Inaugural raynaud's phenomenon is associated with a multisystemic involvement during follow-up in primary sjögren's syndrome—A report of 162 patients

Abstract

Studies of Raynaud's phenomenon occurring in the course of primary Sjögren's syndrome are sparse and contradictory. In this retrospective study, our aim was to determine prevalence, characteristics, and clinical associations of Raynaud's phenomenon (RP) in a group of patients with primary Sjögren's syndrome (pSS), according to revised European criteria, in order to detect, at the time of diagnosis, patients with a potentially worth systemic evolution. One hundred sixty-two patients with pSS were evaluated for the presence (48%, n = 77) or absence of RP. Clinical course of RP was usually mild and caused no vascular lesions. RP was present in 42% (n = 68) at the time of pSS diagnosis. Nail-fold microscopy performed in 89 patients (55%) showed microangiopathy evocative of systemic sclerosis (SS) in ten cases. None presented clinical aspects of SS during follow-up (median 68 months). Nonerosive arthritis, cutaneous vasculitis lesions, myositis, and renal manifestations were significantly more frequent in patients with RP (p < 0.05), than were anti-Sa, anti-SSb, rheumatoid factor, and cryoglobulinemia (p < 0.05). If the RP clinical course is usually mild in pSS, this subgroup of patients should be carefully monitored because of the occurrence of extraglandular manifestations.