Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients

The impairment of the right ventricle (RV) in systemic sclerosis (SSc) is usually related to pulmonary arterial hypertension (PAH). New echocardiographic techniques, such as 3-dimensional echocardiography (3DE) and 2-dimensional speckle tracking (2DSTE), allow an accurate evaluation of the RV function. The aim of this study was to evaluate the RV function using 3DE and 2DSTE in SSc patients with no history of heart disease and no PAH. Forty-five SSc patients, 42 females and 3 males, 28 with limited cutaneous SSc (lcSSc) and 17 with diffuse cutaneous SSc (dcSSc), were studied. Forty-three age- and gender-matched healthy subjects were enrolled as controls. All of them underwent a 3DE and 2DSTE ecocardiographic evaluation of the RV function. Systolic pulmonary arterial pressure (sPAP) and total pulmonary vascular resistance (tPVR) were also estimated by power doppler. RV echocardiographic parameters were compared in the different subsets of SSc patients. A statistical analysis was performed by t-test, ANOVA and multiple logistic regression. RV areas in 2DSTE and volumes in 3DE were higher and RV function parameters were reduced in SSc patients compared with controls. Also sPAP and tVPR were higher, but they did not reach pathological values. Echocardiographic alterations were more pronounced in patients with lcSSc. 3DE and 2DSTE echocardiography allowed us to detect morphological and functional alterations of the RV in a group of SSc patients with no clinical signs of heart disease and no PAH. These patients had significantly higher sPAP and tPVR than healthy controls without reporting values compatible with PAH. These data suggest that RV alterations are related to a pressure overload rather than to an intrinsic myocardial involvement in SSc

The PREdictor of MAlnutrition in Systemic Sclerosis (PREMASS) Score:A Combined Index to Predict 12 Months Onset of Malnutrition in Systemic Sclerosis

Objective: Malnutrition is a severe complication in Systemic Sclerosis (SSc) and it is associated with significant mortality. Notwithstanding, there is no defined screening or clinical pathway for patients, which is hampering effective management and limiting the opportunity for early intervention. Here we aim to identify a combined index predictive of malnutrition at 12 months using clinical data and specific serum adipokines. Methods: This was an international, multicentre observational study involving 159 SSc patients in two independent discovery (n = 98) and validation (n = 61) cohorts. Besides routine clinical and serum data at baseline and 12 months, Malnutrition Universal Screening Tool (MUST) score and serum concentration of leptin and adiponectin were measured for each participant at baseline. The endpoint of malnutrition was defined according to European Society of Clinical Nutrition and Metabolism (ESPEN) recommendation. Significant parameters from univariate analysis were tested in logistic regression analysis to identify the predictive index of malnutrition in the derivation cohort. Results: The onset of malnutrition at 12 months correlated with adiponectin, leptin and their ratio (A/L), MUST, clinical subset, disease duration, Scl70 and Forced Vital Capaciy (FVC). Logistic regression analysis defined the formula: −2.13 + (A/L*0.45) + (Scl70*0.28) as the best PREdictor of MAlnutrition in SSc (PREMASS) (AUC = 0.96; 95% CI 0.93, 0.99). PREMASS 62% and negative predictive value (NPV) > 97% for malnutrition at 12 months. Conclusion: PREMASS is a feasible index which has shown very good performance in two independent cohorts for predicting malnutrition at 12 months in SSc. The implementation of PREMASS could aid both in clinical management and clinical trial stratification/enrichment to target malnutrition in SSc

Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments

The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations

Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature.Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 +/- 26.9 yrs.; mean disease duration 8.9 +/- 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas.Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches.Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities

Medium-/long-term effects of a specific exercise protocol combined with patient education on spine mobility, chronic fatigue, pain, aerobic fitness and level of disability in fibromyalgia.

Objective. To propose a rehabilitation protocol able to produce immediate and long-termbeneficial effects on level of disability and overall performance in ADLs. Materials and Methods. Forty-one FM patients were randomized to an exercise and educationalbehavioral programme group (experimental group, EG = 21) or to a control group (CG = 20). Each subject was evaluated before, at the end (T1), and after 6 months (T6) from the conclusion of the rehabilitation treatment using the Fibromyalgia Impact Questionnaire (FIQ), the visual analogue scale (VAS), the Health Assessment Questionnaire (HAQ), the fatigue severity scale (FSS), the 6-minute walking test (6MWT), tender points count (TPC), and spinal active range of motion. The exercise protocol included 20 sessions consisting in self-awareness, stretching, strengthening, spine flexibility, and aerobic exercises, which patients were subsequently educated to perform at home. Results. The two groups were comparable at baseline. At T1, the EG showed a positive trend in FIQ, VAS, HAQ, and FSS scales and significant improvement in 6MWT and in most spinal active range of motion measurements (P between 0.001 and 0.04). The positive results were maintained at the follow-up. Conclusion. The proposed programme was well tolerated and produced immediate and medium-term beneficial effects improving function and strain endurance

Determinants of impairment in lung diffusing capacity in patients with systemic sclerosis

