Cardiovascular Risk and Quality of Life in Autosomal Dominant Polycystic Kidney Disease Patients on Therapy With Tolvaptan: A Pilot Study.

Introduction: Cardiovascular (CV) complications are the most frequent cause of morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD) patients. In 2017, the Italian Medicines Agency authorised tolvaptan, a vasopressin V2 receptor antagonist, for the treatment of ADPKD, based on the Tolvaptan Phase 3 Efficacy and Safety Study in ADPKD (TEMPO 3: 4), TEMPO 4: 4 and Replicating Evidence of Preserved Renal Function: An Investigation of Tolvaptan Safety and Efficacy (REPRISE) studies.

Aim Of The Study: The aim of the study was to assess the impact of tolvaptan on CV risk and quality of life, evaluated by nutritional, inflammatory, metabolic, instrumental parameters and psychocognitive tests on ADPKD patients.

Left Ventricular Mass Index as Potential Surrogate of Muscularity in Patients With Systemic Sclerosis Without Cardiovascular Disease.

Background: Systemic sclerosis (SSc) may be associated with protein-energy malnutrition. It was demonstrated that left ventricular mass (LVM) reflected poor nutrition status in SSc patients, and no data are available on LVM as a surrogate measure of muscularity. We aimed to evaluate, in SSc patients, the relationship between muscle mass and LVM as a novel indicator of muscularity in this setting.

Reduction of fat free mass index and phase angle is a risk factor for development digital ulcers in systemic sclerosis patients.

Introduction/objectives: This study aims to evaluate the role of fat free mass index (FFMI) and phase angle (PhA) as markers to predict occurrence of new digital ulcers in systemic sclerosis (SSc) patients.

Methods: Body composition evaluation from bioelectrical impedance and clinical assessment were performed in SSc patients at enrollment and after 12 months follow-up.

Results: Seventy-nine SSc patients (67 female) with a mean age of 53 ± 13 years were enrolled.

Assessing Malnutrition in Systemic Sclerosis With Global Leadership Initiative on Malnutrition and European Society of Clinical Nutrition and Metabolism Criteria.

Background: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs. Gastrointestinal tract (GIT) involvement may lead to malnutrition, which can in turn negatively affect morbidity, mortality, and quality of life. The aim of the study was to assess the prevalence of malnutrition in SSc patients with both the European Society of Clinical Nutrition and Metabolism (ESPEN) and the Global Leadership Initiative on Malnutrition (GLIM) criteria and to assess whether it relates with disease activity and severity.

Correction to: Phase angle in systemic sclerosis: a marker for pulmonary function and disease severity.

The name of the author of the original published version of this article was presented incorrectly. The author name "Antonietta Gigantea" should have been presented as "Antonietta Gigante". This has been correctly presented above.

Phase angle could be a marker of microvascular damage in systemic sclerosis.

Objectives: Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction with fibrosis of skin and internal organs. Integrity of the endothelial cell is important to its physiologic function such as production of angiogenetic factors. The aim of this study was to assess whether phase angle (PhA) is altered in patients with SSc and whether its values correlate with vascular endothelial growth factor (VEGF) and digital microvascular damage.

Late Gadolinium Enhancement in Cardiac Magnetic Resonance Imaging Is Associated with High Renal Resistive Index in Patients with Systemic Sclerosis.

Introduction: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by microvascular damage and fibrosis of the skin and internal organs. The major complications are lung fibrosis, pulmonary artery hypertension, scleroderma renal crisis, and cardiac involvement.

Objective: The aim of this study was to assess renal and cardiac involvement in asymptomatic SSc patients using renal Doppler ultrasound (RDU) and cardiac magnetic resonance (CMR).

Assessment of interstitial lung disease in systemic sclerosis using the quantitative CT algorithm CALIPER.

Interstitial lung disease (ILD) remains a major cause of morbidity and mortality in systemic sclerosis (SSc). Study aim is to characterize and quantify SSc-ILD by using Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER). Secondly, our objective is to evaluate which radiological pattern is predictive of lung function decline at 12 months follow-up.

Atrial natriuretic peptide predicts disease progression and digital ulcers development in systemic sclerosis patients.

Aims: Systemic sclerosis (SSc) is an autoimmune disease characterized by micro/macrovascular damage due to the underlying fibrosis. Markers able to predict the progression of cardiovascular damage, including digital ulcers, in SSc are warranted. We aimed at characterizing the relevance of N-terminal proatrial natriuretic peptide (NT-proANP) and N-terminal probrain natriuretic peptide plasma levels in relation to cardiovascular damage and digital ulcers in a cohort of Italian SSc patients.

The predictive role of lung ultrasound in progression of scleroderma interstitial lung disease.

