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). MATERIALS AND METHODS: We enrolled 26 consecutive SSc patients (21 female) according to 2013 ACR/EULAR criteria. Biochemical analysis, clinical evaluation, RDU with intrarenal hemodynamic parameters (renal resistive index [RRI], pulsatility index [PI], systolic/diastolic [S/D] ratio), and CMR with late gadolinium enhancement (LGE) were investigated at the time of enrollment. RESULTS: The median PI value was significantly (p = 0.007) higher in SSc patients with LGE than in SSc patients without LGE (1.37 [1.28-1.58] vs. 1.12 [1.06-1.26]). The median RRI value was significantly (p = 0.002) higher in SSc patients with LGE than in SSc patients without LGE (0.68 [0.65-0.73] vs. 0.64 [0.63-0.65]). The median S/D ratio was significantly (p = 0.02) higher in SSc patients with LGE than in SSc patients without LGE (3.12 [2.83-3.76] vs. 2.78 [2.64-2.84]). CONCLUSIONS: Our study, although performed on a small SSc population, showed RRI and LGE as markers of vascular and fibrotic damage. Early detection of cardiorenal involvement in SSc patients without symptoms is important to avoid further complications.

Hypervirulent Klebsiella pneumoniae in Cryptogenic Liver Abscesses, Paris, France.

Liver abscesses containing hypervirulent Klebsiella pneumoniae have emerged during the past 2 decades, originally in Southeast Asia and then worldwide. We hypothesized that hypervirulent K. pneumoniae might also be emerging in France. In a retrospective, monocentric, cohort study, we analyzed characteristics and outcomes for 199 consecutive patients in Paris, France, with liver abscesses during 2010-2015. We focused on 31 patients with abscesses containing K. pneumoniae. This bacterium was present in most (14/27, 52%) cryptogenic liver abscesses. Cryptogenic K. pneumoniae abscesses were more frequently community-acquired (p<0.00001) and monomicrobial (p = 0.008), less likely to involve cancer patients (p<0.01), and relapsed less often (p<0.01) than did noncryptogenic K. pneumoniae liver abscesses. K. pneumoniae isolates from cryptogenic abscesses belonged to either the K1 or K2 serotypes and had more virulence factors than noncryptogenic K. pneumoniae isolates. Hypervirulent K. pneumoniae are emerging as the main pathogen isolated from cryptogenic liver abscesses in the study area.

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 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled. All patients were screened annually for renal function by laboratory examination, ultrasound and color Doppler ultrasound of renal arteries. RESULTS: Over the five-year observation period, 6 SRC (3 M, 3 F) occurred, four of which required dialysis. One patient developed ANCA-related proliferative glomerulonephritis and the other one acute tubular necrosis. The remaining 113 patients had a preserved renal function (serum creatinine 0.75 ± 0.24 mg/dl, GFR 93.8 ± 20 ml/min, 24h proteinuria 0.20 ± 0.15 g). Doppler indices of intrarenal arterial stiffness increased with progression of capillaroscopic damage and with presence of digital ulcers. A negative correlation was observed between estimated GFR and pulsatile index (p< 0,05, r=-0.198), resistive index(p< 0,01, r=0.267), S/D ratio (p< 0,01, r=-0.237). CONCLUSION: In SSc patients, renal function was normal for 4.1 years despite the presence of increased intrarenal arterial stiffness. SRC was observed in 4.9% of SSc patients. In SSc patients, a periodic follow-up based on clinical and laboratory evaluation, colorDoppler ultrasound and, in some cases, renal biopsy is required to evaluate renal involvement.

