The mortality rate associated with SSc showed a four-fold increase compared with the background population. Lung involvement and sclerodermal renal crisis were found to be independently associated with reduced survival.

The effects of pregnancy on the course of Behçet's disease (BD), and vice versa, are unknown and little has been reported. We have studied three groups of women: (1) group A included 61 pregnancies in 23 women with BD, 25 pregnancies took place in 10 patients already diagnosed (group 1A) and 36 pregnancies occurred in 13 patients before disease diagnosis (group 2A); (2) group B included 30 females with 83 pregnancies affected by recurrent oral ulcers (ROU); (3) group C included 20 healthy women with 61 pregnancies. We investigated the effects of BD on pregnancy and fetal outcome, and the influence of gestation on the course of BD. A questionnaire was used in which specific information about each pregnancy, labour and puerperium was collected. We looked for medical confirmation in all cases where any pathology had been identified. No significant differences were found in the incidence of pregnancy complications between groups. The incidence of perinatal death was also similar and neither congenital abnormalities nor neonatal BD were observed. Only two patients observed a flare of the disease and in two cases the diagnosis of BD was made during the pregnancy. In our series, the outcome of pregnancy was generally good in BD patients, disease manifestations were not consistently worsened and fetal outcome was excellent. The first case of Budd-Chiari syndrome during the puerperium in a BD patient is reported.

Objective. To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques.Methods. Sixty‐three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium‐201 perfusion scintigraphy after a cold‐stress test and radionuclide ventriculography.Results. In the patients with limited SSc, there was a significantly high prevalence of abnormal left‐ and right‐diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold‐stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls).Conclusion. Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.

Objective-To identify survival prognostic factors and markers of morbidity among patients with systemic sclerosis (SSc).Patients and Methods-The study included 72 patients diagnosed with SSc. According to the extent of skin involvement, three groups of patients were established: group 1, without sclerosis and with sclerosis of fingers and neck; group 2, with sclerosis of face and distal to elbows and knees; group 3, with generalised sclerosis including the trunk. All patients were included in a study protocol to determine visceral involvement. Cumulative survival after first symptom has been estimated according to the Kaplan-Meier method. The association between a hypothetical prognostic factor and cumulative survival after first symptom was assessed by log rank test. The association between a hypothetical risk factor and the prevalence of severe morbity was assessed by the odds ratio. Multiple logistic regression models were used to identify the main predictors of severe morbidity. Results-Survival was estimated to be 85% 10 years after first SSc symptom. Survival was higher among SSc patients with skin involvement distal to elbows and knees than among the rest of patients; a forced vital capacity (FVC) on spirometry lower than 70% of expected value was associated with a shorter survival, even after adjustment for diVuse SSc. Skin involvement proximal to elbows or knees was associated with a higher prevalence of severe morbidity (OR = 46.57; p<0.001). According to a multiple logistic regression, severe morbidity was higher among patients with skin involvement proximal to knees or elbows (OR = 40.92; p<0.001) or among patients with pulmonary hypertension detected by Doppler echocardiography (OR = 23.66 p<0.001). Conclusions-In patients with SSc the extent of skin sclerosis was found to be a determining factor on the prognosis. According to skin sclerosis extent two main subsets of SSc patients with diVerent survival incidence and degree of morbidity could be clearly established: limited SSc, formed by patients with no skin sclerosis or with sclerosis distal to elbows and knees and diVuse SSc, formed by patients with skin sclerosis distal and proximal to elbows and knees. Moreover, lung involvement (FVC<70% on survival study and pulmonary hypertension on morbidity study) was an important and independent prognostic factor.

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