Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

1984 ◽  
Vol 7 (1) ◽  
pp. 49-53 ◽  
Author(s):  
D. Morrison ◽  
S. Goldman ◽  
F. P. Alepa
Keyword(s):  
Systemic Sclerosis ◽  
Right Ventricle ◽  
Progressive Systemic Sclerosis ◽  
Crest Syndrome ◽  
The Right

Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis

1984 ◽  
Vol 77 (3) ◽  
pp. 489-496 ◽  
Author(s):  
William P. Follansbee ◽  
Edward I. Curtiss ◽  
Thomas A. Medsger ◽  
Gregory R. Owens ◽  
Virginia D. Steen ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Myocardial Function ◽  
Progressive Systemic Sclerosis ◽  
Crest Syndrome

scholarly journals B-Type Natriuretic Peptide as a Marker of Different Forms of Systemic Sclerosis

2018 ◽  
Vol 37 (4) ◽  
pp. 406-414 ◽  
Author(s):  
Jelena Stefanović Nešković ◽  
Anđelka Ristić ◽  
Milan Petronijević ◽  
Aleksandra Zeljković ◽  
Aleksandra Grdinić ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Right Ventricle ◽  
Brain Natriuretic Peptide ◽  
Right Ventricular ◽  
Natriuretic Peptide ◽  
Right Atrium ◽  
Plasma Concentrations ◽  
Cardiovascular Complications ◽  
Similar Distribution ◽  
The Right

SummaryBackground:Systemic sclerosis (SSc) is an autoimmune connective tissue disease which affects various tissues and organs, including skin, lungs, kidneys, gastrointestinal tract and cardiovascular system. Cardiac involvement is the most commonly recognized problem and a significant cause of morbidity. The brain natriuretic peptide (BNP) is a previously known marker of elevated cardiovascular risk in SSc, but the levels of BNP in various forms of SSc have not been investigated so far.Aim:The aim of our study was to evaluate the influence of SSc on the function of the right ventricle and the right atrium using the echocardiographic parameters. Moreover, we examined the levels of BNP in different forms of SSc as well as the association of disease severity with the plasma concentrations of BNP.Methods:We included 42 patients with newly diagnosed SSc and patients whose disease had been diagnosed earlier. SSc patients and non-SSc control patients were examined by using echocardiography and the concentrations of BNP were determined.Results:We analyzed differences in the parameters of right ventricle (RV) function and right atrium (RA) function between SSc patients and healthy controls. The two groups had similar distribution of gender, but SSc patients were significantly older than controls. RV wall thickness was increased in SSc patients (p<0.001), while right ventricular end-systolic area (RVESA; p=0.408) and right ventricular end-diastolic area (RVEDA; p=0.368) did not differ among the examinees. In contrast, RA minor-axis dimension (p=0.001) and the tricuspid annular plane systolic excursion (TAPSE) (p=0.001) were significantly higher in SSc patients. Also, we analyzed differences in brain natriuretic peptide (BNP) concentrations between diffuse cutaneous systemic sclerosis (DSSc) and limited cutaneous systemic sclerosis (LSSc) patients. DSSc patients had significantly higher concentrations of BNP. We found that levels of BNP were in significant positive correlations with age (p=0.007), disease duration (p=0.023), C reactive protein (CRP) (p=0.032), right ventricle fractional area change (FAC) (p=0.022), pulmonary vascular resistance (PVR) and Rodnan score (p=0.019).Conclusions:Given the obtained results, the laboratory determination of BNP could be useful in differentiating different forms of systemic sclerosis as well as in predicting the severity of the disease and future cardiovascular complications.

scholarly journals Dystrophic calcifications and Raynaud’s phenomenon in an eight-year old girl

2014 ◽  
Vol 142 (3-4) ◽  
pp. 239-242
Author(s):  
Slobodan Grebeldinger ◽  
Jelena Tomic ◽  
Gordana Vijatov-Djuric ◽  
Branka Radojcic ◽  
Nada Vuckovic ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Soft Tissue ◽  
Lupus Erythematosus ◽  
Vascular Trauma ◽  
Crest Syndrome ◽  
Myositis Ossificans Progressiva ◽  
Calcium Deposits ◽  
Tissue Calcium ◽  
The Right

