Idiopathic Pulmonary Fibrosis: Current Clinical Concepts and Challenges in Management

Esophageal involvement and pulmonary manifestations in systemic sclerosis

“…In this instance, because we observed a marked correlation between the degree of esophageal motor disturbances and endoscopic mucosal abnormalities, we did not include a 24-hour pH measurement as a direct proof of GER in the initial evaluation of our SSc patients. Our data are therefore in accordance with previous series suggesting that ILD may be a complication of GER (61)(62)(63)(64)69). Although many investigators have remarked that aspiration pneumonia does not cause definite remodeling of lung parenchymal structure and subsequent fibrosis, Mays et al (61), using barium radiography, have noted an increased prevalence of GER (54% vs. 8.5%) in patients with ILD compared with those without.…”

“…They have implicated microaspirations of gastric content into the lungs as the trigger mechanism in inducing pulmonary parenchymal lesions and have further noted that GER therapy could potentially improve symptoms and PFT parameters in these patients (54 -70). Similarly, esophageal motor disturbances have also been postulated as favoring parameters of ILD (61)(62)(63)(64)69). However, few investigators have previously explored the relationship between esophageal and lung manifestations in patients with SSc, and the results of these series are conflicting (36,38,39,71).…”

Cyclophosphamide pulse therapy in idiopathic pulmonary fibrosis

“…At least one patient died because of final pneumonia. Although this is a recognized complication of terminally ill IPF patients [2,5,26], it cannot be excluded that these pneumonias were induced by treatment, since cyclophosphamide is known to cause sustained immunosuppression (reviewed in [27]). In contrast to the clear advantage in reducing long-term toxicity [10,11], intermittent administration of cyclophosphamide is not superior to the daily regimen regarding infectious complications [27].…”

“…Neither BAUGHMAN and LOWER [8] nor JOHNSON et al [7] reported about any infection during intermittent or daily cyclophosphamide therapy in IPF. Because of the higher incidence of pneumonias in IPF, even without treatment, this raises some doubt [2,5,26]. Nevertheless, the incidence of respiratory tract infections is quite high and demands close follow-up of patients.…”

“…Kolb, J. Kirschner, W. Riedel, H. Wirtz, M. Schmidt aa Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor response to therapy and a prognosis similar to malignancies; the 5-yr survival rate is estimated at only 50% [1][2][3][4]. The pathogenesis of IPF involves abnormalities in the complex pathways of the immune system [2,5].…”