Lungs in progressive systemic sclerosis

On the Respiratory Mechanics Measured by Forced Oscillation Technique in Patients with Systemic Sclerosis

“…These results might be explained by a development of peribronchial fibrosis that is affecting the bronchial wall, which contributes to a reduction of the airway caliber [8], [59]–[62]. In agreement with our results, Blom-Bülow et al [52] reported an increase of the pulmonary resistance by 33% in patients with SSc, and they attributed this finding to an increase of the viscous resistance of the lung tissue combined with peribronchial fibrosis.…”

“…This parameter is related with the total mechanical load of the respiratory system, including the resistive and elastic effects observed in 4 Hz. These results are consistent with the physiopathology of SSc because we found alterations in both the resistive and reactive properties of the respiratory system [6]–[8], [15], [19], [59], [60], [62], [63], [66]. The increase of Z4Hz reflects an increase in respiratory effort, which might be the origin of dyspnea and the consequential reduction of the quality of life that is usually reported by patients diagnosed with SSc.…”

“…As can be observed in Figures 4D and 7D, the analysis based on the eRIC model also resulted in reduced values of compliance in patients with SSc. These results clearly reflects the physiopathology of SSc, which includes diffuse fibrosis affecting the interstitium and alveolar septa, thereby changing their architecture and the bronchial wall [6]–[8], [12], [15], [19], [59], [62], [63], [66]. This accurate description of the physiopathology of SSc afforded by Crs,dyn allows us to infer that the FOT might be an alternative method to quantitatively and non-invasively assess the elastic properties of the respiratory system in these individuals, thus avoiding the use of invasive techniques, such as that based on esophageal balloon.…”

“…However, we did not find modifications of this parameter with the classification based on the lung volumes (Figure 5C). Possibly, the results described in Figure 2C are due to extensive peribronchial fibrosis, which causes a reduction of the internal airway diameter in patients with SSc [8], [59], [61]–[64]. A further relevant factor might be the fibrotic process that results in a reduction of the lung volumes and a consequential anatomical narrowing of the airway [64].…”

Atemfunktionsprüfungen bei Sklerodermie (progressiver Systemsklerose)

“…Das Bestehen der Fibrose wurde auch durch das ständig anwachsende Autopsiematerial bestätigt. Erkrankungen an PSS sind selten, doch ist die Anzahl der Mitteilungen, die sich mit ihrer kardiorespiratorischen Funktion befassen, erstaunlich hoch, obwohl die Untersuchungen sich selbstverständlich nicht auf ein grosses Krankenmaterial erstrecken konnten [1,3,6,7,9,10,12,13,23,30], Mit unseren jetzigen Untersuchungen wollten wir eine Antwort auf die Fragen finden: 1. ob die Spirometrie und die atemmechanischen Untersu chungen dazu geeignet wären, die pulmonalen und extrapulmonalen Fak toren der Funktionsstörungen zu differenzieren, und 2. gibt es einen Unter schied zwischen den spirometrischen und den Atemfunktions-Messgrössen einer rein pulmonal bedingten Atemfunktionsstörung, wie der fibrotisierenden Alveolitis (Hamman-Rich-Syndrom), und denen bei Sklerodermie.…”