DIAGNOSTICS
Reverse Diagnosis: Sjögren's Syndrome and Dry Eye
Chances are that some of your dry-eye patients suffer from this malady.

As optometrists, we are trained to recognize the ocular manifestations of Sjögren's syndrome — primarily dry eye — but are we as prepared to co-manage a Sjögren's patient from a primary care standpoint? Let's review what you need to know.

Managing a "syndrome"

A "syndrome" can be defined as a set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease. Sjögren's syndrome has a cluster of identifiable signs and symptoms, all of which are hallmarks of several disease states. The most obvious example is the presenting dry eye symptoms in Sjögren's-related dry eye.

We are familiar with patient reports of ocular dryness, irritation and visual disturbance that can be cleared with a blink. While we routinely ask patients about these symptoms in a comprehensive eye exam, we are less accustomed to push beyond the ocular findings to explore underlying or related conditions. Take, for example, the 55-year-old female whom you've managed for dry eye and routine eye care for the past five years. Her dry eye has worsened over the past three years and you have successfully used a step-wise approach of lubricants, gel-ointments and prescription topical cyclosporine A (Restasis, Allergan) to manage her condition. But have you taken the "whole body" approach and asked appropriate questions to explore a diagnosis of Sjögren's syndrome? 

Looking for clues

The Sjögren's Syndrome Foundation (see box, page 36) estimates that more than 4 million Americans, or one in 70, have Sjögren's. The mean age of onset is 50 years, and nine out of 10 Sjögren's patients are female. Unfortunately, the disorder is often undiagnosed or the time-to-diagnosis is extremely long, causing burden to the patient, as well as the health care system.

Primary Sjögren's syndrome is defined as the development of dry eye and/or dry mouth in a previously healthy individual. The symptoms are characterized by auto-antibodies in the blood or a positive lip/salivary gland biopsy. Secondary Sjögren's is diagnosed when a person has a known connective tissue disorder (such as rheumatoid arthritis or systemic lupus erythematosus), then develops dry eye or dry mouth. In both cases, the condition is not limited to joint-related issues and ocular dryness, but can include many additional signs and symptoms.

Patients commonly experience dryness of the mouth and airways, which can make it difficult to swallow or result in a dry cough. In addition, more than 50% of Sjögren's patients experience arthritis over the course of the disease. Acid reflux, a symptom of gastrointestinal problems in Sjögren's syndrome, can induce nausea and exacerbate dry mouth.

Although more rare, kidney problems, nerve problems, lymphoma and thyroid disease have also been associated with the disorder.

Dryness of the vaginal surface is often reported to gynecolo-gists, according to the study, "Classification Criteria for Sjögren's Syndrome."And a study in the Journal of Rheumatology concluded that depression occurred in approximately 30% of patients. In addition to other symptoms, these significantly contribute to a decreased quality of life. While many successfully manage the signs and symptoms of the disorder, its chronic and persistent nature can be debilitating.

Therefore, a few extra questions in the history could aid in an early diagnosis, appropriate management and result in a better quality of life. This is especially true for women with mild-to-moderate dry eye. (For a list of Sjögren's-related questions, see, "Questions to Aid in a Sjörgen's Diagnosis," on page 35.)

Seeking moisture

It's extremely appropriate that the newsletter of the Sjögren's Syndrome Foundation is called, "Moisture Seekers." After all, one of the most frequent symptoms leading to a diagnosis is dry eye. Eye care practitioners are uniquely qualified to diagnose and manage dry eye from a primary eyecare perspective. While rarely sight-limiting, dry eye affects up to 15% of the population, according to a 2003 study in the American Journal of Ophthalmology.

Many dry-eye patients fit the profile of Sjögren's syndrome — women over 50 years of age, who comprise a frequent and reliable sector of optometric practice.

Appropriate diagnosis and management of dry eye can significantly build your medical optometric practice.

Over the last fifteen years, our understanding of dry eye has significantly increased, largely driven by the report of the National Eye Institute's Industry workshop on clinical trials in dry eye. This workshop attempted to systematically define dry eye, to set forth a classification scheme for different types of dry eye and to recommend diagnostic criteria for clinicians and researchers. The report set forth the following definition of dry eye: "A disorder of the tear film due to tear deficiency or excessive tear evaporation, which causes damage to the interpalpebral ocular surface and is associated with symptoms of ocular discomfort."

We've all had patients in whom dry eye symptoms outweigh abnormal ocular signs. In contrast, some severe dry-eye patients with significant surface damage report symptoms that don't seem to correlate with the clinical signs. Regardless of the severity of the disorder, asking the appropriate dry eye questions can assist you in monitoring change in the disorder over time or with treatment.

Dry eye symptoms

Assessing dry eye begins with patient-reported symptoms. The most frequently reported ones are dryness, irritation, discomfort and visual disturbance (fluctuation that clears with a blink). Grittiness, soreness and pain are considered more significant symptoms and may indicate progression.

Asking which symptom, in the patient's own words, is the "worst," and also, "most bothersome," can aid in monitoring disease progression. Symptoms such as end-of-day dryness and, "stopping activity to shut eyes," can also help in diagnosing dry eye.

Diagnostic tests for dry eye

While many tests have been reported over the years, critical tests in dry-eye management include assessment of corneal and conjunctival staining with fluorescein and lissamine green dyes, respectively. You can also use fluorescein tear break-up time to monitor tear film stability and the phenol red thread test or the Schirmer test to measure tear production. Sjögren's-related dry eye is defined by a Schirmer score 5mm/5min. In addition to clinical tests, monitoring the frequency of lubricant eye drop use can give an indication of change in disease status or improvement when medical therapy is added. For known Sjögren's patients, a dry-eye examination is warranted every six to 12 months, depending on response to treatment. Severe dry eye patients or patients with unstable disease may need problem-focused examinations at one-to-three month intervals.

What if you suspect Sjögren's?

Listen to your patient. If he or she describes a cluster of symptoms found in Sjögren's, a referral to a rheumatologist for evaluation is warranted. To complete the health care team, dentistry, gynecology, dermatology and neurology may be involved, as appropriate. Most im-portantly, ask your dry-eye patient the critical questions. Patients will remember and appreciate this "reverse diagnosis," or linking an ocular finding with a systemic condition from a "whole body" approach.

References available on request.

Dr. Nichols is an assistant professor at The Ohio State University College of Optometry. Contact her at Knichols@optometry.osu.edu.