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1.
Clin Exp Med ; 23(2): 495-502, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35614365

RESUMEN

Chronic spontaneous urticaria (CSU) is a disorder characterized by wheals and/or angioedema. The coagulation cascade and inflammation pathways are closely linked together. The aim of our study was first to investigate the dynamics of clot formation in plasma (Clot Waveform Analysis, CWA) in a group of 47 patients with CSU along with other coagulative parameters dedicated to the study of hypercoagulability, such as D-Dimer, F 1 + 2 peptide, Fibrinogen, Platelet count and Mean Platelet Volume (MPV). Secondly, 23 out of 47 patients were treated with Omalizumab at four administration intervals from T0 to T4. A statistically significant increase in Activated Partial Thromboplastin (aPTT) ratio, D-Dimer, F1 + 2, Platelet count and MPV was found when compared with 53 healthy controls (HC). In contrast, the 2nd Derivative of aPTT showed lower values than those of the HC. No differences were found between 1st derivative of aPTT and Fibrinogen. D-Dimer only showed a significant difference between T0 and T3. An activation of both coagulation and fibrinolysis along with a weaker clot acceleration may be in agreement with a low-grade DIC. The accelerated turnover of platelets expressed by both an increase in platelet count and MPV further supports this pathway in CSU. Omalizumab does not affect the relationship between the immune and the hemostatic systems.


Asunto(s)
Urticaria Crónica , Coagulación Intravascular Diseminada , Urticaria , Humanos , Omalizumab/uso terapéutico , Urticaria/tratamiento farmacológico , Fibrinógeno
2.
Sci Rep ; 11(1): 2681, 2021 01 29.
Artículo en Inglés | MEDLINE | ID: mdl-33514797

RESUMEN

Endothelial damage and fibro-proliferative vasculopathy of small vessels are pathological hallmarks of systemic sclerosis (SSc). The consequence is the detachment of resident elements that become circulating endothelial cells (CECs). The aim of our study was to evaluate the potential of CECs as biomarker in SSc. We enrolled 50 patients with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subset of SSc, who underwent clinical evaluation to establish the organ involvement. CECs were measured by flow-cytometry utilizing a polychromatic panel. An evident difference was observed in CEC counts comparing controls to SSc patients (median 10.5 vs. 152 cells/ml, p < 0.0001) and for the first time, between the two subsets of disease (median lcSSc 132 vs. dcSSc 716 CEC/ml, p < 0.0001). A significant correlation was established between CECs and some SSc clinical parameters, such as digital ulcers, skin and pulmonary involvement, presence of Scl-70 antibodies, nailfold videocapillaroscopy patterns and EUSTAR activity index. After 12 months, CECs correlated with clinical worsening of patients, showing that a number higher than 414 CEC/ml is a strong negative prognostic factor (RR 5.70). Our results indicate that CECs are a direct indicator of systemic vascular damage. Therefore, they can be used as a reliable marker of disease severity.


Asunto(s)
Células Endoteliales/metabolismo , Angioscopía Microscópica , Esclerodermia Sistémica/sangre , Adulto , Anciano , Células Endoteliales/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/patología , Índice de Severidad de la Enfermedad
3.
Int J Cardiol ; 241: 401-406, 2017 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-28476520

RESUMEN

BACKGROUND: Pulmonary arterial hypertension (PAH) in systemic sclerosis (SS) identifies a poor prognosis subset of patients. Recent studies suggested a "metabolic theory" on the development of pulmonary arterial hypertension. On this basis we performed a metabolomic study in order to evaluate whether differences in pulmonary arterial blood metabolites were identifiable in SS patients with increased pulmonary vascular resistance (PVR). METHODS: We studied 18 SS patients (age 58.7±15.6years) free of pulmonary fibrosis who underwent a right heart catheterization (RHC). A blood sample was collected during the RHC in the distal peripheral circulation of the pulmonary arteries to perform the metabolomic analysis. RESULTS: Based on PVR we divided the population into Group A (n=8; PVR=1.16±0.23WU) and Group B (n=10; PVR=2.67±0.67WU; p<0.001 vs Group A). No significant differences were identified in terms of anthropometric, clinical, echo and therapeutic characteristics. At RHC the 2 groups showed a difference in mean pulmonary pressure values (Group A: 20±4mmHg; Group B: 27±3.4mmHg; p=0.03), with mild PAH in Group B. We applied an OSC-PLS-DA with a clear clusterization; SSc patients with PAH showed an increase in acetate, alanine, lactate, and lipoprotein levels and a decrease in γ-aminobutyrate, arginine, betaine, choline, creatine, creatinine, glucose, glutamate, glutamine, glycine, histidine, phenylalanine, and tyrosine levels CONCLUSIONS: Our results suggest that, despite similar clinical and disease-related parameters, SSc patients who develop PAH have an unfavorable metabolic profile able to cause an impaired production of metabolites with protective effects on endothelial cells.