Lung ultrasound (LUS) correlates with chest high-resolution computed tomography (HRCT) findings in the diagnosis of interstitial lung disease (ILD) in systemic sclerosis (SSc). The aim of this study is to evaluate the predictive value of LUS in the progression of ILD. At baseline, forty-one SSc patients underwent HRCT, LUS for detection of B-lines, and pulmonary function test (PFTs).

Renal Parenchymal Thickness in Patients with Systemic Sclerosis Is Related to Intrarenal Hemodynamic Variables and Raynaud Renal Phenomenon.

Objective: Renal involvement in systemic sclerosis (SSc) ranges from urinary abnormalities, reduction of glomerular filtration rate, and high renal resistive index, to scleroderma renal crisis. Intrarenal resistance indices are considered markers of renal SSc-associated vasculopathy. The aim of this study is to evaluate renal morphological variables, such as renal length, parenchymal thickness, atrophy index, and renal sinus in patients with SSc and to correlate it with renal function and hemodynamic variables.

Prognostic Factors of Renal Involvement in Systemic Sclerosis.

Background/aims: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years.

Methods: 121 SSc patients (100 F, 21 M) with mean age of 54.

Serum level of endostatin and digital ulcers in systemic sclerosis patients.

Patients with systemic sclerosis (SSc) are at a high risk of the development of ischaemic digital ulcers (DUs) that can be complicated with infections, gangrene, and osteomyelitis. The aim of this study is to evaluate the role of endostatin in scleroderma DUs.In total, 90 SSc patients were enrolled in this study.

In systemic sclerosis skin perfusion of hands is reduced and may predict the occurrence of new digital ulcers.

Systemic sclerosis (SSc) patients are at high risk for the development of ischemic digital ulcers (DUs). The aim of this study was to assess in SSc patients a correlation between skin perfusion evaluated by LDPI and DUs and to evaluate the prognostic value of skin perfusion to predict the new DUs occurrence. Fifty eight (47 female, 11 male) SSc patients were enrolled.

Left Ventricular Mass and Intrarenal Arterial Stiffness as Early Diagnostic Markers in Cardiorenal Syndrome Type 5 due to Systemic Sclerosis.

Background: Cardiorenal syndrome type 5 (CRS-5) includes a group of conditions characterized by a simultaneous involvement of the heart and kidney in the course of a systemic disease. Systemic sclerosis (SSc) is frequently involved in the etiology of acute and chronic CRS-5 among connective tissue diseases. In SSc patients, left ventricular mass (LVM) can be used as a marker of nutritional status and fibrosis, while altered intrarenal hemodynamic parameters are suggestive of early kidney involvement.

Serum uric acid as a marker of microvascular damage in systemic sclerosis patients.

Background: Microvascular damage of skin and internal organs is a hallmark of systemic sclerosis (SSc). Serum uric acid (UA) represents a marker of inflammation and endothelial dysfunction. The aims of this study were to evaluate the correlation between serum UA and intrarenal arterial stiffness evaluated by Doppler ultrasound in SSc patients with normal renal function.

Drugs and Rhabdomyolysis: From Liver to Kidney.

Rhabdomyolysis is a syndrome due to a damage of skeletal muscle and the leakage of intracellular contents into the extracellular fluid and the circulation. Several causes may induce rhabdomyolysis and the major one is the crush syndrome. Most cases of non-traumatic rhabdomyolysis are related to drugs.

Prevalence and clinical features of patients with the cardiorenal syndrome admitted to an internal medicine ward.

Background: Many patients admitted to a Department of Internal Medicine have different degrees of heart and kidney dysfunction. Mortality, morbidity and cost of care greatly increase when cardiac and renal diseases coexist.

Methods: A retrospective cohort study was conducted on 1,087 patients admitted from December 2009 to December 2012 to evaluate the prevalence of the cardiorenal syndrome (CRS) and clinical features.

Rhabdomyolysis after midazolam administration in a cirrhotic patient treated with atorvastatin.

The administration of statins in patients with liver disease is not an absolute contraindication. Hepatotoxicity is a rare and often dose-related event and in the literature there are only a few described cases of fatal rhabdomyolysis in patients with chronic liver disease after statin administration. During treatment with 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors, the factors responsible for myopathy may either be related to the patient, or due to interactions with other medications that are metabolic substrates of the same isozymes and therefore able to increase blood statin concentration.

Pathophysiology, diagnosis and clinical management of hepatorenal syndrome: from classic to new drugs.

Advanced cirrhosis is frequently associated with renal dysfunction. Hepatorenal syndrome (HRS) is characterized by the occurrence of kidney injury in cirrhotic patients in the absence of other identifiable causes. HRS is classified in 2 different types.

Nutritional status measured by BMI is impaired and correlates with left ventricular mass in patients with systemic sclerosis.

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.