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Idiopathic pulmonary fibrosis: diagnosis, management and new therapies

Guest Editor: Prof Luca Richeldi

Idiopathic pulmonary fibrosis (IPF) is the most common and most deadly type of interstitial lung disease. It is fatal and progressive, characterized by scarring and damage of lung tissue next to the alveoli. This process causes an irreversible loss of the tissue’s ability to exchange oxygen, which results in shortness of breath. Particularly at early stages, IPF causes similar symptoms as some other more common chronic lung diseases, which makes early diagnosis challenging.

IPF most commonly develops between the ages of 50 and 70, more frequently in men than in women and is associated with cigarette smoking. The disease has been reported worldwide with about 50,000 people dying of IPF each year in the US. The causes of this disease are not known, but recent evidence points to a relevant role for genetic factors, making IPF a multigenic disorder, likely linked to environmental triggers. Acute exacerbations represent relevant clinical events, which challenge clinicians with poor outcome and lack of effective management strategies.

The mainstream of IPF diagnosis is thoracic radiology, in particular high-resolution computed tomography, combined with lung biopsy in some cases. Current non-pharmacological management includes oxygen, rehabilitation and lung transplantion. Multiple mechanisms, including inflammation, immunity and oxidative stress have been empirically targeted over the last decades, by routinely using drugs like prednisone, azathioprine or N-acetylcysteine. However, when evaluated in the context of randomized controlled clinical trials, these approaches failed to show any benefit for patients. In a groundbreaking change of the IPF therapeutic landscape, recently two new treatments have been shown to be effective in reducing the disease progression: pirfenidone and nintedanib are becoming standards of care worldwide. Nonetheless, although we have finally entered a new era in the treatment of IPF, many unmet medical needs remain.

This article collection in BMC Medicine seeks to address the rapidly changing landscape in the field of IPF, by highlighting genetics, biomarker discovery, diagnostic approaches, clinical management, novel treatments, guidelines and regulatory issues. The collection will include original research articles as well as invited reviews and commentaries. If you have any research you would like us to consider for inclusion in this series, please email us at

  1. Commentary

    “Any fool can make a rule and any fool will mind it”

    In principle, accurate guideline recommendations should lead to optimal management based on a secure diagnosis. However, current IPF diagnostic guidelines do not meet the needs of a major sub-group (possibly t...

    Athol U. Wells

    BMC Medicine 2016 14:23

    Published on: 10 February 2016

  2. Commentary

    Idiopathic pulmonary fibrosis – clinical management guided by the evidence-based GRADE approach: what arguments can be made against transparency in guideline development?

    Evidence-based guidelines have undergone an incredible transformation over the last number of years. Significant advances include explicit linkages of systematic evidence summaries to the strength and directio...

    Bram Rochwerg, Holger J. Schünemann and Ganesh Raghu

    BMC Medicine 2016 14:22

    Published on: 10 February 2016

  3. Research article

    Treatment of idiopathic pulmonary fibrosis: a network meta-analysis

    Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease associated with high morbidity and mortality. Effective treatments for IPF are limited. Several recent studies have investigated novel therap...

    Bram Rochwerg, Binod Neupane, Yuan Zhang, Carlos Cuello Garcia, Ganesh Raghu, Luca Richeldi, Jan Brozek, Joseph Beyene and Holger Schünemann

    BMC Medicine 2016 14:18

    Published on: 3 February 2016

  4. Research article

    Evaluating the interstitial lung disease multidisciplinary meeting: a survey of expert centres

    Multidisciplinary meetings (MDM) are the current “gold standard” in interstitial lung disease (ILD) diagnosis and comprise inter-disciplinary discussion of multiple forms of information to provide diagnostic a...

    Helen E. Jo, Tamera J. Corte, Yuben Moodley, Kovi Levin, Glen Westall, Peter Hopkins, Daniel Chambers and Ian Glaspole

    BMC Pulmonary Medicine 2016 16:22

    Published on: 1 February 2016

  5. Research article

    Levels of circulating endothelial cells are low in idiopathic pulmonary fibrosis and are further reduced by anti-fibrotic treatments

    It has been suggested that circulating fibrocytes and endothelial cells actively participate in the intense remodelling of the pulmonary vasculature in patients with idiopathic pulmonary fibrosis (IPF). Indeed...

    Sara De Biasi, Stefania Cerri, Elena Bianchini, Lara Gibellini, Elisa Persiani, Gloria Montanari, Fabrizio Luppi, Cristiano Matteo Carbonelli, Luigi Zucchi, Marialuisa Bocchino, Alessandro Sanduzzi Zamparelli, Carlo Vancheri, Giacomo Sgalla, Luca Richeldi and Andrea Cossarizza

    BMC Medicine 2015 13:277

    Published on: 9 November 2015

  6. Opinion

    Interstitial lung diseases in the hospitalized patient

    Interstitial lung diseases (ILDs) are disorders of the lung parenchyma. The pathogenesis, clinical manifestations, and prognosis of ILDs vary depending on the underlying disease. The onset of most ILDs is insidio...

    Supparerk Disayabutr, Carolyn S. Calfee, Harold R. Collard and Paul J. Wolters

    BMC Medicine 2015 13:245

    Published on: 25 September 2015

  7. Editorial

    Idiopathic pulmonary fibrosis: moving forward

    Idiopathic pulmonary fibrosis (IPF) is the prototype of a large and heterogeneous group of pulmonary disorders, mainly chronic and progressive, usually known as interstitial lung disease (ILD). Over the last f...

    Luca Richeldi

    BMC Medicine 2015 13:231

    Published on: 24 September 2015

  8. Research article

    Relationship between fibroblastic foci profusion and high resolution CT morphology in fibrotic lung disease

    Fibroblastic foci profusion on histopathology and severity of traction bronchiectasis on highresolution computed tomography (HRCT) have been shown to be predictors of mortality in patients with idiopathic pulm...

    Simon L F Walsh, Athol U. Wells, Nicola Sverzellati, Anand Devaraj, Jan von der Thüsen, Samuel A. Yousem, Thomas V. Colby, Andrew G. Nicholson and David M. Hansell

    BMC Medicine 2015 13:241

    Published on: 24 September 2015

  9. Commentary

    Models of disease behavior in idiopathic pulmonary fibrosis

    Idiopathic pulmonary fibrosis is a diffuse parenchymal lung disease of unknown cause. The natural history of disease can vary considerably, making it difficult to predict the clinical trajectory for an individ...

    Kerri A. Johannson, Brett Ley and Harold R. Collard

    BMC Medicine 2015 13:165

    Published on: 24 September 2015

  10. Opinion

    The need for patient-centred clinical research in idiopathic pulmonary fibrosis

    Patient-centredness is an accepted term and is perceived by healthcare professionals to be morally and ethically desirable. We are motivated by the belief that this approach will improve the patient-profession...

    Anne-Marie Russell, Mirjam AG Sprangers, Steven Wibberley, Noel Snell, Daniel M. Rose and Jeff J. Swigris

    BMC Medicine 2015 13:240

    Published on: 24 September 2015