Abstract
BackgroundIn scleroderma, excessive collagen production can alter tracheal geometry, and computed tomography (CT) volumetry of this structure may aid in detecting possible abnormalities. The objectives of this study were to quantify the morphological abnormalities in the tracheas of patients with scleroderma and to correlate these findings with data on clinical and pulmonary function.MethodsThis was a cross-sectional study in which 28 adults with scleroderma and 27 controls matched by age, gender and body mass index underwent chest CT with posterior segmentation and skeletonization of the images. In addition, all participants underwent pulmonary function tests and clinical evaluation, including the modified Rodnan skin score (mRSS).ResultsMost patients (71.4%) had interstitial lung disease on CT. Compared to controls, patients with scleroderma showed higher values in the parameters measured by CT trachea volumetry, including area, eccentricity, major diameter, minor diameter, and tortuosity. The tracheal area and equivalent diameter were negatively correlated with the ratio between forced expiratory flow and forced inspiratory flow at 50% of forced vital capacity (FEF50%/FIF50%) (r = -0.44, p = 0.03 and r = -0.46, p = 0.02, respectively). The tracheal tortuosity was negatively correlated with peak expiratory flow (r = -0.51, p = 0.008). The mRSS showed a positive correlation with eccentricity (r = 0.62, p < 0.001) and tracheal tortuosity (r = 0.51, p = 0.007), while the presence of anti-topoisomerase I antibody (ATA) showed a positive correlation with tracheal tortuosity (r = 0.45, p = 0.03).ConclusionsIn a sample composed predominantly of scleroderma patients with associated interstitial lung disease, there were abnormalities in tracheal geometry, including greater eccentricity, diameter and tortuosity. In these patients, abnormalities in the geometry of the trachea were associated with functional markers of obstruction. In addition, tracheal tortuosity was correlated with cutaneous involvement and the presence of ATA.
Highlights
Scleroderma or systemic sclerosis (SSc) is a chronic progressive autoimmune disease of connective tissue characterized by microvascular involvement, activation of the immune system, and increased deposition of extracellular matrix in the skin and internal organs by excess collagen fibers, leading to fibrosis [1,2,3,4]
The tracheal area and equivalent diameter were negatively correlated with the ratio between forced expiratory flow and forced inspiratory flow at 50% of forced vital capacity (FEF50%/FIF50%) (r = -0.44, p = 0.03 and r = -0.46, p = 0.02, respectively)
Abnormalities in the geometry of the trachea were associated with functional markers of obstruction
Summary
Scleroderma or systemic sclerosis (SSc) is a chronic progressive autoimmune disease of connective tissue characterized by microvascular involvement, activation of the immune system, and increased deposition of extracellular matrix in the skin and internal organs by excess collagen fibers, leading to fibrosis [1,2,3,4]. Interstitial lung involvement is subclinical and asymptomatic at early stages in most patients, interstitial lung disease (ILD) associated with scleroderma (ILD-SSc) is observed in approximately 40% of cases and is a major cause of morbidity and mortality [6]. Ooi et al [7] reported that scleroderma affected small and large airways in 45–100% of patients. In this context, the trachea has been a ’forgotten zone’ in the study of several diseases because the pathological processes involving this structure have often not received the necessary clinical recognition. The objectives of this study were to quantify the morphological abnormalities in the tracheas of patients with scleroderma and to correlate these findings with data on clinical and pulmonary function
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