Background Studies evaluating the difference between men and women diagnosed of scleroderma (SSc) are scarce (1-5) because of the shortage of male patients.
Objectives Assessing the existence of differences in clinical presentation and mortality causes among men and women in a large cohort of Spanish patients diagnosed with SSc.
Methods A registry of patients with SSc has been performed by the Spanish network for systemic sclerosis (RESCLE) since 2008, in which 90 clinical, immunological and capillaroscopic variables were collected prospectively. Data were collected until May 2014 for this study.
Results 1506 patients (1341 women), were included. Overall ratio female/male was 8/1. By subtypes lcSSc and preSSc were more frequent in females [84 (51%) vs 808 (60%), p0.023; 6 (3.6%) vs100 (7.5%), p0.076, respectively] and dcSSc in males [62 (38%) vs 293 (22%), p<0.001]. Diagnostic delay was longer in women (2.92 yrs vs 1.30yrs, p<0.001) and smoking was more prevalent in men. Myositis, tendon rubs, interstitial lung disease (ILD), cardiac conduction disturbances and renal crisis were more frequent in males, while isolated pulmonary arterial hypertension (PAH) and sicca syndrome in women. Positivity for ANA, ACA and antiRo was more frequent in women and antiScl-70 and antiRNA pol III in men. There were no sex differences in capillaroscopic patterns. In the multivariate study remained significant in the male predominance in smoking (OR 2.33, CI 1.49 to 4.14, p=0.002) and ILD (OR 1.70, CI 1.11 to 2 60, p=0.015) and in women sicca syndrome (OR 3, 1.63 to 5.52) and ANA positivity (OR 2.67, 1.29 to 5.51).Follow-up time (years) since the onset of symptoms was in males 8.69 yrs vs women 11.79, p=0.001. Altogether, 261 patients died (17%). Although there was no difference in the age at death, this was more common in men (31% men vs 15% women), and time from the onset of symptoms to death was longer in women [14.06 yrs (5.95 to 24.29) vs 8.33 (3.87 to 12.66), p<0.001]. Death was related to SSc in 112 (46%) (22 males vs 90 in women, p ns) while 129 (54%) (31 males vs. 103 for females, p ns) were due to other causes. ILD was the most common cause of death in men (27,5%) and PH in women (28,6%). PAH was more frequently a cause of death in women 39 (19,9%) than in men 0 (0,0%), p<0,001. The cumulative survival at 20 years from onset was 90.9% in women versus 76.3% in men, p<0.001.
Conclusions 1. In the RESCLE cohort, smoking and ILD were more frequent in males, and sicca syndrome and ANA positivity in women. 2. ILD was the most common cause of death in men and PAH in women. 3. PAH was more frequently a cause of death in women than in men. 4. Men had less survival since the onset of symptoms.
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Disclosure of Interest None declared