Pulmonary Fibrosis (Hamman Rich Syndrome)

A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.

Also Known As:

Networked: 4280 relevant articles (166 outcomes, 597 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Fibrosis (Cirrhosis)
2.	Lung Injury
3.	Idiopathic Pulmonary Fibrosis
4.	Systemic Scleroderma (Systemic Sclerosis)
5.	Anoxia (Hypoxia)

Experts

1.	Phan, Sem H: 29 articles (11/2015 - 01/2002)
2.	Sato, Shinichi: 20 articles (12/2015 - 11/2002)
3.	Azuma, Arata: 18 articles (08/2015 - 10/2002)
4.	Hogaboam, Cory M: 18 articles (05/2014 - 05/2003)
5.	Crestani, Bruno: 17 articles (08/2015 - 08/2002)
6.	Oury, Tim D: 17 articles (11/2011 - 10/2003)
7.	Gauldie, Jack: 16 articles (07/2015 - 08/2002)
8.	Kaminski, Naftali: 15 articles (12/2015 - 04/2002)
9.	Zhang, Wei: 15 articles (12/2015 - 07/2006)
10.	Liu, Tianju: 15 articles (11/2015 - 05/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Fibrosis:

1.	Bleomycin (Blenoxane)FDA LinkGeneric 12/01/2015 - "MSCs are beneficial in rodent bleomycin pulmonary fibrosis models. " 12/01/2015 - "To shed light on the potential usefulness of MSCs for human disease, we aimed to systematically review the preclinical literature to determine if MSCs are beneficial in animal bleomycin pulmonary fibrosis models. " 08/01/2014 - "We investigated the efficacy of natural products with high anti-inflammatory activity, such as passion fruit peel extract (PFPE), in a mouse model of bleomycin-induced pulmonary fibrosis (PF). " 03/01/2012 - "This study developed novel sequences of siRNAs targeting the TGF-β1 gene and evaluated their therapeutic efficacy in two models of pulmonary fibrosis: a model induced by bleomycin and a novel model of the disease developed spontaneously in mice overexpressing the full length of human TGF-β1 in the lungs. " 12/01/2014 - "Intralesional bleomycin has been reported to be effective in microcystic LM; however, its use is limited by concerns about pulmonary fibrosis. "
2.	CollagenIBA 08/01/2012 - "Work of breathing can detect decrements in lung function due to pulmonary fibrosis, correlates well with the amount of collagen in the lungs, and may be a more sensitive quantitative measure of efficacy for drugs being developed to treat pulmonary fibrosis." 01/01/2013 - "In this study, we investigated SAHA's effects on the expression of collagen III alpha 1 (COL3A1) in primary human IPF fibroblasts and in a murine model of pulmonary fibrosis. " 04/01/2009 - "The goal of the present study was to examine the involvement of JNK1 in subepithelial collagen deposition in mice subjected to models of allergic airways disease and interstitial pulmonary fibrosis. " 05/01/2008 - "Our previous study suggested that SOCS1 regulates collagen synthesis by lung fibroblasts, suggesting a role of SOCS1 in the pathophysiology of pulmonary fibrosis. " 12/01/2003 - "The purpose of this study was to establish whether PARC could be directly involved in development of pulmonary fibrosis by stimulating collagen production in lung fibroblasts. "
3.	indium-bleomycinIBA 10/01/2013 - "The purpose of the study is to investigate the potential therapeutic efficacy of LEF in bleomycin (BLM) induced pulmonary fibrosis. " 08/01/2015 - "The study was aimed to investigate the mechanism and administration timing of bone marrow-derived mesenchymal stem cells (BMSCs) in bleomycin (BLM)-induced pulmonary fibrosis mice. " 07/01/2015 - "Our previous study has found that eIF3a is involved in bleomycin-induced pulmonary fibrosis. " 01/01/2013 - "Despite some murine strain variation in the expression of apoptotic regulatory genes in bleomycin-induced pulmonary fibrosis, the results of the present study revealed no significant differences in alveolar epithelial apoptosis between NMRI and C57BL/6 black mice. " 09/07/2012 - "In this study, we used heterozygous ORP150-deficient mice (ORP150(+/-) mice) to examine the role of ORP150 in bleomycin-induced pulmonary fibrosis. "
4.	Transforming Growth Factor beta (TGF-beta)IBA 01/01/2004 - "We used Affymetrix oligonucleotide microarrays U95v2, containing approximately 12,000 human genes, to study the transcriptional profile in response to a four hour treatment with TGFbeta in control lung fibroblasts and in fibroblasts from patients with idiopathic and scleroderma-associated pulmonary fibrosis. " 07/04/2003 - "Surprisingly, despite previous studies that characterized TbetaRIIDeltak as a dominant negative inhibitor of TGFbeta signaling, adult mice expressing this construct demonstrated TGFbeta overactivity and developed dermal and pulmonary fibrosis. " 05/01/2012 - "The signaling pathway associated with pulmonary fibrosis is reliant on transforming growth factor-beta 1 (tgf-β(1)) activation of Smad3, as evidenced by Smad3-deficient mice being resistant to tgf-β(1)-induced pulmonary fibrosis. " 01/01/2012 - "However, once alveolarization is sufficiently advanced as in mid to late gestation fetal monkey, late gestation human or adult mouse, rat or human lung, excessive TGF beta signaling results in pulmonary fibrosis. " 01/01/2012 - "Transforming growth factor-beta (TGF-beta) is a powerful activator of connective tissue synthesis and fibroblast proliferation in the lung, and a critical paracrine signal for the development of pulmonary fibrosis. "
