Biomedical subjects

J Cailes

Publications and source records attributed to J Cailes.

3 recordsLinked to original sources

PubMed1998

Bronchoalveolar lavage cellularity: lone cryptogenic fibrosing alveolitis compared with the fibrosing alveolitis of systemic sclerosis.

Lone cryptogenic fibrosing alveolitis (CFA) is histologically identical to fibrosing alveolitis associated with systemic sclerosis (FASSc), but it has a much worse prognosis after matching for disease severity at presentation. The aims of this study were to gain insights into possible pathogenetic mechanisms contributing to this prognostic difference, by comparing bronchoalveolar lavage (BAL) cellularity in the two diseases, and to evaluate the relationships between BAL findings and the regional and global extent of disease, quantified by thin-section computed tomography (CT) and lung function indices. Patients with CFA were distinguished by more extensive fibrosing alveolitis on CT (p < 0.02) and by higher counts of neutrophils (total per ml, p < 0.02; percentage p < 0.03) and eosinophils (total per ml, p < 0.002; percentages, p < 0.02) in BAL fluid. After adjustment for functional and morphologic measures of disease extent, eosinophil percentages and total counts were increased in CFA (p < 0.05 in all 12 multivariate models), but they were not independently related to regional or global disease severity. Neutrophil percentages and total counts were virtually identical in CFA and FASSc in disease of comparable severity, and they increased with increasingly extensive lobar disease and global disease, as judged by CT, p < 0.0005 in all analyses. Neutrophil levels were more closely linked to the extent of disease on CT than to the severity of functional impairment, on univariate and multivariate analysis. The higher BAL eosinophil levels seen in CFA, compared with those seen in FASSc, after adjustment for disease extent, indicate that an eosinophilic influx may be linked to the pathogenesis of fibrosing alveolitis. By contrast, BAL neutrophil levels increase with increasingly extensive disease on CT, but they do not differ independently between CFA and FASSc, suggesting that neutrophil degradation products are unlikely to account for the excess mortality in CFA, compared with that in FASSc.

Bronchoalveolar Lavage Fluid↗

PubMed1998

Defective endothelially mediated pulmonary vasodilation in systemic sclerosis.

STUDY OBJECTIVES: Obliterative pulmonary vascular disease manifested clinically as pulmonary hypertension (PHT) may complicate systemic sclerosis (SSc). The aim of this study was to investigate possible endothelial dysfunction in patients with SSC complicated by PHT. DESIGN: Prospective, randomized trial. SETTING: Postgraduate teaching hospital. PATIENTS: Patients having SSc with PHT (SSc-PHT) and SSc without PHT (SSc), confirmed using Doppler echocardiography, and normal individuals (control subjects). INTERVENTIONS: I.v. infusion of the endothelially dependent vasodilator, substance P (maximum dose, 100 pmol/min), and the nonendothelially dependent vasodilator, adenosine (maximum dose, 0.05 mg/kg/min). MEASUREMENTS AND RESULTS: Effective pulmonary capillary blood flow (cardiac output minus right-to-left shunt) was measured in inert gas rebreathing, and calculated stroke index (SI) was used to reflect changes in pulmonary vascular resistance. During adenosine infusion, patients with SSc-PHT (n=5; mean age, 53+/-18 years) displayed a 25+/-16% increase in SI (p<0.05 compared with baseline), but no significant changes in SI were detected in the SSc (n=7; 54+/-6 years) or control (n=5; 35+/-5 years) groups. During infusion of substance P, SI rose by 32+/-18% in the control group at the maximum dose (p<0.05), but no change was observed in the SSc group. However, a fall in SI of -6+/-7% was detected in patients with SSc-PHT (p<0.05). CONCLUSIONS: Substance P-mediated pulmonary vasodilation is absent in patients with systemic sclerosis, suggesting that endothelial dysfunction occurs early in the course of the illness, but some responsiveness to adenosine remains.

Adenosine↗

PubMed1995

Abnormal ventricular long-axis function in systemic sclerosis.

STUDY OBJECTIVE: To assess possible effects of systemic sclerosis on ventricular function. DESIGN: Retrospective analysis of patients referred for echocardiographic examination to assess ventricular function. SETTING: Tertiary referral center for cardiac and chest diseases equipped with invasive and noninvasive facilities. PATIENTS: Thirty-four patients with clinical diagnosis of systemic sclerosis, aged 49 +/- 12 years; 24 had pulmonary fibrosis and 10 did not. There were 21 normal controls of similar age. MEASUREMENTS: Two-dimensional guided M-mode echocardiographic recordings of the left ventricular minor and long axis at the left and septal sites and right ventricle were obtained. Transmitral and transtricuspid Doppler flow velocities were also obtained with ECG and phonocardiogram. RESULTS: In 24 patients with pulmonary fibrosis, long-axis excursion was reduced 2.1 +/- 0.5 vs 2.7 +/- 0.4 cm/s as was peak rate of shortening and lengthening, 8.5 +/- 3.3 vs 10.8 +/- 2.4 cm/s and 7.5 +/- 2.5 vs 12 +/- 3.6 cm/s, respectively (p < 0.001), at the right side compared with 10 patients without. The onset of right long-axis shortening and lengthening was delayed with respect to the Q wave of the ECG and P2 of the phonocardiogram (p < 0.001 in both vs controls). The onset of tricuspid forward flow from the second heart sound was also delayed in the two groups, 110 +/- 15 ms and 100 +/- 20 ms vs 80 +/- 15 ms, respectively (p < 0.001). Right ventricular late diastolic filling velocities were increased 35 +/- 15 and 35 +/- 12 cm/s vs 20 +/- 10 cm/s in both groups (p < 0.01), and hence E:A ratio reduced 1.25 +/- 0.5 and 1.4 +/- 0.3 vs 1.9 +/- 0.4, respectively (p < 0.001). Pulmonary flow acceleration time was reduced only in patients with pulmonary fibrosis, 105 +/- 30 ms vs 125 +/- 30 ms (p < 0.001). At the left side, total long-axis excursion was reduced only in patients with pulmonary fibrosis (p < 0.01), while peak shortening and lengthening rates were reduced in both groups (p < 0.05). The onset of shortening from the Q wave and lengthening from the second heart sound were both delayed in the two groups with the latter greatly delayed in patients with pulmonary fibrosis (p < 0.05). CONCLUSIONS: Right and left ventricular long-axis function is frequently abnormal in patients with systemic sclerosis. Abnormalities are more profound in patients with CT evidence of pulmonary fibrosis than in those without. We suggest that these disturbances are due to myocardial fibrosis which, from the anatomic distribution of longitudinally directed fibers, is likely to have been subendocardial.

Blood Flow Velocity↗