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Objective: Systemic sclerosis (SSc) is a multisystemic chronic disease that is complicated by protein-energy malnutrition (PEM). Considering that PEM also may influence left ventricular mass (LVM), the aim of this study was to evaluate whether LVM is related to patients' nutritional status and to determine clinically relevant features of SSc. Methods: Adult patients referring to our institution were considered. Body weight, height, body mass index (BMI), involuntary weight loss, and the presence of gastrointestinal symptoms were recorded. Echocardiography was performed to assess LVM, using the Devereux regression formula. Results were then normalized by body surface area. Pattern, skin thickening, disease activity and severity, and duration were assessed to characterize SSc. Results: Ninety-four patents with SSc (81 women and 13 men; median duration of disease 7 y) were studied. The prevalence of PEM as assessed by BMI < 20 kg/m² was 19%, whereas 15% of patients reported involuntary weight loss of any degree. Patients who lost weight reported gastrointestinal symptoms more frequently (P < 0.05). PEM was not associated with disease activity. LVM (g/m²) correlated with patients' BMI (r = 0.32; P < 0.01), and the vascular domain of disease severity (DDS; r = 0.21; P < 0.05), but it showed a negative correlation with skin thickening (r = -0.21 P = 0.01). Patients with ulcers had a significantly greater LVM than patients without skin lesions. Conclusions: Our study shows that LVM correlates with patients' BMI, skin thickening, and the vascular domain of DSS. Therefore, LVM could serve as a marker of nutritional status and fibrosis in patients with SSc. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate intrarenal arterial stiffness by Doppler ultrasound and examine the correlation between renal Doppler indices, glomerular filtration rate, and digital microvascular damage in systemic sclerosis patients. Methods: Thirty systemic sclerosis patients and 30 healthy controls were enrolled in this study. Doppler indices of intrarenal arterial stiffness, peak systolic flow velocity, end diastolic flow velocity, resistive index, pulsative index, and systolic/diastolic (S/D) ratio were measured on the interlobar artery of both kidneys. Glomerular filtration rate was measured using Tc99m diethylenetriamine pentaacetic acid (DTPA). Equation 7 from the Modification of Diet in Renal Disease was used to estimate glomerular filtration rate. Nailfold videocapillaroscopy findings were classified as early, active, and late patterns. Results: The intrarenal arterial stiffness, evaluated by Doppler indices, was higher in systemic sclerosis patients than healthy controls. In systemic sclerosis patients pulsative index (r = −0.69), resistive index (r = −0.75), and S/D ratio (r = −0.74) showed a negative correlation with measured glomerular filtration rate (P < 0001). High correlation (P = 0008) was observed between measured and estimated glomerular filtration rate (r = 0.55). Pulsative index, resistive index, and S/D ratio significantly increased with progression of capillaroscopic damage. Conversely, measured glomerular filtration rate significantly decreased with capillaroscopic damage progression. Conclusions: Doppler indices of intrarenal arterial stiffness are noninvasive diagnostic tests to evaluate renal damage in SSc patients. Intrarenal arterial stiffness and glomerular filtration rate correlate with capillaroscopic microvascular damage. [Copyright &y& Elsevier]

Abstract: Background: The prevalence of erectile dysfunction (ED) in men with systemic sclerosis (SSc) can be considered a manifestation of endothelium damage. Aim of the study is to investigate ED in SSc patients by color Doppler ultrasound examination and to correlate it with disease severity and digital vascular damage. Methods: In 20 males SSc patients blood flow velocity in the cavernous artery was determined with Duplex ultrasonography. Naifold videocapillaroscopy, Sexual Health Inventory for Men (SHIM) and Medsger Disease Severity Scale (DSS) were performed. Arteriogenic ED was defined by the presence of a reduced peak systolic velocity (PSVs), while diastolic velocity (EDV) and the resistive index (RI) were estimated to evaluate venocclusive dysfunction. SSc patients are classified by capillaroscopic pattern and vascular domain of DSS into two groups: low vascular damage (early or active capillaroscopic pattern and score of vascular domain of DSS≤2) and high vascular damage (late capillaroscopic pattern and score of vascular domain of DSS≥3). Results: In all SSc patients a reduction of SHIM is present (mean 13.5±6.3). Patients with less vascular damage have a significantly (p <0.001) higher score of SHIM than patients with greater vascular damage (19.2±2.4 vs 7.9±2.7). No significant difference (p >0.5) between the two groups of vascular damage was found in PSVs. Venocclusive dysfunction was present only (p <0.001) in the group with high vascular damage. Conclusion: We can assert that there is a relationship between SSc vascular digital damage and ED. [Copyright &y& Elsevier]