5.	CytokinesIBA 11/01/2015 - "Three radiation-induced profibrotic cytokines in the radiation with vehicle-treated group were significantly reduced by TPL treatment, and this partly contributed to the trend of improved survival rate and pulmonary density and function and the decreased severity of pulmonary fibrosis at 5 months. " 11/01/2015 - "This study aimed to investigate the effects of Treg depletion on irradiation-induced pulmonary fibrosis and on fibrocyte, Th17 cell response and production of multiple cytokines in mice. " 10/01/2003 - "Previous studies have shown that the development of pulmonary fibrosis is accompanied by enhanced expression of Th2-profile cytokines, especially IL-4, and diminished expression of Th1 cytokines, including IFN-gamma. " 09/01/2003 - "Data from both human and animal studies are now emerging to suggest that members of this cytokine family play an important role in the pathogenesis of fibroproliferative diseases and thus represent a novel group of cytokines implicated in pulmonary fibrosis. " 01/01/1998 - "Recent studies have identified a myriad of cytokines with potential roles in pulmonary fibrosis. "
6.	Silicon Dioxide (Sand)FDA LinkGeneric 10/09/2013 - "In conclusion, our study demonstrated that silica could suppress the Trx system to perturb the redox balance, elicit oxidative stress, and eventually induce pulmonary fibrosis. " 07/04/2013 - "There is no study to investigate BMP-7's role in the development of pulmonary fibrosis induced by silica. " 02/01/2012 - "The objective of this study was to assess both the capacity of human embryonic stem cells (HUES-3) to differentiate in vitro into ATII cells and the ability of committed HUES-3 cells (HUES-3-ATII cells) to recover in vivo a pulmonary fibrosis model obtained by silica-induced damage. " 01/01/2010 - "Aside from the difference in the fibrotic pattern, these studies showed no indicators of RAGE having an effect on the severity of pulmonary fibrosis following silica injury." 01/01/2010 - "In the current study the role of RAGE in a mouse model of silica induced pulmonary fibrosis was investigated. "
7.	AsbestosIBA 10/01/1990 - "This study suggests that the most common radiographic findings in asbestos-exposed, isolated pleural plaques are associated with a significant reduction in FEV1 and FVC, which cannot be attributed to the presence of radiographic and subradiographic pulmonary fibrosis." 11/01/2009 - "In this study, we demonstrate that Rac1 null mice are protected from asbestos-induced pulmonary fibrosis, as determined by histological and biochemical analysis. " 02/01/2005 - "The aim of the study was to determine the causes of impairment of ventilatory function and diffusing capacity in smoking asbestos-exposed workers (N=590) showing radiological pleural thickenings or pulmonary fibrosis. " 06/01/2004 - "The aim of this study was to determine criteria for independently associated features of pulmonary fibrosis in high-resolution computed tomograms among persons occupationally exposed to asbestos. " 08/01/1990 - "Although there is a correlation between the tissue concentration of asbestos fibers and the severity of pulmonary fibrosis, further studies of the mineralogic correlates of fiber-induced pulmonary fibrosis are needed. "
8.	pirfenidoneIBA 06/01/2005 - "Pirfenidone is an investigational anti-fibrotic agent shown to be effective in blocking pulmonary fibrosis. " 06/01/2011 - "The current, randomized, placebo-controlled, prospective, double-blind trial investigated the safety and efficacy of pirfenidone for mild to moderate HPS-1 and 4 pulmonary fibrosis. " 01/01/2012 - "Adverse events of pirfenidone for the treatment of pulmonary fibrosis: a meta-analysis of randomized controlled trials." 07/01/2014 - "Pirfenidone is an antifibrotic agent for patients with pulmonary fibrosis, but this drug has adverse gastrointestinal (GI) effects. " 01/01/2014 - "CT can be used to assess pirfenidone-induced slowing of progression of pulmonary fibrosis."
9.	Hydroxyproline (4 Hydroxyproline)IBA 02/01/1993 - "Furthermore, anti CD-11a mAb was also effective when given on Days 20 and 25 and the lung hydroxyproline content determined on Day 30 after instillation, i.e., in the treatment of an established pulmonary fibrosis. " 03/01/2008 - "In contrast to other diseases in which RAGE signaling promotes pathology, immunohistochemical and hydroxyproline quantification studies on aged RAGE-null mice indicate that these mice spontaneously develop pulmonary fibrosis-like alterations. " 01/01/2015 - "Pulmonary fibrosis was consistently reduced in PAR-2-deficient mice throughout the fibrotic phase, as evident from reduced Ashcroft scores (29%) and hydroxyproline levels (26%) at d 28. " 01/01/2015 - "The development of pulmonary fibrosis was characterized using hydroxyproline assay and histological staining. " 07/01/2014 - "The severity of pulmonary fibrosis was assessed by histopathologic evaluation and determination of hydroxyproline levels. "
10.	CurcuminIBA 04/01/2006 - "To study the protective effects of curcumin on exaggerated extracellular matrix accumulation of pulmonary fibrosis rats. " 04/01/2006 - "[Experimental study on protective effects of curcumin on exaggerated extracellular matrix accumulation of pulmonary fibrosis rats]." 05/01/2010 - "Dietary curcumin increases antioxidant defenses in lung, ameliorates radiation-induced pulmonary fibrosis, and improves survival in mice." 04/01/2006 - "To study on the effect and mechanism of curcumin on inhibiting injury induced by free radical in pulmonary fibrosis. " 04/01/2006 - "[Experimental study on effect of curcumin on inhibiting injury of free radical in pulmonary fibrosis rats]."

