Our study describes the prevalence, clinical picture, course and sequelae of Raynaud's phenomenon in patients with primary Sjögren's syndrome. Furthermore, our study compares the clinical, serologic and immunogenetic profile of patients with Raynaud's phenomenon versus those without Raynaud's phenomenon. It seems that Raynaud's phenomenon is a common manifestation (33%) in patients with primary Sjögren's syndrome, precedes sicca manifestations in 42% of patients and follows a pleomorphic course; in some patients (14%) it disappears during the course of Sjögren's syndrome, in others (30%) the frequency of attacks decreases while in a 3rd group (56%) it remains the same. The local clinical sequelae of Raynaud's phenomenon in patients with primary Sjögren's syndrome are swollen hands and evidence of small, soft tissue calcifications on radiographs. Digital pulp ulcers, sclerodactyly or periungal telengiectases are not seen. It seems however, that patients with primary Sjögren's syndrome and Raynaud's phenomenon develop glomerulonephritis, myositis and peripheral neuropathy more often than patients without Raynaud's phenomenon. These differences however, had no statistical significance. Finally, the autoantibody profile is similar in patients with and without Raynaud's phenomenon, while patients without Raynaud's phenomenon and primary Sjögren's syndrome showed a negative but not statistically significant association with the HLA-DR4 alloantigen.