Systemic Complications Are Common in Sjögren Syndrome

Christopher J. Rapuano, MD


June 02, 2015


Sjögren syndrome is a multisystem autoimmune disease. Classic presentations include dry mouth and dry eye from involvement of the salivary and lacrimal glands. Systemic involvement is also common. Approximately one third have nonvisceral (eg, vasculitic skin lesions, arthritis, peripheral neuropathies) and visceral (eg, interstitial lung or kidney disease) involvement. Patients with Sjögren syndrome have an increased risk for non-Hodgkin lymphoma. It is estimated that approximately 10% of patients with significant dry eye disease have Sjögren syndrome. However, fewer than one half of patients with Sjögren syndrome have been diagnosed with it prior to their presentation to their eye doctors.

Although this study was performed at a tertiary care hospital and may not be generalizable to all routine office practices, it provides some very important information to those of us seeing patients with dry eyes. Perhaps it shouldn't surprise me that these authors found a greater than 10-year lag between the onset of dry eye symptoms and the diagnosis of Sjögren syndrome. The reason is likely multifactorial, including the fact that many of our patients have dry eye symptoms, the diagnosis of Sjögren syndrome is not straightforward, and getting an appointment with an internist or rheumatologist interested in Sjögren syndrome is not always very easy.

However, as this paper demonstrates, patients with Sjögren syndrome do not only have a high risk for vision-threatening issues but also a high risk for systemic issues. The authors noted that 28% of their patients had peripheral neuropathy, 6% vasculitis, and 5% interstitial nephritis, all three of which were statistically significantly greater in the patients with vision-threatening eye problems. Additionally, 3% had lymphoma.

We should at least consider the possibility of Sjögren syndrome in all of our patients with dry eye, especially the more severe ones. As dry mouth is a common symptom of Sjögren syndrome, we should be asking patients about it. Patients with a combination of dry eye and dry mouth, especially if they have any systemic autoimmune conditions, are at increased risk of having Sjögren syndrome.

As ophthalmologists, we have several options when encountering such a patient. We can refer the patient to their internist or to a rheumatologist. However, in my experience, unless there are some positive laboratory findings pointing to a diagnosis of Sjögren syndrome, many internists and rheumatologists are simply too busy to pay much attention to these patients.

Many cornea specialists have been starting a work-up for Sjögren syndrome, including ordering blood tests such as Sjögren syndrome-A, Sjögren syndrome-B, antinuclear antibody, and rheumatoid factor. Although these may be positive, unfortunately they are often negative early in the disease process. There is a new blood test called Sjo with some novel proprietary markers that are thought to identify Sjögren syndrome at an earlier stage. This test can be performed in the office using a finger stick or blood draw, or patients can be sent to a commercial lab for a blood draw. The results are available in 1 or 2 weeks, with a subsequent interpretation from the company as to the risk for Sjögren syndrome. I have found that patients are more eager to seek further evaluation, and the internist or rheumatologist is more likely to see them sooner in the presence of a positive blood test.

And, although this paper didn't look at this factor, it is presumed that an earlier diagnosis of Sjögren syndrome will improve the treatment and outcomes of this serious condition. That would be an interesting question to address next.



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