Evaluation of estimated glomerular filtration rate and clinical variables in systemic sclerosis patients

Objectives: The most important renal complication of systemic sclerosis (SSc) is scleroderma renal crisis (SRC). Many patients demonstrate less severe renal complications, most likely associated with reduced renal blood flow and a consequent reduction in glomerular filtration rate (GFR). The mechanism of this slowly progressive form of chronic renal disease is unclear. The aim of this study was to evaluate GFR by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) and the 7-variable Modification of Diet and Renal Disease (MDRD) equations in SSc patients and to correlate estimated GFR (eGFR) with clinical variables of the disease. Methods: 105 unselected and consecutive patients with SSc were enrolled. Serum creatinine was measured in all patients and GFR was estimated by 7-variable MDRD and CKD-EPI equations. Nailfold videocapillaroscopy was performed in all patients. Results: The mean value of eGFR evaluated by both 7-variable MDRD and CKD-EPI was significantly different (p < 0.0001) in the three capillaroscopic groups and correlated negatively with the severity of capillaroscopic damage (early: 95 ± 16 mL/min and 101 ± 12 mL/min, active: 86 ± 25 mL/min and 95 ± 17 mL/min, late: 76 ± 21 mL/min and 82 ± 21 mL/min). The mean value of eGFR evaluated by 7-variable MDRD (97 ± 23 mL/min vs. 74 ± 15 mL/min, p < 0.0001) and CKD-EPI (0.83 ± 0.20 mL/min vs. 0.68 ± 0.10 mL/min, p < 0.0001) was significantly higher in SSc patients without history of digital ulcers than in those with. Conclusion: We can conclude that in SSc patients without renal involvement, eGFR decreases with the progression of digital vascular damag

Gastric parietal cell antibodies: demonstration by immunofluorescence of their reactivity with surface of the gastric parietal cells.

Viable, intact gastric cells were obtained by pronase digestion of inverted rat stomach. The cell suspensions contained two main distinct cell population, i.e. 'large' cells (mean diameter 16 microns) and 'small' cells (mean diameter 8.5 microns). By indirect immunofluorescence on smears of dispersed rat gastric cells, the large cells were identified as parietal cells, since all the sera containing parietal cell antibodies (PCA) were seen to react with the cytoplasm of these cells, leaving the cytoplasm of the small cells completely unstained. Thirty-one PCA-positive sera and forty-one PCA-negative sera were tested for gastric cell surface-reactive antibodies by an indirect immunofluorescence technique on suspensions of viable gastric cells. All the PCA-containing sera yielded a membrane immunofluorescence confined to the large cells, while none of the PCA-negative sera induced this fluorescent pattern. The surface reaction persisted unmodified when F(ab')2 fragments processed from IgG PCA-positive sera and FITC-conjugated pepsin fragments of rabbit IgG directed against the F(ab')2 fragments of human IgG were employed for the membrane fluorescence studies. The absorption of PCA-positive sera with viable, intact gastric cells led to the disappearance of both the surface immunofluorescence of the viable large cells and the cytoplasmic fluorescence of the rat parietal cells. These results demonstrate that PCA invariably react with an antigen represented on the surface of parietal cells, and that this antigen is immunologically identical to the intracytoplasmic 'microsomal' antigen

Three-dimensional echocardiography and 2D-3D speckle tracking imaging in chronic pulmonary hypertension. diagnostic accuracy in detecting hemodynamic signs of RV failure

Background and objective. Our aim was to compare three-dimensional (3D) and 2D and 3D speckle tracking (2D-STE, 3D-STE) echocardiographic parameters with conventional right ventricular (RV) indexes in patients with chronic pulmonary hypertension (PH), and investigate whether these techniques could result in better correlation with hemodynamic variables indicative of heart failure. Methods. Seventy-three adult patients (mean age, 53±13 years; 44% male) with chronic PH of different etiologies were studied by echocardiography and cardiac catheterization (25 precapillary PH from pulmonary arterial hypertension, 23 obstructive pulmonary heart disease, and 23 postcapillary PH from mitral regurgitation). Thirty healthy subjects (mean age, 54±15 years; 43% male) served as controls. Standard 2D measurements (RV-FAC -fractional area change-, TAPSE -tricuspid annular plane systolic excursion-) and mitral and tricuspid tissue Doppler annular velocities were obtained. RV 3D volumes, and global and regional ejection fraction (3D-RVEF) were determined. RV strains were calculated by 2D-STE and 3D-STE. Results. RV 3D global-free-wall longitudinal strain (3DGFW-RVLS), 2D global-free-wall longitudinal strain (GFW-RVLS), apical-free-wall longitudinal strain (AFW-RVLS), basal-free-wall longitudinal strain (BFW-RVLS), and 3D-RVEF were lower in patients with pre-capillary PH (p<0.0001) and post-capillary PH (p<0.01) compared to controls. 3DGFW-RVLS (HR 4.6, 95% CI 2.79-8.38, p=0.004) and 3D-RVEF (HR 5.3, 95% CI 2.85-9.89, p=0.002) were independent predictors of mortality. ROC curves showed that the thresholds offering an adequate compromise between sensitivity and specificity for detecting hemodynamic signs of RV failure were 39% for 3D-RVEF (AUC 0.89), -17% for 3DGFW-RVLS (AUC 0.88), -18% for GFW-RVLS (AUC 0.88), -16% for AFW-RVLS (AUC 0.85), 16mm for TAPSE (AUC 0.67), and 38% for RV-FAC (AUC 0.62). Conclusions. In chronic PH, 3D, 2D-STE and 3D-STE parameters indicate global and regional RV dysfunction that is associated with RV failure hemodynamics better than conventional echo indices

