Idiopathic Pulmonary Fibrosis: Prevailing and Evolving Hypotheses about Its Pathogenesis and Implications for Therapy

@article{Selman2001IdiopathicPF,
  title={Idiopathic Pulmonary Fibrosis: Prevailing and Evolving Hypotheses about Its Pathogenesis and Implications for Therapy},
  author={Moiss Selman and Talmadge E. King and Annie Pardo},
  journal={Annals of Internal Medicine},
  year={2001},
  volume={134},
  pages={136-151}
}
Idiopathic pulmonary fibrosis is a specific form of chronic fibrosing interstitial pneumonia limited to the lung. Although its cause remains unknown, advances in cellular and molecular biology have greatly expanded our understanding of the biological processes involved in its initiation and progression. Recently, an international consensus statement defining the diagnosis, evaluation, and treatment of patients with idiopathic pulmonary fibrosis was produced as a collaborative effort by the… Expand
Emerging drugs for idiopathic pulmonary fibrosis
TLDR
The treatment of IPF represents one of the greatest challenges confronting respiratory medicine and, currently, there is no effective therapeutic option for IPF, so new therapeutic approaches are urgently needed. Expand
Treatment of Idiopathic Pulmonary Fibrosis: A European Perspective
TLDR
The evolving theories of pathogenesis of IPF are explored, the evidence supporting traditional anti-inflammatory and recent antifibrotic and antioxidant therapies, the idiosyncrasies of past study design, and the need for standardized treatment guidelines are explored. Expand
Idiopathic pulmonary fibrosis
TLDR
The diagnostic and therapeutic aspects of idiopathic pulmonary fibrosis are reviewed, including the administration of anti-fibrotic drugs and pulmonary biopsies, and the best therapeutic option is pulmonary transplantation. Expand
Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?
TLDR
It is aimed to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. Expand
Idiopathic pulmonary fibrosis: current trends in management.
TLDR
It is hoped that ongoing molecular genetic studies and results of clinical trials will improve outcome of patients and families affected with this disease in the near future. Expand
Idiopathic pulmonary fibrosis
TLDR
Recent data on the clinical course, therapeutic options, and underlying mechanisms thought to be involved in the pathogenesis of idiopathic pulmonary fibrosis are reviewed. Expand
Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications
TLDR
It is shown that smoking, exposure to organic and inorganic dust and genetic factors have been shown to increase the risk of disease, although the cause of IPF remains elusive and its pathogenesis incompletely understood. Expand
Idiopathic pulmonary fibrosis
TLDR
Some recent advances in understanding of the pathophysiology of idiopathic pulmonary fibrosis are considered, which address the fate of the alveolar epithelial cell and mechanisms of fibrogenesis, and identify mechanistic pathways shared with co-existing conditions such as lung cancer and pulmonary hypertension. Expand
Diagnosis and management of idiopathic pulmonary fibrosis: Implications for respiratory care.
TLDR
Lung transplantation has been the only therapy shown to improve survival of those with idiopathic pulmonary fibrosis, and most patients are given trials of corticosteroids, alone or in combination with cytotoxic agents. Expand
Idiopathic pulmonary fibrosis: a treatable disease?
TLDR
The new concept is that IPF is a primary epithelial/fibroblastic disease, and inflammation is nowadays considered to be the secondary and not the primary event, and this new hypothesis is the basis for understanding the disappointing results with an isolated anti-inflammatory treatment. Expand
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 196 REFERENCES
Viruses and idiopathic pulmonary fibrosis.
TLDR
A review attempts to place into context the identification and potential role of viruses in patients with idiopathic pulmonary fibrosis and suggests that viruses may have a potential role in disease progression. Expand
Corticosteroids and the treatment of idiopathic pulmonary fibrosis. Past, present, and future.
TLDR
Data from the New Mexico ILD Registry and from other researchers suggest that the paucity of useful clinical data in IPF has resulted in a hodgepodge of diagnostic approaches and therapeutic criteria, as well as a fatalistic approach to treatment. Expand
Idiopathic pulmonary fibrosis: Diagnosis and treatment: International Consensus Statement
Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lungExpand
Interstitial Lung Disease
TLDR
This new edition of Interstitial Lung Disease includes clinical, pathologic, radiologic and physiologic evaluation of the patient with ILD and provides a basic pathobiology and a complete description of individual disease entities. Expand
Prognostic role of eosinophils in pulmonary fibrosis.
TLDR
The findings suggested that BAL eosinophilia may be a marker of progressive lung disease in patients with IPF and PF-CVD. Expand
Alveolitis: the key to the interstitial lung disorders.
TLDR
It is now recognised that, independent of the type of disease or specific aetiology, the earliest manifestation of interstitial disease is an "alveolitis"-the accumulation of inflammatory and immune effector cells within the alveolar structures. Expand
Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients.
TLDR
Despite its heterogeneity, NSIP deserves to be individualized as an original clinicopathologic entity and should be clearly distinguished from usual interstitial pneumonia, especially because of a better prognosis. Expand
Idiopathic pulmonary fibrosis: predicting response to therapy and survival.
TLDR
It is demonstrated that pretherapy pulmonary function, pathologic and radiographic parameters are different in individuals who respond to initial prednisone therapy and only HRCT imaging and pathologic fibrosis were able to reliably predict long-term survival in patients with biopsy-proven IPF. Expand
Familial idiopathic pulmonary fibrosis. Evidence of lung inflammation in unaffected family members.
TLDR
Alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis, and whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary Fibrosis is not yet known. Expand
Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis.
TLDR
Retrospective analysis of 104 patients with IPF who had open lung biopsy at Mayo Medical Center from 1976 to 1985 was performed to establish the overall survival rate, the spectrum of histopathological subgroups and their associated prognostic significance. Expand
...
1
2
3
4
5
...