Complications and management of Ocular Disorders associated with Rheumatic Disease

JONATHAN H. LASS, M.D. AND GARY M. KAMMER

Eye manifestations can occur in all of the rheumatic disorders. In some conditions, such as juvenile rheumatoid arthritis, ankylosing spondylitis and Sjogren’s syndrome, ocular abnormalities may be the presenting sign, preceding the musculoskeletal abnormalities by months to years. In other conditions, such as the vasculitides, including systemic lupus erythematosus (SLE), polyarteritis nodosa, Wegener’s granulomatosis, and progressive systemic sclerosis (scleroderma), the eye manifestations can be the presenting sign, concurrent with systemic involvement.

Under these conditions, the activity of the eye disease closely correlates with the activity of the systemic illness. Finally, in other conditions, such as adult rheumatoid arthritis, the ocular abnormality may precede, but more commonly parallels, known systemic disease. Unlike the vasculitides, the activity of eye disease in rheumatoid arthritis may be far out of proportion to the activity of the systemic illness.

The major ocular complications include keratitis sicca, peripheral corneal ulceration, scleritis, iridocyclitis, and retinal vasculitis. These complications are seen in all the rheumatic diseases, but certain ones more commonly occur in a particular rheumatic disease, i.e. keratitis sicca in rheumatoid arthritis, acute iridocyclitis in ankylosing spondylitis, and retinal vasculitis in polyarteritis nodosa. Since all of these disorders can be initial presenting sign for systemic rheumatic disease, it is important the clinician recognize them and institute the appropriate workup for diagnostic purposes, in order that there not be any delay in treatment of both the systemic as well as the ocular disease.

Associated With the Rheumatic Disease Keratitis Sicca (Dry Eye)*

Keratitis sicca is probably the most common eye complication of the rheumatic diseases. However, its symptoms and signs are often ignored, leading commonly to misdiagnosis and mistreatment. The following symptoms should alert the clinician that one is dealing with a dry eye patient: soreness, gritty or foreign body sensation, excessive mucus discharge, photophobia, and/or recurrent red, irritated eyes without purulent discharge. Paradoxically, early in the disease, some patients will complain of tearing. In these cases basal tear secretion is decreased, leading to dryness and irritation, but reflex or simulated tear secretion is normal.

Keratitis sicca may occur without other demonstrable system signs or serologic abnormalities. However, it is commonly associated with systemic disease, either primary or secondary Sjogren’s syndrome. Sjogren’s syndrome is a chronic connective tissue disorder characterized by inflammation of the exocrine glands, particularly the salivary and lacrimal glands. The disease is categorized as (1) the primary form, or (2) the secondary form, which is associated with rheumatoid arthritis (15% to 60% of cases), SLE, scleroderma, or, less commonly, other connective tissue disorders.

All mucosal tissues, as well as the lacrimal and salivary glands, can be affected by a diffuse lymphocytic infiltration and eventual destruction of their secretory function. Arthritis is not a prominent manifestation in primary Sjogren’s syndrome. In contrast, in secondary Sjogren’s syndrome, dry eyes and mouth are clearly associated with a connective tissue dis order, particularly rheumatoid arthritis. The differentiation between primary and secondary Sjogren’s syndrome is helpful for prognostication and future systemic therapy, but not ocular therapy. Differentiation can be made on clinical grounds, by serologic differences and by genetic differences. If the patient has primary Sjogren’s, there is less risk for deforming arthritis, but greater risk for lymphoma. If the patient has secondary Sjogren’s, he is subject to all the articular and extraarticular (pulmonary, renal, cardiac, skin) manifestations. The degree of ocular systemic symptomatology should influence the physician whether an extensive systemic workup is indicated when a patient initially presents with a dry eye.

