Type of Proposal
Schulich School of Medicine Windsor
24-3-2015 10:00 AM
24-3-2015 10:50 AM
Importance of the Project
This project has implications on how overlapping diseases affect presentation on prognosis for people diagnosed with Sjogren's syndrome. It suggests that patients who present with overlapping diseases are different, and perhaps should be managed differently than those without overlap.
Existing State of Knowledge
There have been case reports on people with exhibit overlapping diseases in patients diagnosed with Sjogren's syndrome. Not much is known about this population as a whole. Our work relates to this because we assessed a large pool of patients to look for commonalities in the group with overlapping diseases. Thus, this is more applicable and informative than case reports.
To determine if the presence of CREST syndrome (limited scleroderma) affects the incidence or severity of sicca symptoms (dry eyes/mouth) in patients being evaluated for Primary Sjogren's syndrome.
Patients were pre-screened for objective evidence of abnormal serology, dry eyes or dry mouth based on Schirmers test1 and unstimulated salivary flow (USSF)3.
609 patients were assessed on protocol at the Sjogren’s Clinic of the Toronto Western Hospital over 22 years.
The evaluation included a visual analogue score (VAS) for severity of xerophthalmia and xerostomia, as well as Schirmer’s-1 test1, Rose Bengal staining2, USSF3, minor salivary gland biopsy and serology.
PSjS was classified according the American European Consensus Group (AECG) Criteria4.
Patients with anti-centromere antibody (ACA) were designated as CREST Syndrome whether or not they met criteria for PSjS.
A 2-tailed student t-test with heterogenous variance was used to compare measures of severity.
CREST syndrome overlap patients had subjectively and objectively worse xerostomia than PSjS patients without overlap.
CREST syndrome overlap patients had more severe objective xerophthalmia than PSjS patients. A significant difference in ocular symptoms could not be detected.
CREST patients may meet AECG Criteria for PSjS despite a less frequent anti-Ro or –La positivity. These patients have a less frequent but more profound elevation of IgG levels.
Limited Scleroderma (CREST Syndrome) is associated with worse xerostomia and xerophthalmia in patients being evaluated for Primary Sjogren’s Syndrome