progression of symptoms for RP, night blindness generally precedes tunnel vision by years or even decades. Many people with RP do not become legally blind until their 40s or 50s and retain some...

  • Retinoblastoma
    Retinoblastoma
    Retinoblastoma is a rapidly developing cancer that develops in the cells of retina, the light-detecting tissue of the eye. In the developed world, Rb has one of the best cure rates of all childhood cancers , with more than nine out of every ten sufferers surviving into...


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
Glaucoma
Glaucoma is an eye disorder in which the optic nerve suffers damage, permanently damaging vision in the affected eye and progressing to complete blindness if untreated. It is often, but not always, associated with increased pressure of the fluid in the eye...

, band keratopathy
Band keratopathy
Band keratopathy is a corneal disease derived from the appearance of calcium on the central cornea. This is an example of metastatic calcification, which by definition, occurs in the presence of hypercalcemia...

, 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.http://www.intelihealth.com/IH/ihtIH/EMIHC000/9339/10942.html

Uveitis is typically treated with glucocorticoid
Glucocorticoid
Glucocorticoids are a class of steroid hormones that bind to the glucocorticoid receptor , which is present in almost every vertebrate animal cell...

 steroid
Steroid
A steroid is a type of organic compound that contains a characteristic arrangement of four cycloalkane rings that are joined to each other. Examples of steroids include the dietary fat cholesterol, the sex hormones estradiol and testosterone, and the anti-inflammatory drug dexamethasone.The core...

s, 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
Atropine
Atropine is a naturally occurring tropane alkaloid extracted from deadly nightshade , Jimson weed , mandrake and other plants of the family Solanaceae. It is a secondary metabolite of these plants and serves as a drug with a wide variety of effects...

 or homatropine
Homatropine
Homatropine is an anticholinergic medication that inhibits muscarinic acetylcholine receptors and thus the parasympathetic nervous system...

, may be used.
In some cases an injection of PSTTA (posterior subtenon triamcinolone
Triamcinolone
Triamcinolone is a long-acting synthetic corticosteroid given orally, by injection, inhalation, or as a topical ointment or cream....

 acetate) may also be given to reduce the swelling of the eye.
Antimetabolite
Antimetabolite
An antimetabolite is a chemical that inhibits the use of a metabolite, which is another chemical that is part of normal metabolism. Such substances are often similar in structure to the metabolite that they interfere with, such as the antifolates that interfere with the use of folic acid...

 medications, such as methotrexate
Methotrexate
Methotrexate , abbreviated MTX and formerly known as amethopterin, is an antimetabolite and antifolate drug. It is used in treatment of cancer, autoimmune diseases, ectopic pregnancy, and for the induction of medical abortions. It acts by inhibiting the metabolism of folic acid. Methotrexate...

 are often used for recalcitrant or more aggressive cases of uveitis. Experimental treatments with Infliximab
Infliximab
Infliximab is a monoclonal antibody against tumour necrosis factor alpha . It is used to treat autoimmune diseases. Remicade is marketed by Janssen Biotech, Inc...

 or other anti-TNFs' infusions may prove helpful.

See also


External links

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