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. Skin perfusion of hands and region of interest (ROIs) was measured by Laser Doppler perfusion Imager (LDPI). The proximal-distal gradient (PDG) was present when the perfusion mean difference between ROI1 and ROI2 was >30 pU. The skin perfusion of hands is lower in SSc patients than in healthy controls. The skin perfusion decreased with severity of capillaroscopic damage. Both mean perfusion of hand and PDG are significantly (p<0.01 and p<0.0001, respectively) lower in SSc patients with new DUs than in SSc patients without DUs. Only 2 of 11 SSc patients (18.2%) with PDG developed new digital ulcers, conversely 36 of 47 (76.6%) SSc patients without PDG developed new digital ulcers (p<0.001). The ROC curves demonstrated a good accuracy of new DUs prediction for PDG (0.78, p<0.0001). Using this cut-off value of 30 pU, RR for new DUs development in SSc patients without PDG is 4,2 (p<0.001). LDPI indices could be used in association to the capillaroscopic and clinical findings or serological tests in the identification of patients at high risk of developing DUs.

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. We also evaluated the correlation between serum UA and other clinical variables of the disease. METHODS: Forty-five SSc patients underwent clinical assessment, Doppler ultrasound of intrarenal arteries with evaluation of resistive index (RI), pulsatile index (PI), and systolic/diastolic ratio (S/D), echocardiography with systolic pulmonary artery pressure (PAPs), baseline pulmonary function tests, and nailfold videocapillaroscopy (NVC). In all patients serum UA was measured. RESULTS: The serum UA showed a significant positive correlation with sCr (r=0.33, p<0.0001) and PAPs (r=0.38, p<0.01) >and negative correlation with CKD-EPI (r=-0.35, p<0.01). The mean value of serum UA increased with severity of NVC damage. Using this cut-off value of 4.7mg/dl, the mean value of Doppler indices of intrarenal stiffness is significantly different (p<0.05) in SSc patients with low normal or high normal serum UA. CONCLUSIONS: Serum UA concentration is higher in patients with high microvascular damage than in patients with low microvascular damage. These preliminary data must be confirmed in large prospective studies.

Small hepatic veins Budd-Chiari syndrome.

Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio-caval junction, in the absence of heart failure or constrictive pericarditis. Various imaging modalities are available for investigating the gross hepatic vascular anatomy but there are rare forms of this disease where the obstruction is limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this cases only a liver biopsy can demonstrate the presence of a small vessels outflow block. We report two cases of small hepatic veins Budd-Chiari syndrome.

Gabexate mesylate as treatment in the course of ANCA-negative microscopic polyangiitis.

Patients with small vessel vasculitis present fluctuating antineutrophil cytoplasmic antibodies (ANCA) levels to the point that positive ANCA may be missed even if only up to 10% of patients with microscopic polyangiitis (MPA) are ANCA-negative. The first-line treatment of MPA is the association of steroids and cyclophosphamide, especially in the presence of a rapidly progressive glomerulonephritis. Plasmapheresis, intravenous immunoglobulins, and tumor necrosis factor inhibitors have been proposed as alternative to standard therapy. Disseminated intravascular coagulation (DIC) is a possible event in the course of small vessel vasculitis. Gabexate mesylate is a protease inhibitor able to suppress endothelial cell injury, and it may be administered to treat DIC related to different diseases. In ANCA-associated vasculitis, cytokines play a key role in promoting endothelial damage. DIC-related thrombocytopenia may be misinterpreted as drug-induced because of the immunosuppressive properties of cyclophosphamide. Two cases of ANCA-positive MPA associated with DIC and treated with gabexate are reported in the literature with improvement of both hematological disorder and renal function. Our patient presented a rapidly progressive glomerulonephritis, and the renal biopsy showed MPA, in the absence of ANCA. After two weeks of steroid treatment, our patient developed a DIC. This case represents the first report of ANCA-negative MPA managed with gabexate, which showed improvement of coagulation disorders and kidney function. In conclusion, the anti-inflammatory properties of gabexate could be helpful in MPA at increased bleeding risk when immunosuppressive treatment is contraindicated, even in ANCA-negative vasculitis.

Ask-Upmark kidney and tubulointerstitial nephritis in a woman with severe renal failure.

