Chest
Recent Advances in Chest MedicineRecent Advances in Idiopathic Pulmonary Fibrosis
Section snippets
Diagnostic Issues
It is important to accurately diagnose IPF given the prognostic differences among the various idiopathic interstitial pneumonias. There is a growing body of literature addressing the diagnostic approach to these patients. Pivotal to the diagnoses of idiopathic interstitial pneumonias are refinements in the roles of high-resolution CT (HRCT) scanning and surgical lung biopsy (SLB).
Does HRCT Scanning Aid in Diagnosis?
HRCT scanning has improved the ability to make a definitive diagnosis without performing a biopsy in patients with some DPLDs, particularly IPF. Furthermore, the HRCT scanning aids in determining prognosis and may aid in identifying disease progression. As such, the community at large has adopted its use more definitively than ever before.6 But some caution must be exercised, as breadth of experience seems to matter.
A chest radiographic study usually demonstrates honeycombing and reticular
What Is the Value of HRCT Scanning in Defining Prognosis in IPF?
The extent and pattern of fibrosis on HRCT scans carry prognostic implications. For example, a multivariate analysis of a large multicenter trial24 suggested that a higher extent of fibrosis on HRCT scans was an independent predictor of mortality. When using a visual scoring method, IPF exhibits a progressive deterioration both in the extent of disease seen on HRCT scans and in lung function impairment over time, although the changes were subtle and evolved gradually. In clinical practice, the
Utility of SLB in IPF
Given the diagnostic limitations of imaging and the prognostic importance of a UIP diagnosis, lung biopsy continues to play an important role in the evaluation of a patient with DPLD.
Specific Clinical Situations Affecting Prognosis
As information continues to accrue, it has become clearer that a marked heterogeneity in disease progression exists in IPF patients. Many investigative groups have searched for indicators of disease progression. Among these are acute exacerbations of IPF and the development of pulmonary hypertension.
Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is a well-recognized sequela in patients with collagen vascular-associated DPLDs and in patients with DPLDs in general. The role of PAH in IPF is slowly being elucidated.
Therapeutic Approaches
The therapeutic approach to IPF is rapidly evolving, as reviewed by others.5758 In large part, the evolving nature of therapeutic approaches reflects the evolution of concepts regarding the biological processes that result in IPF.596061
Conclusions
The last few years have seen measurable advancements in the approach to diagnosis and defining prognosis in IPF patients. The diagnosis of IPF involves integration of the clinical history, and radiology and pathology findings. Within this triad, typical HRCT scan findings in the presence of a consistent clinical picture can allow for a confident diagnosis in the absence of a lung biopsy. The HRCT scan appearance may also provide prognostic information; an IPF patient with typical HRCT scan
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Atractylodin ameliorates bleomycin-induced pulmonary inflammation and fibrosis in mice
2023, Pharmacological Research - Modern Chinese MedicineChronic diffuse lung diseases
2022, Practical Pulmonary Pathology: A Diagnostic ApproachProtective effects of Rosavin on bleomycin-induced pulmonary fibrosis via suppressing fibrotic and inflammatory signaling pathways in mice
2019, Biomedicine and PharmacotherapyCitation Excerpt :In the early stage of IPF, it mainly manifested as alveolitis and a large number of inflammatory cells infiltrated. In the later stage, a large number of fibroblasts proliferated and collagen accumulated, which gradually developed into irreversible pulmonary interstitial fibrosis [24,25]. Lung index is one of the indexes reflecting the degree of PF [26].
Overview of the Chronic Diffuse Interstitial Lung Diseases
2018, Practical Pulmonary Pathology: A Diagnostic Approach A Volume in the Pattern Recognition SeriesEmodin alleviates bleomycin-induced pulmonary fibrosis in rats
2016, Toxicology Letters
This research was supported in part by National Institutes of Health, National Heart, Lung, and Blood Institute grants P50HL-56402 and 2 K24 HL04212.
Dr. Noth has received grants, honoraria, or advisory fees from Actelion, Boehringer-Ingelheim, Cotherix, Centocor, GenZyme, GlaxoSmithKline, Intermune, Novartis Pfizer, and Roche. Dr. Martinez has received grants, honoraria and advisory fees from GlaxoSmithKline. He has also received honoraria and advisory fees from Boehringer Ingleheim, Pfizer, Novartis, Dey, and Schering Plough. He is a consultant for Altana Pharma.