OBJECTIVES: Lung diffusing capacity for carbon monoxide (DLCO) is impaired in interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) associated to systemic sclerosis (SSc), but the mechanism of DLCO reduction remains controversial. We hypothesised that the determinants of DLCO impairment differ in interstitial or vascular involvement of the lung of SSc patients. METHODS: DLCO was partitioned into alveolar-capillary membrane conductance (Dm) and pulmonary capillary blood volume (Vc) using combined single-breath DLNO and DLCO measurements. Seventeen SSc patients without pulmonary involvement (SSc), 20 SSc patients with ILD (SSc-ILD), with and without PAH, and 21 healthy controls were included. RESULTS: DLNO and Dm were reduced in SSc patients as compared with controls, whereas Vc was not significantly different. SSc-ILD patients showed a highly significant decrease in Dm and Vc as compared with SSc patients and controls. Vc tended to be more reduced than Dm in SSc-ILD patients with PAH. Dm and Vc were negatively correlated with PAPs and HCRT scores, but the relationship with the HRCT score was stronger. CONCLUSIONS: DLNO is more sensitive than DLCO in detecting functional impairment in SSc without radiologic or haemodynamic alterations. A disproportional reduction of Dm relative to Vc suggests a thickening of the blood-gas diffusion barrier in these patients. In SSc patients with detectable ILD, the gas exchange impairment is due to both components of lung diffusing capacity, and partitioning of DLCO in Dm and Vc is of little use in distinguishing the patients with only ILD from those with ILD complicated by PAH

Plasma-exchange as a "rescue therapy" for dermato/polymyositis in acute phase. Experience in three young patients

There are few data in the literature supporting the efficacy of plasma-exchange in dermato/polymyositis. The authors report three cases of patients with acute disease phase showing severe pharyngo-esophageal muscle weakness unresponsive to conventional therapy (corticosteroids and immunosuppressant agents) who were treated with plasma-exchange. As the patients were at high risk of "aspiration pneumonia", tracheostomy and PEG tubes were placed. The patients underwent a series of plasma-exchange for a mean of 15 weeks, during which time they progressively recovered muscle strength, their serum muscle enzyme values returned to normal levels, and MRI showed resolution of muscle edema. The tracheostomy and PEG tubes could be removed. Our findings suggest that plasma-exchange in association with immunosuppressant agents could play a relevant role in the management of dermato/polymyositis in acute phase

Right heart function by 3D-echocardiography and 2D-speckle tracking in scleroderma patients in absence of pulmonary hypertension

Purpose: Systemic Sclerosis (SSc) involves the right heart (RH) with the appearance of pulmonary hypertension (PH). Whether SSc can directly affect RH function in absence of PH is unknown. Recently, 3D-echocardiography (3DE) and 2D-speckle tracking (2D-STE) have been validated to assess heart chamber function and mechanics. Therefore, we used3DEand 2D-STE to assess right ventricular (RV) andright atrial (RA) function in patients with SSc without PH. Methods: 34 SSc patients without PH were compared with 34 age and gender-matched healthy volunteers. All subjects underwent a complete echocardiogram, including 3DE RV volumes and ejection fraction (EF) and global RV and RA longitudinal strain (Ls) by 2D-STE. Results: SSc patients demonstrated similar RV size with lower RV function but no differences about RV global Ls (p=NS) (Table). Pulmonary artery systolic pressure (PASP) and pulmonary vascular resistance (PVR) were higher in SSc patients (Table). RA appeared larger in patients, but with lower active contraction (RA-LsNeg). At bivariate analysis, PVR was inversely correlated with RV EF (r=-0.34, p= 0.008) and RA-LsNeg (r= -0.27, p=0.04) and directly correlated with RA maximum volume increase (r=0.31,p=0.012). Conclusions: a slightly increased afterload in SSc patients appared to be associated to an impairment of RV pump function, with normal RV myocardial mechanics, paralleled by an increase of RA volume with an impairment of RA active myocardial contraction

Nailfold avascular score and coronary microvascular dysfunction in systemic sclerosis: A newsworthy association

BACKGROUND AND AIMS: We aimed to assess the relationship between nailfold videocapillaroscopy (NVC) abnormalities and coronary flow reserve (CFR), a marker of coronary microvascular dysfunction (CMD) in patients with systemic sclerosis (SSc). METHODS: We studied 39 SSc patients (33 females, mean\u202f\ub1\u202fSD age 54\u202f\ub1\u202f12\u202fyears, median disease duration 11\u202fyears, range 6-22) and 22 controls (matched for age and sex) without any evidence of cardiovascular disease. Clinical assessment was performed by modified Rodnan skin score (mRss) and EUSTAR score. Coronary flow velocities in the left anterior descending coronary artery were measured by transthoracic echocardiography. Average peak flow velocities, CFR and microvascular resistance at baseline (BMR) and in hyperaemic (HMR) condition were assessed. CFR 642.5 was considered marker of CMD. Six NVC-abnormalities were evaluated by a semi quantitative scoring system: enlarged and giant capillaries (diameter\u202f>\u202f20\u202f\u3bcm and >50\u202f\u3bcm, respectively), hemorrhages, disarray, capillary ramifications and loss of capillaries (avascular score). Statistic was performed using SPSS. RESULTS: CFR was lower in SSc patients than in controls (2.6\u202f\ub1\u202f0.5 vs 3.3\u202f\ub1\u202f0.5). CMD was detected in 24 patients (61.5%) vs 0 controls (p\u202f<\u202f.0001). CFR was inversely correlated with NVC-avascular score (rho\u202f=-0.750, p\u202f<\u202f.0001). Avascular and capillary ramifications scores (p\u202f=\u202f.001 and p\u202f=\u202f.03, respectively), mRss (p\u202f=\u202f.003) and EUSTAR score (p\u202f=\u202f.01) were higher in patients with CMD than in those without. At multivariable analysis, avascular score was independently associated with CMD (p\u202f=\u202f.01). HMR was directly correlated with avascular score (rho\u202f=\u202f0.416, p\u202f=\u202f.008). CONCLUSIONS: In our SSc patients NVC-avascular score was associated with CMD which seems to be the result of a structural microvascular remodeling