Ask-Upmark kidney is a rare diagnosis of segmental hypoplasia in pediatric population clinically characterized by severe hypertension potentially treatable with partial to total nephrectomy. Although originally was described only as a congenital anomaly, recent data suggest to be caused by vesicoureteral reflux, either in utero or in early childhood and pyelonephritis. The case we reported indicates that Ask-Upmark kidney should be considered as potential cause of hypertension and renal failure both in children and adults. The renal biopsy is necessary for early diagnosis and may consent to normalize blood pressure with nephrectomy; however, if renal damage is severe and progressive with tubulointerstitial nephritis, surgical management is excluded and renal transplant should be considered.

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. METHODS: SSc patients and healthy controls (HCs) were considered and underwent echocardiography to assess LVM and LVM index (LVMI). Body composition, including fat-free mass index (FFMI), phase angle (PhA), and body cell mass index (BCMI), was analyzed by multifrequency bioelectrical impedance analysis. RESULTS: Sixty-four SSc patients (aged 51 ± 13 years) and 30 HCs (aged 46 ± 13 years) were enrolled. BCMI and PhA were lower in SSc patients compared with HCs (P < .001). LVM and LVMI were not different between SSc patients and HCs (P = .068, P = .14, respectively). In SSc patients, a significant correlation was shown between LVMI and FFMI (τ = 0.192, P = .026); in multiple regression analysis, FFMI was significantly associated with the Malnutrition Universal Screening Tool (P < .0001), LVMI (P = .01), and disease severity scale (P = .02). CONCLUSION: Changes in body composition were present in SSc patients, particularly in terms of BMCI and PhA. A correlation between LVMI and FFMI as indexes of muscularity exists in this cohort, suggesting that cardiac mass might be used as a surrogate of nutrition status in this setting.

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. METHODS: Adult SSc patients were included in the study. Biochemical analyses, disease activity index (DAI), disease severity scale (DSS), anthropometric data, and body composition parameters for GLIM and ESPEN assessment were recorded at enrollment. RESULTS: One hundred and two SSc patients were enrolled (86 females, mean age 55 ± 14 years). The prevalence of malnutrition was 8.8% (9 of 102), according to ESPEN, and 16.6% (17 of 102), according to GLIM criteria. GLIM severity grading of malnutrition was moderate in 12.7% (13 of 102) and severe in 3.9% (4 of 102) . In SSc patients with malnutrition according to GLIM criteria, DAI and DSS were significantly higher than in SSc patients without malnutrition (P < .0001), whereas no association was observed between malnutrition and DAI or DSS when using the ESPEN criteria. CONCLUSION: Prevalence of malnutrition is higher with GLIM than with ESPEN criteria. Irrespective of the method used, prevalence of malnutrition in SSc is lower than one would expect in a chronic, autoimmune disease with GIT involvement.

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). PFTs were performed also after 12 months to evaluate pulmonary function deterioration. In multiple regression analysis, positive correlation exists between the number of B-lines and HRCT score (r = 0.51, p < 0.05), conversely a negative correlation exists between number of B-lines and carbon monoxide diffusing capacity (DLCO) (r = - 0.49, p < 0.05) and FVC (r = - 0.42, p < 0.05). The number of B-lines significantly (p < 0.05) increased with progression of digital microvascular damage. At 12 months, a positive correlation exists between number of B-lines and delta of DLCO. The ROC curves demonstrated a good accuracy of worsened DLCO prediction for Delta DLCO (0.72, p < 0.05 95% CI 0.56-0.88). The sum of B-lines correlates with the radiological score evaluated by HRCT. We also demonstrate that the number of B-lines can predict the worsening of the ILD. Although HRCT is the gold standard technique for assessing ILD, the LUS could become a useful tool for guiding the use of HRCT.Key Points• In systemic sclerosis patients, the number of B-lines can predict the worsening of the interstitial lung disease• High-resolution computed tomography of chest is the gold standard technique for assessing interstitial lung disease• Lung ultrasound could become a useful tool for guiding the use of high-resolution computed tomography of chest.

