Background/Purpose: Primary Sjögren’s Syndrome (pSS) is an autoimmune disease, and Raynaud’s Phenomenon (RP) is an extraglandular manifestation that can evolve into a systemic sclerosis-overlap syndrome. Hence, the importance of identifying systemic sclerosis (SSc) in its early stages. The aim of this study was to describe the occurrence of RP in a cohort of patients with pSS. To estimate the frequency of Very Early SSc, and to describe its clinical and immunological features. To determine the frequency of development of SSc (2013 classification criteria for systemic sclerosis ACR/EULAR) in patients with pSS and RP.

Methods: An observational, descriptive, retrospective cohort study was carried out. Patients with pSS, according to ACR/EULAR classification criteria 2002 and/or 2016, attending to a public hospital in Argentina, were included. Patients with SSc-overlap syndrome were excluded. In order to identify Very Early SSc, we looked for RP associated with SSc marker autoantibodies and/or sclerodermiform pattern (SDP) in capillaroscopy. Continuous variables were described as mean and standard deviation (SD) or median and interquartile range (IQR). Categorical variables were expressed as percentages.

Results: Two hundred and seven patients with pSS were included. The mean age in years was 58 (± 14). The mean follow-up time in months was 111 (± 63), and 15% (n:32) of the patients with pSS had RP. The median time of RP evolution was 113 months (IQR: 45-168). Regarding capillaroscopy in patients with pSS and RP, 32% (n:8) had non-specific findings, 32% (n:8) early SDP, 24% (n:6) active SDP, and 4% (n:1) late SDP; 56% (n:18) of the patients with pSS and RP met the criteria for Very Early SSc with a median follow-up time of 55 months (IQR: 30-73); 53% (n:9) had only SDP, 12% (n:2) had SDP with specific antibodies, and 35% (n:6) had only specific antibodies. Anti-centromere antibody (ACA) predominated in 83% (n:5) and the anti Pm-Scl in 17% (n:1). Of all the patients with pSS and RP, 16% (n:5) developed SSc after a median follow-up of 62 months (IQR: 24-63).

Conclusion: The frequency of RP among our patients with pSS is similar to the one described in various series. Over half of them met the criteria for Very Early SSc. Abnormal capillaroscopy is the most frequently observed criterion. Of these patients, 16% developed SSc. ACA’s were the predominant antibodies. Consequently, it is of the utmost importance to contemplate RP in patients with pSS since Very Early SSc may have critical prognostic and therapeutic implications. Finally, the significance of capillaroscopy in the assessment of these patients is worth highlighting.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/development-of-systemic-sclerosis-in-patients-with-primary-sjogren-syndrome-and-raynauds-phenomenon/