Peripheral Corneal Ulceration

Peripheral corneal infiltration and/or ulceration is another presentation for a connective tissue disorder. This may be the initial presentation, but more commonly the patient has known rheumatic disorder and develops this ocular complication. Initially the patient may have minimal symptoms of foreign body sensation, light sensitivity, and blurred vision. Symptoms may progress to severe pain associated with cornea1 ulceration, scleritis, iritis,and eventual perforation.

Treatment of peripheral corneal ulceration requires management of both the ocular and systemic disease. Topical corticosteroids are of some use in the infiltrative stage of the disease, i.e. prednisolone acetate 1% four times daily for 2 weeks, then tapered.

Ocular Disorders

However, if ulceration occurs, their use must be in conjunction with systemic therapy (nonsteroidal anti-inflammatory agent, corticosteriod, immunosuppressive) and the patient must be closely monitored for progressive ulceration.

Scleritis**

Scleritis is another ocular complication of the rheumatic diseases. Scleritis is particularly common in rheumatoid arthritis.
Approximately 4% to 10% of all rheumatoid arthritis patients develop either episcleritis or scleritis. Most scleritis patients have a pre-existing rheumatic disease, but occasionally it may be the presenting sign. Unlike conjunctivitis or keratitis, patients with scleritis, and to a lesser degree, episcleritis, complain of deep, boring orbital pain with or without light sensitivity. Moreover, the injection is localized rather that diffuse, as in conjunctivitis or keratitis. Scleritis alone is not vision-threatening.

However, it is commonly associated with keratitis, iritis, secondary glaucoma, cataract, macular edema, exudative retinal
detachment, and disc edema, with a potential for severe reduction in vision. In its most severe form, necrotizing anterior scleritis with adjacent inflammation, perforation with loss of the eye may ensue. Therapy should therefore be based on the rheumatic diagnosis, the degree of pain, the potential for actual loss of vision, and the potential or occurrence of perforation.

Iridocyclitis

Iridocyclitis or anterior uveitis does not occur more frequently in the rheumatic population than in the general population.
Exceptions are juvenile rheumatoid arthritis (JRA) in children, ankylosing spondylitis in young adults, Reiter’s or Behcet’s disease in adults. Therefore, the evaluation for rheumatic disease in the child or adult with acute or chronic iridocyclitis should be directed toward these disorders rather than rheumatoid arthritis, SLE, or the vasculitides. The treatment of iridocyclitis in all these disorders is directed at the reduction of inflammation and prevention of secondary glaucoma and cataract. Unlike the treatment of peripheral cornea1 ulceration and scleritis, in which control of the systemic disease is essential, local therapy is usually sufficient and systemic therapy plays less of a role.

Topical corticosteroids are the mainstay and may be used hourly, if necessary, for severe inflammation, and then tapered. Periocular corticosteroids are particularly helpful in severe cases and should be considered before using short term, high-dose systemic corticosteroids.

Summary

Since the eye is so commonly involved in the rheumatic disorders, a team approach to the ocular and systemic management of these disorders is required. First the ophthalmologist and physician managing the systemic illness must be on the alert that ocular signs may be the initial presenting sign for an underlying rheumatic disease. Once diagnosis of a rheumatic disease is made, ocular manifestations may occur and require prompt therapy. Ocular therapy must be coordinated with systemic therapy by the team. Finally, a number of the systemic therapies for these disorders have ocular side effects, i.e. corticosteroids, nonsteroidal anti-inflammatory agents, gold, and antimalarials. Routine ophthalmologic examinations must therefore be obtained. With such a team approach, the management of the ocular complications of the rheumatic disorders will achieve the greatest success.

Editorial Comment: *Keratitis Sicca: The lacrimal plug is used to close off tear ducts temporarily or permanently to provide more liquid for the eye. There are various tearreplacement products, consult with your ophthalmologist for recommended products.

**Scleritis: Topical and non-steroidal anti-inflammatory treatments are available for Scleritis.

Editorial comment provided by Dr. Robert
Masi, Ophthalmologist