(See also Introduction to Corneal Disorders Introduction to Corneal Disorders Symptoms that suggest corneal involvement (eg, rather than simple conjunctivitis) include unilateral involvement, pain (foreign body sensation and ache—not just a gritty sensation), particularly... read more .)
Peripheral ulcerative keratitis is a serious corneal ulceration; it often occurs with autoimmune connective tissue diseases that are active, long-standing, or both, such as rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease that primarily involves the joints. RA causes damage mediated by cytokines, chemokines, and metalloproteases. Characteristically... read more (RA), granulomatosis with polyangiitis Granulomatosis with Polyangiitis (GPA) Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent... read more (formerly called Wegener granulomatosis), and relapsing polychondritis Relapsing Polychondritis Relapsing polychondritis is a rare, episodic, inflammatory, and destructive disorder involving primarily cartilage of the ear and nose but also potentially affecting the eyes, tracheobronchial... read more .
Patients often have decreased visual acuity, photophobia, and foreign body sensation. A crescentic area of opacification develops in the periphery of the cornea due to infiltration by white blood cells (WBCs). Ulceration, which stains with fluorescein, develops shortly after opacification. This is the opposite to the usual infectious ulcer that begins with an epithelial defect and later develops opacification. Infectious causes, such as bacteria, fungi, and herpes simplex virus, must be ruled out by culturing the ulcer and eyelid margins.
Among patients with autoimmune connective tissue disease and peripheral ulcerative keratitis, the 10-year mortality rate is about 40% (usually due to myocardial infarction) without treatment and about 8% with systemic cytotoxic therapy.
Any patient with peripheral ulcerative keratitis should be promptly referred to an ophthalmologist. Systemic cyclophosphamide, other immunosuppressants, or biologicals such as rituximab treat the keratitis, life-threatening vasculitis, and underlying autoimmune disease. Treatment also includes local approaches to control inflammation (eg, tissue adhesive and bandage contact lenses) and repair damage (eg, patch grafts). Other possibly helpful drugs include collagenase inhibitors, such as systemic tetracycline or topical 20% N-acetylcysteine.