Acute Anterior Uveitis

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Acute anterior uveitis is the most common form of uveitis.

Disease Entity

Acute Anterior Uveitis

Disease

Uveitis is not a single disease. Similar to arthritis (joint inflammation), uveitis can be a part of many different disease processes. Different types of uveitis often follow characteristic patterns that are distinguished by factors such as what part of the eye is affected, if the inflammation involves one or both eyes, if the inflammation began suddenly or gradually, if the inflammation completely resolves with treatment which can be stopped (acute or recurrent acute), or if it recurs off therapy (chronic) disease. Of these many subsets, the most common presentation for uveitis is undoubtedly acute anterior uveitis or AAU.

Anterior means that the front portion of the uvea, the iris and ciliary body, are primarily affected by the inflammation. The anterior part of the uvea is the iris that surrounds the pupil and the adjacent ciliary body that synthesizes aqueous humor, the fluid that fills the front of the eye. Inflammatory cells in anterior uveitis may therefore be seen in the anterior chamber (iritis), and sometimes the anterior vitreous (behind the lens, in iridocyclitis).

Acute disease is characterized by a sudden onset and limited duration. The symptoms of acute anterior uveitis are pain, redness, and photophobia (sensitivity to light), that typically develop rapidly, over a few days. The symptoms will resolve with appropriate anti-inflammatory therapy. If therapy can be tapered and inflammation does not recur for at least three months off of treatment, the disease is said to be of limited duration. The episode may be a single episode, or may be recurrent, with symptoms and signs recurring after at least three months of absence of inflammation off of all therapy. This is in contrast to chronic disease, where inflammation (with or without symptoms) recurs when medication is tapered and stopped.

Etiology

AAU may occur as an isolated medical problem without any association with illness or inflammation elsewhere in the body. It might also arise as part of a localized infection such as that due to the virus that causes cold sores, herpes simplex. It can occur rarely as an adverse reaction to a medication. AAU may also be associated with an illness affecting multiple parts of the body. The most common illnesses associated with AAU are also associated with a genotype known as HLA-B27. A detailed explanation of HLA-B27 and those diseases associated with HLA-B27 is found elsewhere on this website, but the most common association with AAU is ankylosing spondylitis, an inflammatory arthritis associated with HLA B27. Most diseases of other organs that are linked to AAU are apparent from associated symptoms. For example, bowel inflammation or colitis can be associated with AAU. A patient who has this association would usually have abdominal problems such as crampy abdominal pain, weight loss, and/or diarrhea. Some of the less common diseases associated with AAU include sarcoidosis, interstitial nephritis (a rare kidney inflammation), relapsing polychondritis (a rare autoimmune disease), and vasculitis (an inflammation of the blood vessel wall).

Risk Factors

HLA-B27 allele, anklyosing spondylitis, psoriatic arthritis, certain medications

Pathophysiology

Unknown. A leading theory is that exposure of an individual with a genetic predisposition to an infectious agent results in cross reactivity with ocular specific antigens (molecular mimicry) with resultant iritis.

Primary prevention

None available. There is evidence from animal literature that the gut microbiome is important in the risk of developing HLA B27 related disease [1][2]

Diagnosis

Characteristic disease in the setting of known risk factors (HLA-B27), with exclusion of other possible causes. All patients should have a work up with at a minimum syphilis titers. Depending on presentation and review of systems other diagnostic tests may be warranted including ocular fluid testing for viruses such as HSV,CMV, and VZV.

Physical Examination

The symptoms of AAU are generally eye redness, pain, and sensitivity to light (photophobia). Sometimes the vision is reduced, but this varies. Tearing, lid puffiness, and some drooping of the eyelid may also be present. Inflammation or infection in other parts of the eye may produce similar symptoms. For example, an ophthalmologist must distinguish AAU from diseases of other eye structures such as conjunctivitis (pink eye), keratitis (inflammation of the cornea), or scleritis (inflammation of the white part of the eye). The slit lamp is the most important tool that is used in distinguishing these entities. In AAU, white blood cells accumulate in the fluid filled space in the front of the eye. They are readily detectable by the slit lamp. AAU requires these cells to be present in the anterior chamber in order for the diagnosis to be made. All patients with AAU should initially undergo a dilated eye examination. This permits the examiner a view toward the back of the eye. The possible cause of AAU changes considerably if the inflammation in the front of the eye is accompanied by marked inflammation in the back of the eye.

