ESSENTIALS OF DIAGNOSIS
Usually immunologic but possibly infective or neoplastic.
Inflammation may be confined to the eye or may be systemic.
Acute anterior uveitis: sudden redness and blurry vision often with photophobia.
Posterior uveitis: gradual loss of vision, commonly with floaters, in a variably inflamed eye.
Intraocular inflammation (uveitis) is classified as acute or chronic and as nongranulomatous or granulomatous, according to the clinical signs, and by its involvement of the anterior, intermediate, posterior, or all (panuveitis) segments of the eye.
In most cases the pathogenesis of uveitis is primarily immunologic, but infection may be the cause, particularly in immunodeficiency states. The systemic disorders associated with acute nongranulomatous anterior uveitis are the HLA-B27-related conditions (ankylosing spondylitis, reactive arthritis, psoriasis, ulcerative colitis, and Crohn disease). Chronic nongranulomatous anterior uveitis occurs in juvenile idiopathic arthritis. Behçet syndrome produces both anterior uveitis, with recurrent hypopyon, and posterior uveitis, characteristically with branch retinal vein occlusions. Both herpes simplex and herpes zoster infections may cause nongranulomatous and granulomatous anterior uveitis as well as retinitis (acute retinal necrosis).
Diseases producing granulomatous anterior uveitis also tend to be causes of posterior uveitis. These include sarcoidosis, toxoplasmosis, tuberculosis, syphilis, Vogt-Koyanagi-Harada disease (bilateral uveitis associated with alopecia, poliosis [depigmented eyelashes, eyebrows, or hair], vitiligo, and hearing loss) (eFigure 7–32), and sympathetic ophthalmia that occurs after penetrating ocular trauma. In toxoplasmosis, there may be evidence of previous episodes of retinochoroiditis. Syphilis characteristically produces a “salt and pepper” fundus but may present with a wide variety of clinical manifestations. The principal pathogens responsible for ocular inflammation in HIV infection are cytomegalovirus (CMV), herpes simplex and herpes zoster viruses, mycobacteria, Cryptococcus, Toxoplasma, and Candida.
Vogt–Koyanagi–Harada (VKH) disease. A: Poliosis of the eyelashes. B: Alopecia, vitiligo, and poliosis of the hair. (Reproduced, with permission, from A: Lueder GT. Pediatric Practice: Ophthalmology. McGraw-Hill, 2011; B: Riordan-Eva P, Augsburger JJ. Vaughan & Asbury's General Ophthalmology, 19th ed. McGraw-Hill, 2018.)
Retinal vasculitis and intermediate uveitis predominantly manifest as posterior uveitis with central or peripheral retinal abnormalities in retinal vasculitis and far peripheral retinal abnormalities (pars planitis) in intermediate uveitis. Retinal vasculitis can be caused by a wide variety of infectious agents and noninfectious systemic conditions but also may be idiopathic. Intermediate uveitis is often idiopathic but can be due to multiple sclerosis or sarcoidosis.
Anterior uveitis is characterized by inflammatory cells and flare within the aqueous. In severe cases, there may be hypopyon (layered collection of white cells) (eFigure 7–33) and fibrin within the anterior chamber. Cells may also be seen on the corneal endothelium as keratic precipitates. In granulomatous uveitis, there are ...