Idiopathic Pulmonary Fibrosis

Seminars in Respiratory and Critical Care Medicine (Impact Factor: 2.71). 09/2012; 33(05):433-439. DOI: 10.1055/s-0032-1325154


Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive, chronic fibrosing interstitial lung disease occurring primarily in older adults and limited to the lungs. The prognosis is dire, with half of all patients progressing to death from respiratory failure within 3 to 5 years from initial diagnosis. The pathogenesis of IPF is complex and incompletely understood, and the natural history of this disease is variable and unpredictable. There is no widely accepted treatment for IPF, except for lung transplantation. Nevertheless, these are important and exciting times for the diagnosis and management of patients with IPF. Novel diagnostic approaches (e.g., biomarker-based), improved prognostic models, and clinical trials of novel drug agents may alter disease management substantially over the next few years. For now, clinicians should practice comprehensive management, including symptom-based management, aggressive management of comorbidities, and patient education and support.

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    ABSTRACT: Pulmonary hypertension (PH) may complicate parenchymal lung disease, specifically interstitial lung diseases and chronic obstructive pulmonary disease, and uniformly increases the mortality risk. The epidemiology and degree of PH is variable and unique to the underlying lung disease. The clinician should exercise a high index of suspicion for PH complicating parenchymal lung disease especially given the nonspecific symptomatology and the limitations of echocardiography in this patient population. In general, PH-specific therapies in this setting have been poorly studied, with concern for increased shunting and/or ventilation/perfusion (V/Q) mismatch and resultant hypoxemia. A better understanding of the mechanisms underlying PH related to parenchymal lung disease may lead to novel pharmacological targets to prevent or treat this serious complication.
    Seminars in Respiratory and Critical Care Medicine 10/2013; 34(5):600-19. DOI:10.1055/s-0033-1356548 · 2.71 Impact Factor
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    ABSTRACT: Patients with idiopathic pulmonary fibrosis (IPF) often do worse following infection, but the cause of the decline is not fully understood. We previously demonstrated that infection with a murine gamma herpes virus (γHV-68) could exacerbate established lung fibrosis following administration of fluorescein isothiocyanate. In this study, we anesthetized mice and injected saline or bleomycin intratracheally on day 0. On day 14, mice were anesthetized again and infected with either a Gram negative bacteria (Pseudomonas aeruginosa), or with H1N1 or γHV-68 viruses. Measurements were then made on days 15, 21 or 35. We demonstrate that infection with P. aeruginosa does not exacerbate extracellular matrix deposition post-bleomycin. Furthermore, fibrotic mice are effectively able to clear P. aeruginosa infection. In contrast, bleomycin-treated mice develop worse lung fibrosis when infected with γHV-68, but not when infected with H1N1. The differential ability of γHV-68 to cause increased collagen deposition could not be explained by differences in inflammatory cell recruitment or whole lung chemokine and cytokine responses. Alveolar epithelial cells from γHV-68-infected mice displayed increased expression of TGFβ receptor 1, increased SMAD3 phosphorylation and evidence of apoptosis measured by cleaved poly ADP ribose polymerase (PARP). The ability of γHV-68 to augment fibrosis required the ability of the virus to reactivate from latency. This property appears unique to γHV-68, as the β herpes virus, cytomegalovirus, did not have the same effect.
    AJP Lung Cellular and Molecular Physiology 05/2014; 307(3). DOI:10.1152/ajplung.00300.2013 · 4.08 Impact Factor
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    ABSTRACT: Background: Palliative care, integrated early, may reduce symptom burden in patients with idiopathic pulmonary fibrosis (IPF). However, limited information exists on timing and clinical practice. The purpose of this study was to describe the time course of events prior to death in patients with IPF managed at a specialty center with a focus on location of death and timing of referral for palliative care. Methods: Data were retrospectively extracted from the health system's data repository and obituary listings. The sample included all decedents, excluding lung transplant recipients, who had their first visit to the center between 2000 and 2012. Results: Median survival for 404 decedents was 3 years from diagnosis and 1 year from first center visit. Of 277 decedents whose location of death could be determined, > 50% died in the hospital (57%). Only 38 (13.7%) had a formal palliative care referral and the majority (71%) was referred within 1 month of their death. Decedents who died in the academic medical center ICU were significantly younger than those who died in a community hospital ward (P = .04) or hospice (P = .001). Conclusions: The majority of patients with IPF died in a hospital setting and only a minority received a formal palliative care referral. Referral to palliative care occurred late in the disease. These findings indicate the need to study adequacy of end-of-life management in IPF and promote earlier discussion and referral to palliative care.
    Chest 09/2014; 147(2). DOI:10.1378/chest.14-1127 · 7.48 Impact Factor
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