Abstract: The prominent or isolated weakness of cervical extensor muscles is a relatively rare clinical sign. Commonly, this is known as “dropped-head syndrome”. This abnormal flexion of the head may occur in a variety of neuromuscular diseases and in a few non-neurological disorders as well. Systemic sclerosis is a clinically heterogeneous disorder which affects small arteries, microvessels and connective tissue with the involvement of multiple organs such as lung, heart, kidney and gastrointestinal tract. There is no evidence in literature of association between dropped head syndrome and rheumatic diseases, particularly systemic sclerosis. The case we describe concerns a 74-year-old woman with dropped-head syndrome associated to Systemic sclerosis and pulmonary hypertension in absence of myositis signs. [Copyright &y& Elsevier]

Résumé: Le déficit musculaire prédominant ou isolé des extenseurs du cou est un signe clinique rare, qui constitue le syndrome couramment appelé « de la tête tombante ». Cette flexion anormale de la tête est généralement secondaire à un trouble neuromusculaire, plus rarement non neuromusculaire. La sclérodermie systémique, dont la présentation clinique est hétérogène, est caractérisée par une atteinte des petites artères, de la microcirculation et du tissu conjonctif qui peut toucher divers organes tels que les poumons, le cœur, les reins ou le tube digestif. À ce jour, il n’existe pas dans la littérature de cas rapportant l’association d’un syndrome de la tête tombante à une maladie rhumatologique, en particulier la sclérodermie systémique. Nous rapportons le cas d’une patiente de 74 ans, présentant un syndrome de la tête tombante associé à une sclérodermie systémique avec hypertension artérielle pulmonaire, sans signe de myosite. [Copyright &y& Elsevier]

Sarcoidosis is a systemic granulomatous disorder of unknown etiology characterized by non-caseating granulomas at the site of disease. A confident diagnosis should be established by the evidence of typical granulomas on biopsy and after exclusion of other conditions. Clinically recognizable Gastrointestinal involvement (GI) occurs in less than 1.6% of patients with sarcoidosis, with data revealing small intestine participation in 0.03% of the cases and few anecdotal reports describe a peritoneal presentation. Clinical manifestations of peritoneal sarcoidosis are abdominal discomfort, bloating, weight loss, epigastric and peri-umbilical pain with or without ascites, bowel obstruction. Treatment depends on symptoms and disease activity. Herein we describe the case of a 42-years-old male patient who developed an acute, life–threatening small bowel obstruction as first manifestation of sarcoidosis. To the best of our knowledge, this is the only report showing such extensive and acute onset of intra-abdominal sarcoidosis in the absence of a previous disease manifestation and without pulmonary involvement. • Abdominal sarcoidosis is a sporadic event and localization without pulmonary involvement is rarer mimicking GI diseases. • Reaching abdominal sarcoidosis diagnosis is not a straightforward process. • It is pivotal to confirm sarcoidosis diagnosis before subjecting patients to an appropriate treatment. • Corticosteroids are considered the cornerstone treatment. • Asymptomatic patients may undergo a watch and wait follow-up with no need for a pharmacological therapy. • Surgery is not often required but individualized patient evaluation is recommended. [ABSTRACT FROM AUTHOR]

Abstract: Diesel exhaust particles (DEP) are major constituents of ambient air pollution and are associated with respiratory and cardiovascular diseases as well skin cell alterations in vitro. The epidermal cells are among the first cell populations exposed to chemical pollutants, including DEP, and are an important source of pro-inflammatory mediators. We evaluated the effects of carbonaceous soot particles from current low-emission (Euro IV) diesel engines on the oxidative and inflammatory response of normal human skin cells and compared the results with those induced by carbonaceous soot particles from an older diesel engine (BS) operating under black smoke conditions. We observed that both soot nanoparticles were spontaneously internalised by keratinocytes and distributed mostly around the cell nucleus. Moreover, at the same mass concentration, Euro IV soot particles exhibited a much higher oxidative, pro-fibrotic and toxic potential on these cell types than soot particles from the older diesel engine. These results are in agreement with and confirm our previous findings on human macrophage cells and strengthen the assumption that, at the same mass concentration, soot particles produced under low emission conditions are more cytotoxic than particles from the older diesel engine. This effect could be assigned to the defective surface structure of Euro IV diesel soot, rendering it highly active. Our findings highlight that the reduction of soot emission in terms of mass does not automatically lead to a reduction of the dangerous effects and show that soot particles from different diesel engines possess different biological behaviour towards human cells. [Copyright &y& Elsevier]