Incidence and prevalence of systemic sclerosis in Valcamonica, Italy, during an 18-year period.

Objectives: To investigate the epidemiology of systemic sclerosis in Valcamonica, an Italian Alpine valley, during an 18-year-long period. Methods: Patients with systemic sclerosis living in Valcamonica between 1999 and 2016 were identified by capture/recapture method using: (1) clinical databases of the only secondary Rheumatology Unit present in the valley and of the tertiary referral center for this area; (2) administrative data, extracting records with the International Classification of Diseases, 10th Revision, code for systemic sclerosis. Patients were included in the analysis when either the 1980 American Rheumatism Association classification criteria for systemic sclerosis or the 2013 American College of Rheumatology/European League Against Rheumatism criteria were satisfied. To study temporal changes, mean yearly incidence during three different 6-year interval was calculated. Prevalence rates were estimated at four different time points. Results: General population with age over 14 years living in Valcamonica varied during the evaluated period between 85,168 and 91,245 inhabitants. A total of 65 patients with systemic sclerosis were identified (female 84.6%, limited cutaneous systemic sclerosis: 84.6%; anticentromere: 64.6%). Systemic sclerosis incidence and prevalence increased during the study period (p = 0.029 and p < 0.0001, respectively). The increase of incidence was accounted for by cases satisfying only the 2013 criteria, with limited cutaneous systemic sclerosis, and with anticentromere, whereas the incidence of systemic sclerosis cases classified according to the 1980 criteria did not significantly increase. The prevalence at 31 December 2016 was 58.6 (95% confidence interval, 44.8-76.6) per 100,000 persons aged >14 years. Survival at 10 years after systemic sclerosis diagnosis was 83.0% (standard error, 5.6). Conclusion: Systemic sclerosis incidence and prevalence increased over time in this area, due to the increased recruitment of patients with milder forms of the disease.

Systemic vasculitis and pregnancy: a multicenter study on maternal and neonatal outcome of 65 prospectively followed pregnancies.

OBJECTIVE: Systemic vasculitis (SV) are uncommon diseases that rarely affect women during their reproductive age; little data, mainly retrospective, is available on this topic. The aim of our study was to evaluate maternal/neonatal outcome and disease course before, during and after pregnancy. METHODS: Sixty-five pregnancies in 50 women with SV were followed by a multispecialistic team in 8 institutions between 1995 and 2014. Clinical data on pregnancy, 1year before and 1year after delivery was retrospectively collected. The rate of pregnancy complications was compared to that of a General Obstetric Population (GOP) of 3939 women. RESULTS: In 2 patients the diagnosis of SV was done during pregnancy; 59 out of the remaining 63 started when maternal disease was quiescent. We recorded 56 deliveries with 59 live births, 8 miscarriages and 1 fetal death. In SV, preterm, particularly early preterm (<34weeks) deliveries and cesarean sections appeared significantly more frequent than in GOP (11.3% vs 5.0%, p=0.049 and 48.2% vs 31.0%, p=0.009). Vasculitis-related complications occurred in 23 pregnancies (35.4%), with 5 severe events (7.7%) including 3 cases of transient ischemic attack (TIA). Data about the post-partum period were available for 56 pregnancies: 12 flares (21.4%) occurred, with 1 severe event (1.8%). CONCLUSION: SV patients can have successful pregnancies (especially during a disease remission phase) despite an increased rate of preterm delivery. Severe flares were limited, but the occurrence of 3 TIA suggests that particular attention should be given to possible thrombotic complications in SV patients during pregnancy and puerperium.

Evaluation of mortality, disease activity, treatment, clinical and immunological features of adult and late onset systemic Lupus erythematosus.

OBJECTIVES: We retrospectively compared disease activity, treatment, clinical and laboratory features, and rate of mortality of 535 SLE patients with adult and late disease onset. METHODS: patients were divided into two groups based on the onset of the disease before or after 50 years of age. Clinical data were collected from medical reports. Disease activity was measured by ECLAM score. Parameters were compared by χ²-test, Fisher's test, Student's t or the Mann-Whitney test. RESULTS: Forty patients (7.5%) were included in the late SLE onset group (group A), while 495 (92.5%) in the adult SLE onset group (group B). Sicca symptoms were more frequent in group A (p < 0.0008), while glomerulonephritis (p < 0.0069), reduced C3 (p < 0.0006) and low C3 (p < 0.00002) and C4 levels (p < 0.0006) were more prevalent in group B. Twenty-two deaths (4.3%) were recorded: 14 (2.8%) in group B and 8 (20%) in group A. Deaths were mainly due to infections in group B (28.5%) and cardiovascular events in group A (50%). A lower use of HCQ and LDA were recorded in deceased versus living patients (p < 0.0001 and 0.0166, respectively), while a higher ECLAM score was measured at onset in dead versus living patients (p < 0.048). CONCLUSIONS: Late onset SLE occurred in 7.5% of patients and it was associated with sicca symptoms. The use of HCQ and LDA is positively correlated with survival. Death in late onset SLE occurred more frequently for cardiovascular involvement. Higher disease activity at onset of the disease might represent a poor prognostic factor for death in adult onset.

Atherosclerosis and rheumatoid arthritis: more than a simple association.

In the last decades a large amount of evidence linked rheumatoid arthritis (RA) to atherosclerosis. In fact, RA patients have an increased risk of cardiovascular events that is not fully explained by other classic cardiovascular risk factors. RA and atherosclerosis may share several common pathomechanisms and inflammation undoubtedly plays a primary role. The proinflammatory cytokines such as tumor necrosis factor alpha and interleukin-6, involved in the pathogenesis of RA, are also independently predictive of subsequent cardiovascular disease (CVD). In RA, inflammation alters HDL constituents and the concentration of LDL and HDL, thus facilitating atherosclerosis and CVD events. On the other hand, also the increase of oxidative processes, frequently observed in RA, induces atherosclerosis. Interestingly, some genetic polymorphisms associated with RA occurrence enhance atherosclerosis, however, other polymorphisms associated with RA susceptibility do not increase CVD risk. Several other mechanisms may influence atherosclerotic processes in RA. Moreover, atherosclerosis may be directly mediated also by underlying autoimmune processes, and indirectly by the occurrence of metabolic syndrome and impaired physical activity. Finally, the effects of RA therapies on cardiovascular system in general and on atherosclerosis in particular are really wide and different. However, the starting point of every RA treatment is that disease control, or better remission, is the best way we have for the reduction of CVD occurrence.