II. Defintions

  1. Uvea
    1. Contains three pigmented and continuous structures: Choroid, Ciliary Body and Iris
  2. Uveitis
    1. Uveal tract inflammation
    2. Defined as chronic if duration more than 3 months

III. Epidemiology

  1. Incidence: 17 to 52 per 100,000 per year in North America
  2. Prevalence: 58 to 115 per 100,000 per year in North America
  3. Peak ages: 20-59 years old

IV. Pathophysiology

  1. Autoimmune reaction (similar to Episcleritis and Scleritis)
  2. Uveitis may occur in isolation or as part of a systemic inflammatory condition (see causes below)
  3. Diagnosis is as a result of spillover inflammation into the aqueous and vitreous resulting in cells and flare

V. Types

  1. Anterior Uveitis (Iritis or Iridocyclitis)
    1. Inflammation of iris of the uveal tract (and the ciliary Muscle) in the anterior chamber
    2. When the ciliary Muscle/body is involved it is known as Iridocyclitis
    3. May occur from spillover inflammation from the Cornea (keratouveitis) or Sclera (sclerouveitis)
  2. Intermediate Uveitis
    1. Inflammation of the vitreous chamber (vitritis)
  3. Posterior Uveitis
    1. Inflammation of the Retina (retinitis) or Choroid (Choroiditis or chorioretinitis)
    2. May extend to the Optic Nerve head (Neuroretinitis)
  4. Panuveitis
    1. Generalized ocular inflammation

VI. Causes

  1. See Medication Causes of Uveitis
  2. Traumatic Iritis
    1. Blunt Eye Trauma causes intraocular inflammation and blood barrier breakdown
    2. Onset may be delayed 2-3 days after injury
    3. Traumatic Iritis duration is typically 7-10 days
  3. Bacterial Infection
    1. See Endophthalmitis under differential diagnosis below
    2. Bloodbourne infection
      1. Involves any uveal location
    3. Cat-Scratch Disease
      1. Involves Posterior Uveitis, specifically Neuroretinitis
    4. Lyme Disease
      1. Involves any uveal location
    5. Mycobacterium tuberculosis
      1. Involves any uveal location
      2. Affects <1% of Tb cases
    6. Syphilis
      1. Involves any uveal location
      2. Affects <5% of Syphilis cases
    7. Toxocariasis
      1. Involves posterior uveal location (Posterior Uveitis)
    8. Leprosy
  4. Other infectious causes
    1. Local infection from perforating wound or Corneal Ulcer
    2. Viral Exanthem
    3. HIV Infection
  5. Rheumatologic Conditions
    1. Rheumatoid Arthritis
      1. Occurs in up to 50% of pauciarticular Juvenile Rheumatoid Arthritis
        1. Accounts for 80% of Uveitis cases in children
      2. Occurs in <5% of adult Rheumatoid Arthritis
        1. Typically a result of Scleritis or Keratitis spill-over
    2. Spondyloarthropathy (often seronegative, affects Anterior Uveitis)
      1. Ankylosing Spondylitis
      2. Reiter's Syndrome (Reactive Arthritis)
      3. Psoriatic Arthritis
    3. Behcet's Disease
      1. May affect any uveal location
      2. Occurs in >60% of cases
    4. Relapsing Polychondritis
      1. Involves anterior chamber (Anterior Uveitis)
    5. Sarcoidosis
      1. Involves any uveal location
    6. Systemic Lupus Erythematosus (SLE)
      1. Involves any uveal location
      2. Affects <1% of SLE cases
    7. Vogt-Koyanagi-Harada Syndrome
      1. Involves Posterior Uveitis
      2. Uveitis occurs in 100% of cases
    8. Kawasaki's Disease
  6. Inflammatory Bowel Disease (Anterior Uveitis, affecting >15% of IBD cases)
    1. Ulcerative Colitis
    2. Crohn's Disease
  7. Neurologic disorders
    1. Multiple Sclerosis (Intermediate Uveitis, affecting 30% of MS cases)
  8. Renal disorders
    1. Tubular Interstitial Nephritis
      1. Involves anterior chamber (Anterior Uveitis)

VII. Symptoms

  1. Eye Pain
    1. Moderate deep aching Sensation
    2. May radiate into periocular structures (e.g. brow pain or temporal pain)
    3. Eye Pain is often absent in Intermediate Uveitis and Posterior Uveitis
    4. Not relieved with Topical Anesthetic (e.g. tetracaine)
  2. Photophobia
  3. Vision decreased or blurred
  4. Visual Floaters
  5. Eye tearing

