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Interstitial Keratitis

(Parenchymatous Keratitis)


Melvin I. Roat

, MD, FACS, Sidney Kimmel Medical College at Thomas Jefferson University

Last full review/revision May 2020| Content last modified May 2020
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Interstitial keratitis is chronic, nonulcerative inflammation of the mid-stroma (the middle layers of the cornea) that is sometimes associated with uveitis. The cause is usually infectious. Symptoms are photophobia, pain, lacrimation, and vision blurring. Diagnosis is by slit-lamp examination and serologic tests to determine the cause. Treatment is directed at the cause and may require topical corticosteroids.

Interstitial keratitis, a manifestation of certain corneal infections, is rare in the US. Most cases occur in children or adolescents as a late complication of congenital syphilis Congenital Syphilis Congenital syphilis is a multisystem infection caused by Treponema pallidum and transmitted to the fetus via the placenta. Early signs are characteristic skin lesions, lymphadenopathy, hepatosplenomegaly... read more Congenital Syphilis . Ultimately, both eyes may be involved. A similar but less dramatic bilateral keratitis occurs in Cogan syndrome Cogan Syndrome Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. The disease appears to result... read more , Lyme disease Lyme Disease Lyme disease is a tick-transmitted infection caused by the spirochete Borrelia species. Early symptoms include an erythema migrans rash, which may be followed weeks to months later by neurologic... read more Lyme Disease , and Epstein-Barr virus infection Infectious Mononucleosis Infectious mononucleosis is caused by Epstein-Barr virus (EBV, human herpesvirus type 4) and is characterized by fatigue, fever, pharyngitis, and lymphadenopathy. Fatigue may persist weeks or... read more Infectious Mononucleosis . Rarely, acquired syphilis Syphilis Syphilis is caused by the spirochete Treponema pallidum and is characterized by 3 sequential clinical, symptomatic stages separated by periods of asymptomatic latent infection. Common manifestations... read more Syphilis , herpes simplex Herpes Simplex Virus (HSV) Infections Herpes simplex viruses (human herpesviruses types 1 and 2) commonly cause recurrent infection affecting the skin, mouth, lips, eyes, and genitals. Common severe infections include encephalitis... read more Herpes Simplex Virus (HSV) Infections , herpes zoster Herpes Zoster Herpes zoster is infection that results when varicella-zoster virus reactivates from its latent state in a posterior dorsal root ganglion. Symptoms usually begin with pain along the affected... read more Herpes Zoster , or tuberculosis Tuberculosis (TB) Tuberculosis (TB) is a chronic, progressive mycobacterial infection, often with a period of latency following initial infection. TB most commonly affects the lungs. Symptoms include productive... read more Tuberculosis (TB) may cause a unilateral form in adults.

Symptoms and Signs of Interstitial Keratitis

Photophobia, pain, lacrimation, and vision blurring are common. The lesion begins as patches of inflammation in the mid-stroma that cause opacification. Typically with syphilis and occasionally with other causes, the entire cornea develops a ground-glass appearance, obscuring the iris. New blood vessels grow in from the limbus (neovascularization) and cause orange-red areas (salmon patches). Anterior uveitis and choroiditis are common in syphilitic interstitial keratitis. Inflammation and neovascularization usually begin to subside after 1 to 2 months. Some corneal opacity usually remains, causing mild to moderate vision impairment.

Diagnosis of Interstitial Keratitis

  • Corneal opacification and other typical findings on slit-lamp examination

  • Serologic testing to determine etiology

The specific etiology must be determined. The stigmas of congenital syphilis, vestibuloauditory symptoms, history of an expanding rash, and tick exposure support specific etiologies. However, all patients should have serologic testing, including all of the following:

  • Fluorescent treponemal antibody absorption test or the microhemagglutination assay for Treponema pallidum are the usual screening tests

  • Lyme titer

  • Epstein-Barr virus panel

New tests such as Treponema pallidum particle agglutination assay, Treponema pallidum enzyme immunoassay, chemiluminescence immunoassay, and nucleic acid amplification tests (NAAT) may also be useful in diagnosing ocular syphilis.

Patients with negative serologic test results may have Cogan syndrome Cogan Syndrome Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Cogan syndrome affects young adults, with 80% of patients between 14 and 47 years. The disease appears to result... read more , an idiopathic syndrome consisting of interstitial keratitis and vestibular and auditory deficits. To prevent permanent vestibuloauditory damage, symptoms of hearing loss, tinnitus, or vertigo require urgent referral to an otolaryngologist.

Treatment of Interstitial Keratitis

  • Sometimes topical corticosteroids

Keratitis may resolve with treatment of the underlying condition. Additional topical treatment with a corticosteroid, such as prednisolone 1% four times/day, is often advisable. An ophthalmologist should treat these patients.

Key Points

  • Interstitial keratitis, which is rare in the US, involves chronic inflammation of the middle corneal layers.

  • Findings include pain, tearing, decreased visual acuity, and often orange-red discoloration of the cornea and anterior uveitis.

  • Test patients for syphilis, Lyme disease, and Epstein-Barr virus infection.

  • Treatment is by an ophthalmologist; sometimes topical corticosteroids are prescribed.

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