Kawasaki Disease

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a primary vasculitis mediated by immunoglobulin A (IgA) affecting children younger than 5 years. The cause is unknown, but recently, a locus at 19q13 was found to be associated with increased risk for the disease. Abnormalities include fever, conjunctival injection, mucous membrane changes, extremity changes involving the skin, rash, and cervical lymphadenopathy. The most significant complication of Kawasaki disease is coronary artery aneurysm, which occurs in 15%–25% of untreated children. Coronary artery evaluation by echocardiography is therefore indicated.

After conjunctivitis, anterior uveitis during the acute phase of the illness is the second most common ocular finding, occurring in approximately 10% of cases. This is generally self-limited. Rare ocular findings include keratitis, papilledema, optic neuritis, and conjunctival hemorrhage.

Treatment with aspirin and intravenous IgG reduces the incidence of coronary artery aneurysm formation.

Alves NR, Magalhães CM, Almeida R de F, Santos RC, Gandolfi L, Pratesi R. Prospective study of Kawasaki disease complications: review of 115 cases. Rev Assoc Med Bras. 2011;57(3):295–300.

Other Causes of Anterior Uveitis

Many cases of anterior uveitis are idiopathic or are caused by trauma. Other causes include a variety of infectious and noninfectious diseases (see Table 24-1).

Intermediate Uveitis

The term intermediate uveitis is an anatomically based description of the primary site of the ocular inflammation. The inflammation is localized to the vitreous base overlying the ciliary body, pars plana, and peripheral retina, as well as the anterior vitreous (Fig 24-2). Intermediate uveitis accounts for 5%–15% of all cases of uveitis and approximately 12%–28% of uveitis cases in the pediatric age group. In children, it may occur with a variety of conditions, including sarcoidosis, syphilis, Lyme disease, multiple sclerosis, and tuberculosis. Idiopathic disease, known as pars planitis, accounts for 85%–90% of cases. The distinction between pars planitis and intermediate uveitis is not always clear. The 2 terms are often used interchangeably.