P143 Pruritus and cutaneous manifestations are common and not-to-be-underestimated extraglandular features of Sjögren disease: results from a patient survey

Abstract

Sjögren disease (SjD) is a multisystemic autoimmune condition associated with various cutaneous manifestations including xerosis, purpura, small-vessel vasculitis, urticarial vasculitis and annular erythema. The frequency of cutaneous SjD varies in the literature, with cutaneous manifestations in up to 74% of cases. Pruritus has been suggested as an underestimated symptom and may be the heralding sign of cutaneous SjD. It is unclear how pruritus and cutaneous SjD affect patients and whether they would want to be reviewed by dermatologists for skin assessment and care. The objective of this study was to assess the skin disease burden in SjD using an internally developed questionnaire. We conducted a survey in 33 consecutive patients with primary SjD reviewed in tertiary SjD clinic (run by rheumatology). Overall, 31 of 33 (94%) of patients were female, and the mean age was 55 years (SD 12.2). In total, 24 of 33 (73%) reported their skin was affected by SjD, present for an average of 4 years prior to diagnosis. The reported features were pruritus (52%), rash (33%), xerosis (21%) and other (9%). For those affected, patients reported a mild-to-moderate degree of itch, with mean of 4 out of 10 on a numerical rating scale. Overall, 21 of 33 (64%) felt dermatology review would be helpful for symptomatic relief (33%), diagnostic help (33%) and skincare advice (21%). About three-quarters of the patients with SjD seen in our tertiary clinic reported having cutaneous SjD, in line with published literature. Xerosis and pruritus were the most common features and often predated a diagnosis of SjD. The findings from this survey support the need for timely input from dermatologists to improve care and overall patient experience. Future work should improve cutaneous assessment in SjD, recognizing the impact on health-related quality of life, as well as establishing a clear and effective pathway for dermatology input in SjD.