VIII. Signs

  1. Pupil constricted and poorly reactive (due to iris spasm)
    1. Other acute eye conditions typically cause Mydriasis
    2. Pain on direct light exposure
    3. Pain also occurs on Consensual Light Reflex when light is shone into opposite eye
  2. Conjunctival injection
    1. Present in most cases of Anterior Uveitis
    2. Injection is localized around the iris, in contrast with Conjunctivitis in which the the injection is more widespread
  3. Ciliary Flush (or blush)
    1. Injection of bulbar Conjunctiva around limbus (circumcorneal hyperemia)
  4. Anterior flare
    1. Flare is an increased turbidness or cloudiness of the Aqueous Humor
  5. Anterior chamber "cells" slough
    1. Anterior cells seen on Slit Lamp exam
    2. Often in combination with flare (known as "cells and flare")
  6. Corneal Keratic Precipitates
    1. Yellow or white deposits of aggregated cells on the posterior surface of the Cornea (endothelial surface)
    2. Results from the anterior chamber cells
  7. Sterile Hypopyon
    1. White Blood Cells in severe inflammatory response may settle and accumulate at the anterior chamber floor
  8. Irregular pupil shape
    1. Iris may adhere to adjacent structures (anterior lens or posterior Cornea)
  9. Intraocular Pressure abnormal
    1. May be either increased or decreased

IX. Labs

  1. First-line labs to consider if systemic cause is suspected
    1. Complete Blood Count
    2. Basic metabolic panel
    3. Urinalysis
    4. Erythrocyte Sedimentation Rate (ESR)
    5. C-Reactive Protein (CRP)
  2. Other tests to consider
    1. Rapid Plasma Reagin (RPR)
    2. Tuberculin Skin Test or Quantiferon-TB
    3. HLA-B27 (if Spondyloarthropathy suspected)
    4. Other rheumatologic of infectious disease tests as indicated based on history and exam

X. Imaging

  1. Chest XRay
    1. Consider if Sarcoidosis, Tuberculosis suspected

XI. Differential Diagnosis

  1. See Acute Red Eye
  2. Acute Glaucoma
  3. Primary ocular Lymphoma
    1. Consider in age over 50 years old with intermediate or Posterior Uveitis
  4. Endophthalmitis
    1. Bloodborne infection with intraocular infection
    2. Patients typically present appearing toxic or septic
    3. Some cases may develop more insidiously
      1. KlebsiellaPneumoniae in diabetic patients
      2. Systemic Fungal infections
      3. Less virulent Bacterial Infections (e.g. Central Line infection, IV Drug Abuse)

XII. Complications

  1. Decreased Visual Acuity
    1. Severe Visual Impairment: 35%
    2. Legally blind: 10%
  2. Iris scarring
    1. Impaired pupillary movement
    2. Secondary Glaucoma

XIII. Precautions

  1. Children be asymptomatic with minimal exam findings despite significant inflammation (e.g. juvenile RA)
  2. Intermediate and Posterior Uveitis may have relatively normal anterior chambers
  3. Most Uveitis findings require the magnification of a Slit Lamp exam

XIV. Management

  1. Urgent referral all cases to Ophthalmology
    1. Risk of serious complications if delayed management
  2. Timely Pupillary dilatation
  3. Topical Corticosteroids may be indicated (based on ophthalmology evaluation)

XV. References

  1. Rosenbaum in Yanoff (1999) Ophthalmology, p. 4.1
  2. Trobe (2012) Physician's Guide to Eye Care, AAO, p. 67-8
  3. Harman (2014) Am Fam Physician 90(10): 711-6 [PubMed]

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Related Studies

Ontology: Iridocyclitis (C0022073)