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. METHODS: There were 92 patients with SSc and 40 healthy controls (HC) enrolled in this study. Doppler and renal ultrasound (US) including renal length, parenchymal thickness, atrophy index, renal sinus, and intrarenal resistive index were measured in patients with SSc and HC. RESULTS: Renal US showed significant differences between HC and patients with SSc. The renal length (mm; 106.7 ± 5.1 vs 102.3 ± 8.4) and renal sinus (70.7 ± 7.9 vs 65.3 ± 7.7 mm) were significantly (p = 0.001) higher in HC than patients with SSc. The parenchymal thickness was significantly (p = 0.004) higher in HC than patients with SSc (18 ± 3.1 vs 16.3 ± 2.5 mm). Pulsatility index, resistive index, and systolic/diastolic ratio were significantly (p < 0.0001) lower in HC than patients with SSc. The renal length was significantly (p = 0.004) higher in diffuse cutaneous SSc (105 ± 8.4) than in limited cutaneous SSc (99.5 ± 7.5). CONCLUSION: In SSc, kidney involvement is subclinical and is related to vascular injury, Raynaud phenomenon, and chronic hypoxia that can modify renal morphology. Serum creatinine is a poor marker of renal damage, and renal US could be a useful tool - together with Doppler - to evaluate renal involvement in a systemic and chronic disease such as SSc.

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. In SSc patients with a digital ulcers history, FFMI value is lower (p < 0.05) and phase angle (PhA) value is higher (p < 0.01) than SSc patients without a digital ulcers history. After 12 months of follow-up, 30 patients (38%) presented at least one new episode of digital ulcers. Patients with reduced FFMI had a relative risk of 6.7 for new digital ulcers (CI 2.1-21.8, p < 0.001). Patients with reduced PhA had a relative risk of 10.1 for new digital ulcers (CI 3.5-29.5, p < 0.0001). In multivariate analysis, FFMI and PhA were associated with major vascular complication (digital ulcers, pulmonary arterial hypertension, and scleroderma renal crisis). FFMI loss, assessed as delta between follow-up and baseline, is higher in SSc with short duration (≤ 3 years) than SSc patients with long duration [0.4 (0-0.50) vs - 0.10 (- 0.2-0)]. CONCLUSION: In SSc patients, reduction of the FFMI and PhA represents after 12 months a risk factor for development of new digital ulcers and major vascular complication. Key Points • Fat free mass index represents a risk factor for development of digital ulcers • Phase angle represents a risk factor for development of digital ulcers • Body compositions in systemic sclerosis are a marker of activity disease.

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. METHODS: Patients with SSc and matched healthy controls underwent VEGF determination and bioimpedentiometry (BIA) for PhA assessment. Clinical assessment, disease activity index (DAI), disease severity scale, and nailfold videocapillaroscopy (NCV) were performed in patients with SSc. RESULTS: Fifty-five patients (46 women) with a mean age of 53.2 ± 13.7 y were studied. The mean value of VEGF was significantly higher in patients with SSc than in the healthy controls (240.3 ± 149.5 versus 139 ± 87.5; P = 0.035). The mean value of PhA was significantly lower in the patient grouop than in the healthy controls (4.51 ± 0.87 versus 5.22 ± 0.55; P < 0.0001). A significant positive correlation was found between VEGF and PhA (P = 0.009, beta coefficient = 1.48) in SSc patients. A negative correlation between VEGF and DAI (P = 0.048, ß coefficient = 0.48) was found. PhA median value was significantly (P = 0.006) lower in patients with late pattern SSc (4.2 [2.5-5.3]). PhA median value was significantly (P < 0,0001) lower in patients with digital ulcers (DUs; 4.2 [2.5-5.3]) than in those without DUs (3.80 [2.50-5] versus 4.75 [2.80-7.3]). These data were confirmed in both female and male patients. CONCLUSIONS: The evaluation of VEGF with PhA, NVC, and DUs could be useful to estimate cellular and microvascular damage in patients with SSc.

