Sussing Out Sjögren’s
Identifying this condition early is possible.
KELLY K. NICHOLS, O.D., M.P.H., PH.D.
It takes 4.7 years on average from presentation to achieve a definitive Sjögren’s syndrome diagnosis. In many cases, the diagnostic path can be through an eyecare provider, yet the anguish of bumping around the healthcare system for years, often with vague diagnoses, such as fatigue, is a commonality in Sjögren’s patients.
Early detection and management could potentially modify disease progression, but, more importantly, ease the burden of not knowing what’s wrong, ultimately improving Sjögren’s syndrome patients’ quality of life.
As Sjögren’s syndrome is a chronic, progressive autoimmune disease that impacts the lacrimal and salivary glands (and other body systems, such as the stomach), diagnosis involves a dry eye disease (DED) assessment.
Current definitive diagnosis follows the American-European Consensus (see below.) Recently, the American College of Rheumatology proposed a new diagnostic criterion. It simplifies the criteria to two of three findings: (1) ocular signs (corneal fluorescein staining), (2) abnormal salivary gland biopsy and (3) a positive Ro or La (SS-A or SS-B) serology test. Both criteria are more likely to identify late-stage Sjögren’s syndrome, not early stage in which therapies could prevent progression.
The good news: New blood tests sensitive at detecting early Sjögren’s syndrome have been developed. One company, in particular, will be working with the Sjögren’s Syndrome Foundation to spread awareness about a soon-to-be-available in-office test.
In the meantime
For roughly the last five years, DED talks have focused on meibomian gland dysfunction as the leading cause. Studies reveal 60% of Sjögren’s patients had significant meibomian gland (MG) drop out, and that MG structure is altered in Sjögren’s patients before clinical symptoms are observed. Thus, clinical DED testing, a careful case history (ask about family history of autoimmune disease), meibomian gland evaluation and aqueous production measurement help in diagnosing and managing DED in Sjögren’s patients. OM
|American-European Consensus Sjögren’s Classification Criteria.|
• Ocular symptoms (at least one - dry eyes > three months, foreign body sensation, t.i.d. artificial tear use);
• Oral symptoms (at least one - dry mouth > three months, recurrent or persistently swollen salivary glands, requires liquids to swallow dry foods);
• Ocular signs (at least one – Schirmer’s test, [without anesthesia] ≤5 mm/five minutes, positive vital dye staining [van Bijsterveld ≥4]);
• Histopathology lip biopsy revealing focal lymphocytic sialadenitis (focus score ≥1 per 4 mm)
• Oral signs (at least one - unstimulated whole salivary flow [≤1.5 mL in 15 minutes], abnormal parotid sialography, abnormal salivary scintigraphy)
• Autoantibodies (at least one - anti-SSA [Ro] or Anti-SSB [La])
DR. NICHOLS IS A FOUNDATION FOR EDUCATION AND RESEARCH IN VISION (FERV) PROFESSOR AT THE UNIVERSITY OF HOUSTON COLLEGE OF OPTOMETRY. SHE LECTURES AND WRITES EXTENSIVELY ON OCULAR SURFACE DISEASE AND HAS INDUSTRY AND NIH FUNDING TO STUDY DRY EYE. SHE IS ON THE GOVERNING BOARDS OF THE TEAR FILM AND OCULAR SURFACE SOCIETY OF OPTOMETRY AND IS A PAID CONSULTANT TO ALCON, ALLERGAN, INSPIRE AND PFIZER. E-MAIL HER AT KNICHOLS@OPTOMETRY.UH.EDU, OR SEND COMMENTS TO OPTOMETRICMANAGEMENT@GMAIL.COM.