Development of systemic sclerosis in patients with primary Sjögren's syndrome and Raynaud's phenomenon

Autores: Andrés Martínez Perdomo, Julieta Morbiducci, Constanza Arguissain, Marisel Bejarano, María Natalia Tamborenea, Anastasia Secco

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Introduction: Raynaud's phenomenon (RP) is an extraglandular manifestation in primary Sjögren's syndrome (pSS). This phenomenon is a hallmark of systemic sclerosis (SSc).

Objectives: to evaluate the presence of RP in patients with pSS, describe their clinical and immunological characteristics, and determine the development of SSc in patients with pSS and RP.

Materials and methods: patients with pSS and those who presented RP during follow-up were included. Very early systemic sclerosis (VESS) was defined as RP associated with SSc marker autoantibodies and/or a scleroderma-like pattern (SDP) on capillaroscopy.

Results: two hundred and seven patients with a diagnosis of pSS were included. Of these, 32 patients presented both pSS and RP, and 18 of them met criteria for VESS. Among those who met criteria for VESS, 5 developed limited SSc with positive anticentromere antibodies (ACA).

Conclusions: we found a frequency of RP in patients with pSS similar to that described in other series. More than half of the patients with RP and pSS in this cohort met criteria for VESS, with 5 progressing to SSc during a median follow-up of 62 months and positive ACA.