Introduction. Dystrophic calcifications are the most common subtype of skin calcinosis. Tumorous soft tissue calcium deposits usually contain hydroxyapatite and amorphous calcium phosphate. Differential diagnosis of skin calcinosis encompasses Thibierge-Weissenbach syndrome, systemic sclerosis, scleroderma, CREST syndrome (calcinosis, Raynaud?s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia), dermatomyositis, systemic lupus erythematosus, ad myositis ossificans progressiva. Case Outline. We present the case of an eight-year old girl with tumorous soft tissue calcium deposits and Raynaud?s phenomenon. At the age of 3.5 years, our patient was admitted to Pediatric Surgery Clinic because of bilateral acrocyanosis localized at the fingertips area of hands, with the signs of vascular trauma. Therapy with vasodilators and hyperbaric oxygen treatment were completed. This therapy resulted in improvement. At the age of eight, the patient was admitted again due to intermittent, painful cramps localized in both hands. Punctiform deposits were present at the tips of fingers and toes, which looked like calcifications and were spontaneously eliminated, with the remnants of crater-shaped defects. A hard tumorous deformity localized in soft tissue was present in the extensor area of the right elbow. Laboratory indicators of inflammation were within the reference values, and antinuclear antibodies were positive. A nodus localized at the right elbow was extirpated. Pathohistological findings: connective and fat tissue with large deposits of calcium. Conclusion. Further follow-up of our patient is necessary due to possible development of complete picture of CREST syndrome or systemic sclerosis.

scholarly journals AB0611 STRAIN ANALYSIS OF THE RIGHT VENTRICLE USING 2D-SPECKLE TRACKING ECHOCARDIOGRAPHY IN A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1601.1-1602
Author(s):  
A. Spinella ◽  
P. Macripo’ ◽  
E. Cocchiara ◽  
E. Galli ◽  
F. Lumetti ◽  
...  
Keyword(s):  
Uric Acid ◽  
Systemic Sclerosis ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Speckle Tracking ◽  
Speckle Tracking Echocardiography ◽  
Left Ventricular ◽  
Right Ventricular Strain ◽  
2D Speckle Tracking ◽  
The Right

Background:Systemic Sclerosis (SSc) is a rare and life-threatening connective tissue disease with multiple organ impairment. Cardio-pulmonary involvement is common: pulmonary fibrosis, pulmonary hypertension (PH), and electrical disorders are the most serious complications and causes of increased mortality.Objectives:We evaluated features related with the onset and development of PH in a cohort of SSc patients. We further studied ecocardiographic abnormalities, by means of 2D-speckle tracking echocardiography (STE) with specific reference to the right ventricular strain measure (RV-strain).Methods:We analyzed data from 50 SSc patients (pts) referred to our University-based Rheumatology Centre and SSc Unit from January 2007 to June 2019 (F/M 45/5; lc/dcSSc 45/5; mean age 59.20±14.357 years; mean disease duration 12.08±8.75 years). All pts underwent general and cardio-pulmonary assessment in our Cardio-Rheumatology Clinic. The following parameters were considered: blood exams, in particular inflammation indexes, uric acid test and serum autoantibodies; pulmonary function tests; high resolution scan of the lungs (HRCT); standard electrocardiogram (ECG) and RV-strain measured by 2D-STE. These examinations were performed according to clinical picture and current methodologies. We compared SSc subjects with (10/50) and without (40/50) PH diagnosis during follow-up regardless of treatments.Results:SSc pts with PH didn’t show significant alterations concerning RV-strain if compared with pts without PH (p=0.707). Nevertheless, RV-strain value was modified in relation to TAPSE alterations in all pts but this data correlated with right ventricular dilatation only in PH subjects. Furthermore, interesting significant values about dilatation of right and left atria (p=0.007, p=0.048), dilatation of inferior vena cava (p=0.037) and right ventricle (p=0.023) were observed. Left ventricular hypertrophy (p=0.012) as well as valvular insufficiencies (mitral and aortic) were more frequent in PH group too (p=0.016). These pts showed higher incidence of skin ulcers (p=0.0001), higher values of blood pressure (p=0.004), elevated uric acid levels (p=0,027) and anti-centromere antibodies positivity (p=0.0001).Conclusion:Our research provides further evidence of the prognostic value of echocardiographic findings in SSc subjects, with focus on PH. Population enlargement is ongoing in order to identify more accurate results about RV-strain, considering the efficacy of PH treatments on cardiac contractility. Speckle tracking echocardiography proves to be a sensitive, low-cost, non-invasive and reliable tool to detect early cardiac impairment in Ssc, full of potential future prospects.Disclosure of Interests:None declared

scholarly journals Juvenile Onset Systemic Sclerosis

1984 ◽  
Vol 77 (9) ◽  
pp. 793-794 ◽  
Author(s):  
S M Burge ◽  
T J Ryan ◽  
R P R Dawber
Keyword(s):  
Systemic Sclerosis ◽  
Progressive Systemic Sclerosis ◽  
Crest Syndrome ◽  
Juvenile Onset ◽  
Anticentromere Antibody

A case of progressive systemic sclerosis (CREST syndrome), which began in childhood, is described. Juvenile onset CREST syndrome is extremely rare; there are few well documented cases and no published series of children with this form of progressive systemic sclerosis. An anticentromere antibody was recently detected in this case and its significance is discussed.

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