  • Anterior chamber cell
  • Keratic Precipitates
  • Flare
  • Hypopyon
  • Iris nodules
  • Posterior synechiae
  • Fibrin
  • Pupillary miosis
  • Band keratopathy

Diagnostic procedures

The basic workup for acute anterior uveitis includes HLA-B27 and syphilis testing. For bilateral granulomatous disease one should also consider testing for sarcoid (CXR at least–the utility of angiotensin converting enzyme (ACE) and lysozyme testing is debated among uveitis specialists as many factors impact the level.[3]) Other laboratory testing can be performed based on clinical suspicion for various diseases. Ocular fluid testing may be warranted in patients who present with a hypertensive anterior uveitis to test for herpetic etiology (although many providers treat empirically based on exam findings).

Differential diagnosis

Infectious

  • Syphilis
  • Tuberculosis
  • HSV
  • CMV
  • Toxoplasmosis
  • Rubella
  • VZV

Inflammatory

  • HLA-B27 Associated
  • Inflammatory Bowel Disease
  • Psoriatic arthritis
  • Sarcoidosis
  • Tubulointerstitial nephritis and uveitis
  • Post-infectious

Malignancy

  • Lymphoma
  • Retinoblastoma

Other

  • Idiopathic
  • Medication-induced

Management

Medical Therapy

The mainstay of therapy for AAU is topical drops. These usually include a topical corticosteroid drop such as prednisolone acetate 1% and often a dilating drop such as cyclopentolate. The corticosteroid drop treats the underlying inflammation. The dilating drop reduces pain and helps to prevent the complication of the pupil sticking to the adjacent lens. The frequency of the drops depends primarily on the intensity of the inflammation. Some forms of AAU are associated with an infection such as herpes and will also require therapy directed at the known infectious cause. Other patients may have an illness of other organs that will also impact the treatment recommendations. On occasion AAU is severe enough to warrant treatment by the local injection (shot) of corticosteroid near the eye itself or by oral therapy such as prednisone. Any treatment, including eye drops, may result in adverse effects; these must be balanced with potential benefits in recommending therapies. Complications of inflammation such as an elevated intraocular pressure and cystoid macular edema may also affect treatment choices.

For recurrent or chronic disease and flares that are vision threatening despite local therapy, systemic immunosuppression therapy may be indicated that sometimes requires the expertise of a rheumatologist or uveitis specialist.

Surgery

There is no specific surgical treatment for uveitis. Surgery is reserved for dealing with the complications of uveitis. Typically intraocular surgery is only performed when the eye has been quiet for 3 months.

Complications

AAU may have complications such as the formation of posterior synechiae, band keratopathy or a rise in intraocular pressure, which may lead to glaucoma. AAU can also cause fluid to accumulate in the portion of the retina responsible for central vision, the macula. This complication is known as cystoid macular edema (CME). Patients may also develop a cataract either from inflammation or corticosteroids. These complications may affect the choices for treatment.

Prognosis

Some forms of AAU have a tendency to recur. Prompt initiation of treatment at the time of recurrence may shorten the duration of the attack or improve the prognosis, but treatment should always be guided by a physician who should confirm the patient's suspected diagnosis at the earliest possible time.

Acknowledgements

This article is based on a patient education monograph originally prepared for the American Uveitis Society in January 2003 by James T. Rosenbaum, MD Oregon Health Sciences University Portland, OR, USA

Additional Resources

References

  1. Rosenbaum JT, Lin P, Asquith M. Does the Microbiome Cause B27-related Acute Anterior Uveitis? Ocul Immunol Inflamm. 2016 Mar 22:1-5.
  2. Kabeerdoss J, Sandhya P, Danda D. Gut inflammation and microbiome in spondyloarthritis. Rheumatol Int. 2016 Apr;36(4):457-68
  3. 10 Clinical Pearls for Treating Uveitis
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