Definition (MSH) Acute or chronic inflammation of the iris and ciliary body characterized by exudates into the anterior chamber, discoloration of the iris, and constricted, sluggish pupil. Symptoms include radiating pain, photophobia, lacrimation, and interference with vision.
Definition (CSP) acute or chronic inflammation of the iris and ciliary body characterized by exudates into the anterior chamber, discoloration of the iris, and constricted, sluggish pupil; symptoms include radiating pain, photophobia, lacrimation, and interference with vision.
Concepts Disease or Syndrome (T047)
MSH D015863
ICD9 364.3
ICD10 H20 , H20.9
SnomedCT 267620006, 193481009, 193499001, 77971008
English Cyclitides, Heterochromic, Cyclitis, Heterochromic, Heterochromic Cyclitides, Heterochromic Cyclitis, Iridocyclitides, Unspecified iridocyclitis, Iridocyclitis, unspecified, iridocyclitis (physical finding), iridocyclitis, iridocyclitis was observed, Iridocyclitis NOS, Iridocyclitis [Disease/Finding], iridocyclitis (diagnosis), Unspecified iridocyclitis (disorder), Inflammation of iris and ciliary body, Iridocyclitis (disorder), Iridocyclitis, NOS, Iridocyclitis
Dutch niet-gespecificeerde iridocyclitis, Iridocyclitis, niet gespecificeerd, iridocyclitis, Cyclitis, heterochrome, Heterochrome cyclitis, Iridocyclitis
French Iridocyclite non précisée, Cyclite hétérochromique, Iridocyclite
German unspezifische Iridozyklitis, Iridozyklitis, nicht naeher bezeichnet, Heterochrome Zyklitis, Iridozyklitis, Zyklitis, heterochrome
Italian Iridociclite non specificata, Ciclite eterocromica, Iridociclite
Portuguese Iridociclite NE, Ciclite Heterocrômica, Iridociclite
Spanish Iridociclitis no especificada, iridociclitis no especificada (trastorno), iridociclitis no especificada, iridociclitis (trastorno), iridociclitis, Iridociclitis, Ciclitis Heterocrómica
Japanese 詳細不明の虹彩毛様体炎, ショウサイフメイノコウサイモウヨウタイエン, コウサイモウヨウタイエン, 異虹彩色性毛様体炎, 虹彩毛様体炎, 毛様体炎-異虹彩色性
Swedish Iridocyklit
Czech iridocyklitida, Blíže neurčená iridocyklitida, Iridocyklitida
Finnish Iridosykliitti
Korean 상세불명의 홍채섬모체염, 홍채섬모체염
Polish Zapalenie tęczówki i ciała rzęskowego
Hungarian Nem meghatározott iridocyclitis, Iridocyclitis
Norwegian Iridosyklitt

Ontology: Iritis (C0022081)

Definition (NCI_FDA) Inflammation of the iris.
Definition (NCI) Inflammation of the iris.
Definition (MSH) Inflammation of the iris characterized by circumcorneal injection, aqueous flare, keratotic precipitates, and constricted and sluggish pupil along with discoloration of the iris.
Definition (CSP) inflammation of the iris characterized by circumcorneal injection, aqueous flare, keratotic precipitates, and constricted and sluggish pupil along with discoloration of the iris.
Concepts Disease or Syndrome (T047)
MSH D007500
SnomedCT 65074000
English Iritides, Iritis, iritis (diagnosis), iritis, iritis (physical finding), iritis was observed, Iritis [Disease/Finding], Iris inflammation, IRITIS, Iritis (disorder), Iritis, NOS
French IRITIS, Iritis
Spanish IRITIS, iritis (trastorno), iritis, Iritis
German IRITIS, Iritis
Italian Irite, Iridite
Japanese 虹彩炎, コウサイエン
Swedish Irisinflammation
Czech iritida, zánět duhovky, duhovka - zánět, Iritida
Finnish Värikalvotulehdus
Russian IRIT, ИРИТ
Portuguese IRITIS, Irite
Polish Zapalenie tęczówki
Hungarian Iritis
Norwegian Iritt, Regnbuehinnebetennelse
Dutch iritis, Iritis

Ontology: Panuveitis (C0030343)

Definition (NCI) A disorder characterized by inflammation of the entire uvea which includes the iris, ciliary body, and choroid. Causes include systemic infections, sarcoidosis, and cancers.
Definition (MSH) Inflammation in which both the anterior and posterior segments of the uvea are involved and a specific focus is not apparent. It is often severe and extensive and a serious threat to vision. Causes include systemic diseases such as tuberculosis, sarcoidosis, and syphilis, as well as malignancies. The intermediate segment of the eye is not involved.
Concepts Disease or Syndrome (T047)
MSH D015864
ICD9 360.12
ICD10 H44.11
SnomedCT 75614007
English Panuveitis, panuveitis (diagnosis), panuveitis, Panuveitis [Disease/Finding], Panuveitis (disorder), Diffuse uveitis
Dutch panuveïtis, Panuveitis
Portuguese Pan-uveíte, Pan-Uveíte
Japanese 全ブドウ膜炎, ゼンブドウマクエン
Swedish Panuveit
Czech panuveitida, Panuveitida
Finnish Panuveiitti
Spanish Panuveítis, panuveítis (trastorno), panuveítis
Croatian Not Translated[Panuveitis]
Polish Zapalenie wszystkich części błony naczyniowej, Panuveitis
Hungarian Panuveitis
Norwegian Panuveitt
German Panuveitis
Italian Panuveite
French Uvéite totale, Panuvéite

Ontology: Uveitis (C0042164)

