Poster (15A175)

A quality improvement project to facilitate delivery of high quality care to patients with Sjögren’s Syndrome

Author(s)

Dr Bryan Murphy, Dr Claire Riddell, Dr Elisabeth Ball

Department(s)/Institutions

Musgrave Park Hospital, Belfast

Introduction

With emerging evidence for B-cell depletion therapy in Sjögren’s Syndrome1,2,3 it is important patients who may benefit from these therapies are accurately diagnosed, disease severity recorded and those at risk for lymphoma appropriately monitored.

We audited patients attending the Belfast Lupus and Connective Tissue Disease Clinic to document Sjögren’s disease burden; established a database and contributed to the United Kingdom Primary Sjögren’s Syndrome Registry (UKPSSR).

Aims/Background

Identify primary (PSS) and secondary Sjögren’s Syndrome (SSS) patients attending the clinic

Determine diagnostic accuracy according to 2002 American- European Consensus Criteria (AECG)4 and 2012 American College of Rheumatology classification criteria (ACR)5 Record disease severity using clinician ESSDAI6 and patient ESSPRI6 scores

Ensure appropriate follow-up of patients to prevent accumulation of disease damage and enable early lymphoma diagnosis

Register data to UKPSSR (http://sjogrensregistry.org/index.php); a national database for PSS research

Method

Patients identified as PSS or SSS from reviews over a 1year period

Audit of charts was performed and registered with the local audit department

Where available documented demographics, diagnostic features, blood tests and lymphoma risk-factors

PSS patients invited to a clinic where ESSDAI/ESSPRI scores, Schirmer’s test and salivary-flow were documented to confirm primary status and consenting patients registered to UKPSSR

Results

Results and database; see Table 1. ACR diagnostic criteria are limiting as we do not routinely refer for lip biopsy or ocular staining scores; 12/15 met AECG criteria. ESSDAI rises with prolonged disease duration however correlation with ESSPRI, Schirmer’s and salivary-flow was not always clear. Three patients had three lymphoma risk-factors, one developed parotid lymphoma. Another had resolution of parotid enlargement following Rituximab.

Conclusions

Data collection provides invaluable information to guide clinical decisions and service development. Using our database we now document relevant features, tests and lymphoma risk-factors and consider ocular staining or salivary gland biopsy if uncertain. Disparity between some patients’ higher self-reported ESSPR compared with clinician ESSDAI raises management challenges. Those with lymphoma riskpage 50 factors will be reviewed more frequently given reported relativerisk of non-Hodgkins lymphoma at 13.76 in PSS7. Data was registered with the UKPSSR to aid ongoing research into PSS.