Asunto(s)
Dermatoglifia del ADN/métodos , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/genética , Metabolómica/métodos , Esclerodermia Sistémica/diagnóstico por imagen , Esclerodermia Sistémica/genética , Adulto , Anciano , Femenino , Humanos , Hipertensión Pulmonar/epidemiología , Espectroscopía de Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/epidemiología
4.
Int J Cardiovasc Imaging ; 31(3): 529-36, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25547289

RESUMEN

Several studies have evidenced high prevalence of myocardial systolic and diastolic dysfunction among patients with systemic sclerosis (SSc). Exercise echocardiography has shown a diagnostic and prognostic role in identifying early left ventricular (LV) dysfunction in several myocardial pathological settings. The aim of our study was to evaluate early signs of LV impairment under exercise and their correlation to patient's exercise tolerance. Forty-five patients (age 60.4 ± 10.3 years) with SSc and 20 age and sex comparable controls were enrolled in the study. All patients underwent clinical evaluation, 2D echocardiography associated with Tissue Doppler and speckle tracking to evaluate LV deformation indexes, and an exercise echocardiography to evaluate left ventricle contractile reserve (LVCR) and exercise pulmonary pressures. Finally, a 6-minute walking test (6MWT) to evaluate exercise tolerance was also performed. Compared to controls, SSc patients showed an impaired diastolic function (E/E' 10.9 ± 3.7 vs 8.36 ± 2.01; p < 0.01) associated with larger left atrial dimensions (LAVI 28.4 ± 8.7 vs 19.3 ± 4.6 mL/m(2); p < 0.01). During exercise echocardiography, a reduced global longitudinal strain at peak exercise (S-GLS) was highlighted compared to controls (15.7 ± 3.6 vs 18.2 ± 2.2; p = 0.001). A S-GLS cutoff <18%, identified by ROC analysis, identified SSc patients with a reduced diastolic function, exercise tolerance at the 6MWT and higher pulmonary pressures. Our data show that in SSc patients a reduced LVCR characterizes the patients with a more extensive cardiovascular impairment in terms of LV diastolic function, pulmonary pressures and exercise tolerance. These data underline the importance of exercise echocardiography for the preclinical screening of the LV impairment in this population.


Asunto(s)
Ecocardiografía Doppler en Color , Ecocardiografía de Estrés , Prueba de Esfuerzo , Tolerancia al Ejercicio , Contracción Miocárdica , Esclerodermia Sistémica/complicaciones , Disfunción Ventricular Izquierda/diagnóstico por imagen , Función Ventricular Izquierda , Anciano , Área Bajo la Curva , Presión Arterial , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Estudios Prospectivos , Arteria Pulmonar/fisiopatología , Curva ROC , Factores de Riesgo , Esclerodermia Sistémica/diagnóstico , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Caminata
5.
Ann Ital Med Int ; 20(3): 187-91, 2005.
Artículo en Italiano | MEDLINE | ID: mdl-16250185

RESUMEN

A case of Dercum's disease in a 51-year-old obese woman with a history of brain vasculitis, and painful subcutaneous multiple lipomas is described. This disease, included in the category of rare diseases by the World Health Organization, first described in 1892 by Francis Xavier Dercum, is characterized by its prevalence among women, its familiarity, by the presence of multiple painful subcutaneous lipomas and its association with obesity, hypercholesterolemia and asthenia. The disease has to be differentiated from Madelung syndrome, the multiple familiar lipomatosis and Proteus' syndrome. The quality of life of the patients is often poor and since treatments so far have not been conclusive a better knowledge of the pathogenesis of the disease is desirable.


Asunto(s)
Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/genética , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Linaje
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