Definition (CHV) an inflammation of the uvea (middle part of the eye)
Definition (NCI_CTCAE) A disorder characterized by inflammation to the uvea of the eye.
Definition (NCI) An inflammatory process affecting a part of or the entire uvea. Causes include inflammatory agents (e.g., herpes simplex, herpes zoster, leptospirosis) and systemic diseases (e.g., inflammatory bowel disease, multiple sclerosis, systemic lupus erythematosus, ankylosing spondylitis). Patients present with pain and redness in the eye, light sensitivity, and blurred and decreased vision.
Definition (MSH) Inflammation of part or all of the uvea, the middle (vascular) tunic of the eye, and commonly involving the other tunics (sclera and cornea, and the retina). (Dorland, 27th ed)
Definition (CSP) inflammation of part or all of the uvea, the middle (vascular) tunic of the eye, and commonly involving the other tunics (sclera and cornea, and the retina).
Concepts Disease or Syndrome (T047)
MSH D014605
ICD10 H20.9
SnomedCT 193499001, 267719008, 155116005, 128473001, 75614007
English Uveitides, Uveitis, uveitis (physical finding), uveitis, uveitis was present, Uveitis NOS, Uveitis [Disease/Finding], Uveal inflammation, uveitis (diagnosis), UVEITIS, Uveitis (disorder), uvea; inflammation, Uveitis, NOS, Intraocular inflammation
French UVEITE, Uvéite SAI, Uvéite
Portuguese UVEITE, Uveíte NE, Uveíte
Spanish UVEITIS, Uveítis NEOM, Uveítis, uveítis (trastorno), uveítis
German UVEITIS, Uveitis NNB, Uveitis
Dutch uveïtis NAO, uvea; ontsteking, uveïtis, Uveitis
Italian Uveite NAS, Uveite
Japanese ブドウ膜炎, ブドウ膜炎NOS, ブドウマクエン, ブドウマクエンNOS
Swedish Inflammation i ögats druvhinna
Czech uveitida, živnatka - zánět, Uveitida, Uveitida NOS
Finnish Suonikalvoston tulehdus
Croatian UVEITIS
Polish Zapalenie błony naczyniowej
Hungarian Uveitis, Uveitis k.m.n.
Norwegian Uveabetennelse, Uveitt

Ontology: Anterior uveitis (C0042165)

Definition (MSH) Inflammation of the anterior uvea comprising the iris, angle structures, and the ciliary body. Manifestations of this disorder include ciliary injection, exudation into the anterior chamber, iris changes, and adhesions between the iris and lens (posterior synechiae). Intraocular pressure may be increased or reduced.
Concepts Disease or Syndrome (T047)
MSH D014606
SnomedCT 193500005, 231946008, 77971008, 410692006
English Anterior Uveitides, Uveitides, Anterior, Uveitis, Anterior, Uveitis anterior, Anterior uveitis, Uveitis, Anterior [Disease/Finding], anterior uveitis, uveitis anterior, Anterior uveitis (diagnosis), Anterior uveitis, NOS, Anterior uveitis [Ambiguous], Anterior uveitis (disorder), Anterior Uveitis
Dutch voorste uveïtis, uveïtis anterior, Uveitis anterior
German anteriore Uveitis, Uveitis anterior
Portuguese Uveíte anterior, Uveíte Anterior
Spanish Uveítis anterior, Uveítis Anterior, uveítis anterior, uveítis anterior (trastorno)
Japanese 前部ブドウ膜炎, ゼンブブドウマクエン
Swedish Uveit, främre
Czech uveitida přední, Přední uveitida
Finnish Anteriorinen uveiitti
Polish Zapalenie błony naczyniowej przedniego odcinka, Zapalenie przedniego odcinka błony naczyniowej
Hungarian Uveitis anterior, Anterior uveitis
Norwegian Uveitis anterior, Fremre uveitt
French Uvéite antérieure
Italian Uveite anteriore

Ontology: Uveitis, Intermediate (C0042166)

