New Guidelines for Sjögren's Syndrome

Sjogren's syndrome gets new guidelines to improve diagnosis and research

(RxWiki News) Many Americans may not know about Sjögren's syndrome. Yet, the condition often affects people with common immune system disorders like rheumatoid arthritis. Until recently, doctors had little guidance on how to diagnose the condition.

An international group of researchers have developed a new set of guidelines to improve diagnosis and research of Sjögren's syndrome.

"Tell your doctor if you have dry mouth and eyes."

Because Sjögren's syndrome often accompanies diseases like rheumatoid arthritis and lupus, care for patients with the condition generally falls into the hands of rheumatologists. However, according Steve Shiboski, PhD, of the University of California San Francisco (UCSF), and colleagues, diagnosing and treating Sjögren's syndrome requires three areas of specialty practice: rheumatology, ophthalmology (study of the eye) and oral medicine.

Some of the symptoms of Sjögren's syndrome - such as dry mouth and dry eyes - are not specific to Sjögren's syndrome, said guideline co-author Lindsey Criswell, MD, MPH, chief of the UCSF Division of Rheumatology, in a UCSF news release. In other words, these symptoms could be signs of other conditions.

"In previous years, when those common and non-specific symptoms were part of the classification criteria [diagnosis], some of those patients indeed had Sjögren's syndrome, but others who met those criteria probably had something else," said Dr. Criswell.

Correctly diagnosing patients is important. It is similarly important to correctly diagnose those without Sjögren's syndrome, as treatment of the condition requires drugs that can have some dangerous side effects, said Dr. Shiboski in the UCSF news release.

The new guidelines were created to make diagnosis of Sjögren's syndrome simpler and more specific.

The guidelines recommend that a diagnosis of Sjögren's syndrome should be made in patients with at least two of the following three features:

  • Presence of antibodies associated with Sjögren's syndrome, specifically anti-SSA and/or anti-SSB
  • Labial salivary gland biopsy (removal of salivary glands from the lips) that shows signs of inflammation linked to Sjögren's syndrome
  • Positive test for keratoconjunctivitis sicca, an eye disease caused by eye dryness

In addition, the guidelines suggest which patients should not be included in studies on Sjögren's syndrome or its treatments. These people include those with:

  • History of head and neck radiation treatment
  • Hepatitis C infection
  • Acquired immunodeficiency syndrome
  • Sarcoidosis - a disease in which the lymph nodes, lungs, liver, eyes, skin and other tissues become inflamed
  • Amyloidosis - when a specific type of protein builds up in the organs, causing damage
  • Graft versus host disease
  • IgG4-related disease

According to the guideline authors, doctors should find it easy to use these guidelines for diagnosing Sjögren's syndrome, even though they may involve more than one health specialist.

Study results show that using these guidelines improved the accuracy of diagnosis, making them a better option in situations where a misdiagnosis could pose a threat to patients, the authors concluded.

The research was funded by the National Institute for Dental and Craniofacial Research, National Eye Institutes and Office of Research on Women's Health.

The study and guidelines were published in Arthritis Care & Research, a journal of the American College of Rheumatology.

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Review Date: 
September 10, 2012