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.

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. In the prospective study (IRB 5435), 66 SSc patients underwent high-resolution computerized tomography (HRCT) at baseline. HRCT was performed according to standard protocol using a CT 64GE light speed VCT power scanner. CALIPER classified lung parenchyma on volume units. Every volume unit was classified into radiological parenchymal patterns (honeycombing, reticular and ground glass). Pulmonary function tests (PFTs) were performed at baseline and after 12 months of follow-up. Cigarette smoking and other lung diseases unrelated to SSc are exclusion criteria. CALIPER analysis showed normal lung parenchyma 87.4 ± 9.8%, ground glass 2.8 ± 5.3%, reticular 4 ± 5.7%, and honeycombing 1 ± 1%. In multiple regression analysis, FEV1 (p < 0.0001), FVC (p = 0.001), and DLCO (p < 0.0001) measurements at baseline showed a negative correlation with the reticular pattern percentage. At follow-up, DLCO reduction showed a positive correlation (p < 0.001) with the percentage of ground glass pattern (r = 0.33, beta coefficient = 0.51). In the ROC curve analysis, ground glass score is a good predictor (0.75, p = 0.009; 95% CI 0.59-0.91) of DLCO worsening, defined as a decrease of more than 10% of DLCO. Using a cutoff ≥ 4.5 for ground glass score, the RR for DLCO worsening is 6.8 (p < 0.01; 95% CI 1.6-29.2). The results of this study show that CALIPER is useful not only for quantifying lung damage but also for assessing worsening PFTs, but larger studies are needed to confirm these preliminary data.Key Points• At baseline reticular pattern showed negative correlation with PFTs• At follow-up ground glass pattern predicts worsening of DLCO• CALIPER is a useful to quantify lung damage.

Antiphospholipid antibodies and renal involvement.

Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with the hypercoagulable state affecting all vascular districts with thrombosis named antiphospholipid syndrome (APS). APS is an autoimmune disease with multifactorial etiology that includes cellular, molecular, genetic and pathogenic mechanisms. The APS clinical features are a combination of arterial and/or venous thrombosis, hematological events, recurrent fetal losses, neurological disorders and intra-abdominal manifestations. The renal involvement is associated with both primary and secondary APS. Clinical features include hypertension, renal artery stenosis, thrombotic microangiopathy and other histological manifestations of the nephropathy (APSN), venous renal thrombosis, APSN in the course of systemic lupus erythematosus and renal failure. APSN is an independent risk factor that should be included in the classification criteria for definite APS with characteristic clinical and histological features.

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. METHODS: Forty-two consecutive patients with a diagnosis of SSc without cardiac and/or renal impairment were enrolled to assess whether cardiac muscle mass can be related to arterial stiffness. Thirty subjects matched for age and sex were also enrolled as healthy controls (HC). All patients performed echocardiography and renal ultrasound. RESULTS: Doppler indices of intrarenal stiffness and echocardiographic indices of LVM were significantly increased in SSc patients compared to HC. A positive correlation exists between LVM/body surface area and pulsatile index (p < 0.05, r = 0.36), resistive index (p < 0.05, r = 0.33) and systolic/diastolic ratio (p < 0.05, r = 0.38). Doppler indices of intrarenal stiffness and LVM indices were significantly higher in SSc patients with digital ulcers than in SSc patients without a digital ulcer history. CONCLUSIONS: SSc is characterized by the presence of microvascular and multiorgan injury. An early cardiac and renal impairment is very common. LVM and intrarenal arterial stiffness can be considered as early markers of CRS onset. The clinical use of these markers permits a prompt identification of organ damage. An early diagnosis allows the appropriate setting of pharmacological management, by slowing disease progression. © 2016 S. Karger AG, Basel.