Juvenile Idiopathic Arthritis Uveitis Clinical Presentation

Updated: Jan 06, 2016
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Always complete a thorough history of new patients. The ocular immunology and uveitis survey form, developed by Dr. C Stephen Foster, provides a complete checklist (present illness, past medical history, family history, and review of systems) and may assist the physician in the history. The patient questionnaire may be downloaded from the Massachusetts Eye and Ear Infirmary Immunology and Uveitis Service.

Many patients are referred first by a pediatrician or rheumatologist and often are asymptomatic. Typically, patients have no pain or photophobia and the eye appears white.

Asymptomatic patients may recall previous insignificant symptoms that may be useful in determining the duration of the ocular disease. Therefore, ask specific questions regarding past ocular history, such as previous episodes of pink eye or conjunctivitis, blurry vision, ocular pain, or abnormal pupil size/shape.

Past medical history may include the following:

  • Crucial to establish chronicity and to determine the subtype of JIA

  • Age at onset

  • Specific joints involved and number of joints

  • Other systemic manifestations are as follows:

    • Fever, rash, lymphadenopathy, fatigue, weight loss, hepatosplenomegaly, pericardial effusion, pleural effusion

    • Anemia

    • Growth retardation, delay of secondary sexual characteristics

    • Chronic low back pain, diarrhea, psoriasis

  • Current systemic medications for JIA

Past ocular history may include the following:

  • Previous episodes and treatment

  • Previous surgeries

  • Previous complications, as follows:

    • Cataract

    • Glaucoma

    • Band keratopathy

    • Cystoid macular edema (CME)

Review of systems may reveal the following:

  • General - Weight loss, fatigue, fever

  • Skin - Rash, nodules, changes in nails

  • Neck - Lymphadenopathy

  • Respiratory - Cough, wheezing

  • Cardiac - Chest pain/discomfort, dyspnea

  • Gastrointestinal - Hepatomegaly, diarrhea, frequent bowel movements

  • Genitourinary - Delayed secondary sexual characteristics

  • Musculoskeletal - Muscle or joint pain, arthritis, back pain, limitation of motion

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Physical

Ocular manifestations

Perform a complete ophthalmic examination.

  • Vision - Best-corrected visual acuity, including near acuity

  • Anterior uveitis

    • Cells and flare; chronic flare (very common)

    • Nongranulomatous uveitis (>90%)

    • Bilateral (70-80%)

    • Iridocyclitis in approximately 90% of patients; rarely panuveitis or vitritis

    • Chronic smoldering or recurrent disease in greater than 90%; rarely acute monophasic course (< 5%)

  • Conjunctiva and sclera - Most patients have no conjunctival injection even during acute exacerbations.

  • Cornea

    • Band keratopathy (shown in the image below) - Corneal degeneration that derives its name from the distinctive appearance of calcium deposition in a band across the central cornea

      Juvenile idiopathic arthritis uveitis. Band kerato Juvenile idiopathic arthritis uveitis. Band keratopathy. Courtesy of Manolette Roque, MD, Roque Eye Clinic.
    • Keratic precipitates - Small-medium, rarely mutton fat

  • Iris - Posterior synechiae; pupillary membrane; rarely may develop Koeppe nodules

  • Pupil examination

  • Lens - Posterior subcapsular cataracts

  • Posterior uveitis

    • Dilated fundus examination - Vitritis, CME, hypotony maculopathy

    • Anterior vitreous (anterior vitreous cells not uncommon)

  • Others - Intraocular pressure, secondary glaucoma (open angle or pupillary block), hypotony

Articular manifestations

Assessment must consist of a quick evaluation of the skin and joints (warmth, redness, effusion, and deformity). Identify the particular joint and number involved.

The ophthalmologist evaluating the patient may perform this; however, the pediatrician or rheumatologist is expected to execute a complete musculoskeletal examination.

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Causes

The cause of uveitis and arthritis in JIA remains unknown. Akin to many other autoimmune diseases, the target antigen is unidentified. Associated factors may include the possibility of infectious triggers, a genetic predisposition, an autoimmune response, psychological stress, female sex, and hormone interaction. [13, 9]

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Complications

Ocular complications may be sight threatening and include glaucoma, cataract, cyclitic membrane and hypotony, and band keratopathy. [14, 15, 8, 5]

Although uveitis in JIA usually is anterior, vitritis, CME, and optic nerve edema may be present.

Complications from lack of treatment [16]  may include the following:

  • Cataracts (40-80%)

  • Band keratopathy (30-80%)

  • Macular edema or epiretinal membrane formation (30-50% in chronic cases)

  • Vitreous haze/debris (20-30%)

  • Glaucoma (10-30%)

  • Chronic hypotony and phthisis (5-20%)

  • Other posterior pole complications (eg, disc neovascularization, macular hole) are rare. [17, 18]

Complications from treatment [12, 16]  may include the following:

  • Cataracts, keratitis, and steroid-induced glaucoma from topical steroids

  • Lid abnormalities, orbital socket contraction, and globe perforation from regional corticosteroids

  • Gastrointestinal bleeding from nonsteroidal NSAIDs

  • Growth retardation, weight gain, acne, mood swings, and infections from systemic corticosteroids

  • Bone marrow suppression and pancytopenia from immunosuppressive therapy (methotrexate, cyclosporine-A, cyclophosphamide, chlorambucil)

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