Regional diastolic function by tissue Doppler echocardiography in systemic sclerosis: correlation with clinical variables

The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (SSc), while systolic dysfunction is present in a small percentage of patients. The aim of this study was to asses the LV "regional" diastolic abnormalities in SSc patients by the mean of Doppler tissue imaging (DTI). Echocardiographic echo-Doppler (DE) and DTI parameters were analyzed for 67 SSc patients: abnormal E/A ratio at DE was detected in 24, while abnormal e/a at DTI was observed in 41. A significant prevalence of DTI diastolic abnormalities in the segments reflecting longitudinal versus those reflecting radial LV motion was found. The segments of the basal regions of LV myocardium were significantly more involved than those of the middle portion. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal e/a DTI

Clinical course and prognosis of the lymphoproliferative disease of granular lymphocytes. A multicenter study.

Lymphoproliferative disease of granular lymphocytes (LDGL) is a recently recognized, relatively rare atypical lymphocytosis characterized by the presence of over 2000 lymphocytes with cytoplasmic azurophilic granules/mm3 in the peripheral blood. The clinical course is heterogeneous, varying from spontaneous regression to progressive, malignant disease. As a consequence, clinical intervention is not standardized. In a worldwide multicenter study, the authors observed 151 patients with LDGL for a mean follow-up time of 29 months. Forty-three patients were asymptomatic at the time of diagnosis. In the remaining cases, clinical symptoms included fever (41 cases), infections (58), neutropenia (47), anemia (17), and thrombocytopenia (12). In 69 cases, LDGL coexisted with an associated disease. Most patients had a nonprogressive clinical course despite the presence of severe symptoms. In 19 patients, death related to LDGL occurred within 48 months. The authors investigated which features at diagnosis were significantly associated with increased mortality. In the univariate analysis, lymph node and liver enlargement, fever at presentation, skin infiltration, a low (less than or equal to 5000/mm3) or high (greater than 20,000/mm3) peripheral leukocyte count, relatively low (less than or equal to 3000) or high (greater than 7000/mm3) absolute peripheral granular lymphocyte (GL) count, and a low (less than or equal to 15%) percentage of HNK-1-positive cells were found to be predictors of increased mortality. In the multivariate analysis, significant independent predictors were fever at diagnosis, a low (less than or equal to 15%) percentage of HNK-1-positive peripheral blood mononuclear cells (PBMC) and a relatively low (less than or equal to 3000) GL count. These results showed that about 25% of the patients with LDGL were diagnosed after a routine blood count and had no clinical symptoms. The remaining patients were symptomatic, with some experiencing a fatal clinical course. The author's analysis of the significant prognostic features of LDGL may help in understanding the heterogeneous nature of this syndrom

Disordini immunoematologici

I disordini immunoematologici comprendono un esteso gruppo di entità nosologiche nell'ambito delle quali le malattie ematologiche, solitamente citopenie, sono caratterizzate da un'eziopatogenesi di natura immunologica. Le reazioni immunitarie conducono allo sviluppo di anemia emolitica, trombocitopenia, o neutropenia, isolate o associate. Immunodeficienze, malattie autoimmuni sistemiche e reazioni avverse a farmaci possono essere i meccanismi immunitari alla base di queste patologie. Anche il trasferimento di anticorpi materni per via transplacentare può causare fenomeni di citopenia nel feto e nel neonato. Altri disordini immunoematologici includono la presenza di anticorpi diretti contro fattori emocoagulativi, con possibile sviluppo di trombosi o emorragie, e reazioni emolitiche e non emolitiche in seguito alla somministrazione di emoderivat

Immunity, autoimmunity and autoimmune diseases in older people

Immunosenescence is defined as all the changes occurring in the immune system in the aged. Recent studies suggest that in older patients the immune system undergoes a functional remodelling. Two contrasting phenomena coexist in immunosenescence: the decreasing of immune response and the autoantibody production. Possible consequences are an increase of autoimmune phenomenon, neoplasia incidence, and predisposition to infections. The study of autoimmune manifestations in the elderly population should be considered as a priority for future medical research because of the increase in life expectancy, especially in developed countries. This review analyzes the clinical expression of systemic autoimmune diseases in older patients. Copyright © by BIOLIFE, s.a.s