Current opinion in pulmonary medicine
-
Since the late 1990 s, when a more uniform definition of idiopathic pulmonary fibrosis (IPF) was proposed, more than 3000 patients have been enrolled in clinical studies exploring novel therapies. Some of the most relevant trials have been published only recently. ⋯ Despite multiple clinical trials, there is no strong, definitive evidence in favor of any agent to treat IPF. On the other hand, the placebo arms of these large trials have provided us with important critical information on the natural history of this disease. Clinical heterogeneity represents a critical issue to be taken into account in designing future clinical trials. The limited effectiveness of current treatment regimes has fuelled the search for a variety of new therapeutic approaches.
-
Review Comparative Study
Comparative manifestations and diagnostic accuracy of high-resolution computed tomography in usual interstitial pneumonia and nonspecific interstitial pneumonia.
Of the idiopathic interstitial pneumonias, the differentiation between idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonitis (NSIP) raises considerable diagnostic challenges, as their clinical presentations share many overlapping features. IPF is a fibrosing pneumonia of unknown cause, showing a histologic pattern of usual interstitial pneumonia (UIP), and has a poorer prognosis than does NSIP. This review examines whether the radiographic features of IFP and NSIP as assessed by high-resolution computed tomography (HRCT) can be used to distinguish between these two entities. ⋯ The finding of honeycombing as the predominant HRCT feature suggests the diagnosis of UIP and may exclude the need for biopsy. Predominant features of GGOs are not specific enough to distinguish between NSIP and UIP.
-
Surveillance of hepatic involvement in sarcoidosis has not been standardized. Therefore, management of hepatic involvement is a clinical challenge. This review analyses published data on the pharmacological treatment of hepatic sarcoidosis. ⋯ This review illustrates the importance of an appropriate therapeutic approach of sarcoidosis patients with hepatic involvement. It emphasizes the need for future studies to evaluate treatment options to avoid disease progression and hepatic complications.
-
Familial pulmonary fibrosis has long been recognized and suggests that pulmonary fibrosis may have a genetic origin in some cases with an autosomal dominant transmission. ⋯ These genes point to alveolar epithelium injury and repair as a major component of the fibrotic process.
-
This review emphasizes key findings in physiologic research of sarcoidosis reported over the past year. ⋯ Periodic assessment of HRQOL measures, exercise-induced hypoxemia, and right-sided cardiac pressures for pulmonary hypertension provides, to date, the best insight into the magnitude of physiologic impairment, serving as guideposts for management (including lung transplantation) and prognosis.