Raynaud's phenomenon in primary Sjögren's syndrome. Prevalence and clinical characteristics in a series of 320 patients.
OBJECTIVE
To determine the prevalence of Raynaud's phenomenon (RP) in a large series of patients with primary Sjögren's syndrome (SS) and to identify the clinical and immunological features related to its presence.


METHODS
In a cross sectional study, we investigated 320 consecutive patients with primary SS (294 women, 26 men; mean age at onset 60 yrs, range 16-87 yrs). All patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Diagnosis of RP in patients with SS was defined as intermittent attacks of digital pallor and/or cyanosis in the absence of any other associated disease or anatomical abnormalities.


RESULTS
RP was present in 40 (13%) patients. All were women, with a mean age of 57 yrs (range 18-78). RP preceded onset of sicca symptomatology in 18 (45%) patients. The main triggering factor was exposure to cold, which induced RP in all patients, while emotional stress was a factor in 12 patients, as was job related predisposition in 2. Fifteen (38%) patients required pharmacological treatment with calcium channel blockers (12 patients) or angiotensin converting enzyme inhibitors (2 patients) during colder months, and one patient required treatment with intravenous prostacyclin for ischemic complications. Compared with SS patients without RP, those with RP showed a higher prevalence of articular involvement (50 vs 31%; p = 0.031), cutaneous vasculitis (30 vs 11%; p = 0.003), antinuclear antibodies (95 vs 65%; p < 0.001), anti-Ro/SSA (59 vs 31%; p < 0.001) and anti-La/SSB antibodies (44 vs 20%, p = 0.003).


CONCLUSIONS
We found RP in 13% of patients with primary SS, in almost half of whom RP was the first autoimmune symptomatology. These patients constituted a subset of SS with a higher frequency of some extraglandular features and positive immunological markers. The clinical course of RP seems to be milder in patients with primary SS than in those with other systemic autoimmune diseases such as systemic sclerosis, with no vascular complications and pharmacological treatment needed in only 40% of patients.
García-Carrasco
Mario
M
Department of Systemic Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
Sisó
Antoni
A
Ramos-Casals
Manuel
M
Rosas
José
J
de la Red
Gloria
G
Gil
Victor
V
Lasterra
Susana
S
Cervera
Ricard
R
Font
Josep
J
Ingelmo
Miguel
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000032:analysis; D001324:Autoantigens / Q000097:blood; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D003430:Cross-Sectional Studies; D004247:DNA / Q000276:immunology; D005260:Female; D006801:Humans; D007596:Joints / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D020733:RNA, Small Cytoplasmic; D011928:Raynaud Disease / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D012217:Rheumatoid Factor / Q000097:blood; D012261:Ribonucleoproteins / Q000097:blood; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D000097585:SS-B Antigen; D000099207:SS-A Antigen
D000806:Angiotensin-Converting Enzyme Inhibitors; D000974:Antibodies, Antinuclear; D001324:Autoantigens; D002121:Calcium Channel Blockers; D004247:DNA; D020733:RNA, Small Cytoplasmic; D012217:Rheumatoid Factor; D012261:Ribonucleoproteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen; C116006:RO60 protein, human