Definition (MSH) Inflammation of the pars plana, ciliary body, and adjacent structures.
Concepts Disease or Syndrome (T047)
MSH D015867
SnomedCT 398322000, 308126005, 193491003, 155118006, 314429009
English Chronic Cyclitis, Cyclitis, Chronic, Uveitis, Intermediate, Uveitis, Intermediate [Disease/Finding], chronic iritis, intermediate uveitis, iritis chronic, Chronic cyclitis (disorder), Intermediate uveitis, Intermediate uveitis (disorder), Chronic cyclitis, Peripheral uveitis, Chronic posterior cyclitis, Peripheral uveoretinitis, Intermediate Uveitis
Dutch perifere uveïtis, intermediaire uveïtis, Cyclitis, chronische, Intermediaire uveitis, Uveitis, intermediaire
French Uvéite périphérique, Cyclite chronique, Uvéite intermédiaire
German periphere Uveitis, intermediaere Uveitis, Zyklitis, chronische, Uveitis, intermediäre
Italian Uveite periferica, Ciclite cronica, Uveite intermedia
Portuguese Uveíte periférica, Uveíte intermédia, Ciclite Crônica, Uveíte Intermediária
Spanish Uveítis periférica, Uveítis Intermedia, ciclitis crónica, ciclitis crónica (trastorno), uveítis intermedia (trastorno), uveítis intermedia, Uveítis intermedia, Ciclitis Crónica
Japanese 周辺部ブドウ膜炎, シュウヘンブブドウマクエン, チュウカンブブドウマクエン, ブドウ膜炎-中間部, 中間部ブドウ膜炎, 中間部ぶどう膜炎, 中間部葡萄膜炎, 慢性毛様体炎, 葡萄膜炎-中間部, ぶどう膜炎-中間部, 毛様体扁平部炎
Swedish Uveit, mellanliggande
Czech uveitida intermediální, Intermediární uveitida, Periferní uveitida
Finnish Intermediaaninen uveiitti
Polish Zapalenie błony naczyniowej części pośredniej, Zapalenie części pośredniej błony naczyniowej
Hungarian Intermedier uveitis, Peripheriás uveitis
Norwegian Intermediær uveitt

Ontology: Uveitis, Posterior (C0042167)

Definition (NCI) An inflammatory process that affects the choroid.
Definition (MSH) Inflammation of the choroid as well as the retina and vitreous body. Some form of visual disturbance is usually present. The most important characteristics of posterior uveitis are vitreous opacities, choroiditis, and chorioretinitis.
Concepts Disease or Syndrome (T047)
MSH D015866
SnomedCT 193451001, 43363007, 46627006
English Uveitis, Posterior, Posterior uveitis NOS, Uveitis, Posterior [Disease/Finding], posterior uveitis, Posterior uveitis NOS (disorder), Posterior uveitis (diagnosis), uveitis posterior, Posterior uveitis, Posterior uveitis (disorder), Posterior uveitis, NOS, Posterior Uveitis, Uveitis, posterior NOS
Swedish Uveit, bakre
Czech uveitida zadní, Zadní uveitida
Finnish Posteriorinen uveiitti
Spanish Uveítis Posterior, uveítis posterior, SAI (trastorno), uveítis posterior, SAI, Uveítis posterior, uveítis posterior (trastorno), uveítis posterior
Croatian Not Translated[Uveitis, Posterior]
Polish Zapalenie błony naczyniowej tylnego odcinka, Zapalenie tylnego odcinka błony naczyniowej
Norwegian Uveitis posterior, Bakre uveitt
Dutch uveïtis posterior, Uveitis posterior
German posteriore Uveitis, Uveitis posterior
Hungarian Posterior uveitis
Japanese コウブブドウマクエン, 後部ブドウ膜炎
Portuguese Uveíte posterior, Uveíte Posterior
French Uvéite postérieure
Italian Uveite posteriore

Ontology: Neuroretinitis (C0154874)

Concepts Disease or Syndrome (T047)
MSH D012173
ICD10 H30.9
SnomedCT 410471004
Dutch neuroretinitis
German Neuroretinitis
Italian Neuroretinite
Portuguese Neurorretinite
Spanish Neuroretinitis, neurorretinitis, neurorretinitis (trastorno)
Japanese 視神経網膜炎, シシンケイモウマクエン
Czech neuroretinitida, Neuroretinitida
French Neurorétinite
English neuroretinitis (diagnosis), neuroretinitis, Neuroretinitis NOS, Neuroretinitis (disorder), Papilloretinitis, Neuroretinitis
Hungarian Neuroretinitis
Norwegian Nevroretinitt

Ontology: Keratic precipitates (C0423285)

Concepts Finding (T033)
SnomedCT 193767008, 155155006, 267730006, 246998009
Dutch hoornachtige bezinksels
French Précipités
German keratische Praezipitate
Italian Precipitati corneali
Portuguese Precipitados queráticos
Spanish Precipitado querático, precipitados queráticos (hallazgo), precipitados queráticos
Japanese 角膜後面沈着物, カクマクコウメンチンチャクブツ
Czech Rohovkové precipitáty
Hungarian Szaruhártya precipitatumok
English Keratitic precipitates, Corneal precipitates, KP - Keratic precipitates, Keratic precipitates, Keratic precipitates (finding)