Therapies and Procedures

1.	Lung Transplantation 01/01/2009 - "Lung transplantation in pulmonary fibrosis: challenging early outcomes counterbalanced by surprisingly good outcomes beyond 15 years." 06/01/2013 - "The purpose of this study was to determine the influence of changes in pulmonary artery pressure during the waiting period on survival after lung transplantation for pulmonary fibrosis. " 12/01/2007 - "This study investigates factors influencing the chance of patients with pulmonary fibrosis reaching lung transplantation. " 07/01/2000 - "This study reviews this experience and compares single lung transplantation and bilateral lung transplantation for pulmonary fibrosis. " 11/01/2015 - "On visualization, the donor's lung was acceptable for lung transplantation to a 68-year-old male patient with a chronic history of pulmonary fibrosis. "
2.	Drug Therapy (Chemotherapy) 09/01/2014 - "Pulmonary Fibrosis Secondary to FOLFOX Chemotherapy: A Case Report." 01/01/2013 - "The evolving pharmacotherapy of pulmonary fibrosis." 04/01/2011 - "Except one patient who had asymptomatic pulmonary fibrosis, chest computed tomography before chemotherapy showed no fibrotic changes. " 01/01/2011 - "He developed pulmonary fibrosis during the course of his chemotherapy, and therefore further chemotherapy was stopped. " 01/01/2009 - "Pulmonary fibrosis associated with psychotropic drug therapy: a case report."
3.	Transplantation (Transplant Recipients) 02/01/2014 - "There are several reports on therapeutic effects of adult stem cell transplantations in animal models of pulmonary fibrosis. " 11/01/2009 - "The decreased levels of IFNgamma in lung tissues following transplantation might be associated with pulmonary fibrosis in late non-infectious pulmonary complications." 12/01/2007 - "Patients with pulmonary fibrosis who died awaiting transplantation had similar disease severity at assessment as those who achieved transplantation. " 10/01/2007 - "No extrarenal manifestations were identified, although two patients developed lethal pulmonary fibrosis postrenal transplantation. " 06/01/2005 - "The post-transplantation survival in patients with pulmonary fibrosis is about 65-70% at one year and 40% at five years. "
4.	Transplants (Transplant) 06/01/2015 - "Running short on time: lung transplant evaluation for telomere-related pulmonary fibrosis." 04/01/2015 - "There were 9 patients (7 men) with TERT (n = 6) or TR (n = 3) mutations who received a single (n = 8) or a double (n = 1) lung transplant for pulmonary fibrosis. " 01/01/2015 - "Identification of short telomeres has potential clinical implications in pulmonary fibrosis: it may be a marker for an increased predisposition toward the development of the disease, it might affect risk stratification as it has been associated with lower survival rates and post-transplant complications that reflect the syndromic nature of this molecular defect." 07/01/2014 - "While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients." 12/01/2007 - "Between March 1999 and March 2004, 129 patients with pulmonary fibrosis underwent formal transplant assessment. "
5.	Aftercare (After-Treatment) 11/01/2010 - "Ten weeks after treatment pulmonary fibrosis was still present. " 06/01/2002 - "Mice receiving 740 kBq of 211At-labeled monoclonal antibody 201B developed pulmonary fibrosis 3-4 months after treatment, as did mice treated with 3700 kBq of the alpha-particle emitter 213Bi-labeled monoclonal antibody 201B in previous work. " 09/01/1994 - "After treatment, her chest roentgenogram revealed severe pulmonary fibrosis and loss of lung volume." 10/01/1989 - "Pulmonary fibrosis has been reported after treatment with this drug. " 11/01/2013 - "Patients who had survived for more than 5 years after treatment were included in an analysis of late adverse events (excluding radiation pneumonitis and pulmonary fibrosis). "