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Background: In systemic sclerosis (SSc) the lack of an angiogenic response to hypoxia may be due to inappropriate synthesis of angiogenic and angiostatic factors. Tissue kallikrein (t-kallikrein), regulating the kallikrein-kinin system and acting on the microcirculation, is a potent angiogenic agent, and kallistatin is its natural inhibitor. Objective: To evaluate, in patients with SSc, t-kallikrein and kallistatin levels and their correlation with clinical features and measures of microvascular involvement. Patients and methods: Serum levels of t-kallikrein and kallistatin (ELISA) and t-kallikrein skin expression (immunohistochemistry) were studied in patients with SSc, and evaluated for subset (dSSc or lSSc), clinical and immunological features, and microvascular involvement (ulcers, telangiectasias, nailfold videocapillaroscopy). Results: Circulating levels of t-kallikrein were higher in SSc than in controls (p

Background. In systemic sclerosis (SSc), digital ulcers (DU) are painful, difficult to heal and frequently infected, thus greatly affecting quality of life and increasing SSc-related disability. Vitamin E has been previously used in cutaneous lesions for its antioxidant and anti-inflammatory effects.Objectives. To study the healing effect of D-alpha-tocopheryl acetate (acetic ester of alpha-tocopherol) (VE) gel on DU of SSc patients..Methods: 27 SSc patients with a total of 86 DU were enrolled in an open pilot study. The patients were randomly assigned to two groups: 15 patients were treated until DU healing with the local standard ulcer care protocol with the application of vitamin E gel (experimental group), while 12 patients were treated with standard ulcer care protocol only (control group). In both groups, DU were treated twice a week and pain was scored by a NRS (numeric rating scale). In both groups the cost of medications was analysed.Results. VE induced a faster healing of DU in respect to controls (13.22+/-2.72 weeks, versus 20.94+/-3.65; p

Objective was to evaluate health-related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using the Short Form 36 (SF-36) and to correlate SF-36 scores with clinical and biologic markers. The SF-36 was administered to 24 controls and 24 SSc patients. SSc patients also were evaluated for subset (limited SSc [lSSc] and diffuse SSc [dSSc]), age, disease duration, angiotensin- converting enzyme (ACE) levels, autoantibodies, and skin and internal organ involvement. The physical summary score (PSS) was lower in SSc patients than in controls (P < 0.05), whereas the mental summary score (MSS) was higher in dSSc than in lSSc patients (P < 0.05). Five of 8 single SF-36 domain scores were lower in SSc patients than in controls (P < 0.05). Vitality was higher in dSSc than in controls (P < 0.001). In SSc, elder age correlated with lower PSS ; low ACE levels and high skin score correlated with higher general mental health and role limitations due to physical problems, respectively (P < 0.05). Patients with heart involvement had higher scores in general health perceptions (P < 0.05). The SF-36 shows that HRQOL is impaired in patients with SSc. Higher scores in MSS and vitality in patients with dSSc and correlations of high SF-36 scores with specific organ involvement suggest that SSc patients with severe disease are more able to cope with HRQOL modification.

In systemic sclerosis (SSc), alterations of the autonomic nervous system are detectable in different organs (cardiovascular and gastrointestinal system) even before the skin involvement becomes evident (prescleroderma) (1). It is still a matter of research whether the neurovegetative alteration may be the trigger of the disease itself or rather the earliest consequence of a vascular derangement of the main internal organs. Nothing is known about the relationship between lung involvement (alveolitis)-one of the earliest and most frequent alterations-and sympathetic activity. In SSc, a significant correlation between indirect indices of cardiac sympathetic activity and lung dysfunction has been found (2). Microneurography evaluates the activity of the autonomic nervous system in awake humans through intraneural recordings of the sympathetic postganglionic vasomotor activity to muscles (MSNA) (3). Although the treatment for SSc is not fully established, cyclophosphamide (CYC), either by oral (4) or pulsed modality (5), has been recently proposed as an effective treatment to control lung alveolitis in SSc. We report the case of a patient affected by early SSc who presented with Raynaud's phenomenon and alveolitis paralleled by an increase in MSNA. The successful control of alveolitis by CYC was followed by a reduction in MSNA and disappearance of Raynaud's phenomenon attacks

ObjectiveAnkylosing spondylitis (AS) is a chronic, progressive, and disabling disease, but the diagnosis is often missed and markedly delayed. An early diagnosis is important to establish a treatment to reduce disability and modify the natural course of disease. The aim of this study was to investigate the diagnostic (DD) and therapeutic (TD) delay according to the decade of diagnosis. The DD and TD correlation with radiological severity score and the new imaging techniques used in diagnosis (magnetic resonance [MRI], computerised tomography, scintigraphy for sacroiliac joints) were also investigated.Methods135 AS patients (45 female and 90 male, 36.5 +/- 10.2 years old at diagnosis) with disease onset between 1950 and 2008, were investigated; the time from onset to diagnosis (DD) and treatment (TD), the New York and ASAS criteria fulfilment, the New York sacroiliac radiological score, bamboo spine presence at first visit and the new imaging technique used at diagnosis were recorded and their correlations were analysed.ResultsThe New York and ASAS criteria were met at the first visit, by 87% and 96%, respectively. The delay from onset of symptoms to diagnosis and treatment was 9 +/- 8 and 12 +/- 11 years, respectively, but decreased significantly between different decades (p0.00.1 and p