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Spagnolo P.,University of Modena and Reggio Emilia | Sverzellati N.,University of Parma | Wells A.U.,Interstitial Lung Disease Unit | Hansell D.M.,Royal Brompton Hospital
European Radiology | Year: 2014

Sarcoidosis is a systemic granulomatous disorder of unknown aetiology with a wide spectrum of radiological appearances and almost invariably pulmonary involvement. Lung involvement accounts for most of the morbidity and much of the mortality associated with sarcoidosis. Imaging contributes significantly to the diagnosis and management of patients with sarcoidosis. In typical cases, chest radiography may be sufficient to establish the diagnosis with little margin of error and CT is not necessary. However, CT can play a critical role in several clinical settings: atypical clinical and/or radiographic findings; normal or near-normal chest radiograph but clinical suspicion of sarcoidosis; and detection of complications. Moreover, in many patients, CT findings are atypical and unfamiliar to most radiologists (e.g. sarcoidosis mimicking other lung diseases and vice versa), and in these cases histological confirmation of the diagnosis is recommended. CT is also useful in assessing disease extent and may help to discriminate between reversible and irreversible lung disease, thus providing critical prognostic information. This review concentrates on the more difficult imaging aspects of sarcoidosis, in particular differential diagnosis and disease complications. Key points: • Sarcoidosis is characterized by a wide spectrum of radiological appearances. • In typical cases, imaging substantially contributes to the diagnosis of sarcoidosis. • CT plays a critical role in atypical and complicated cases. • CT may discriminate between reversible and irreversible lung disease. © 2014 European Society of Radiology. Source

Wells A.U.,Interstitial Lung Disease Unit
European Respiratory Review | Year: 2013

Idiopathic pulmonary fibrosis (IPF), the most prevalent of the idiopathic interstitial pneumonias, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for its optimal management. The 2011 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway, with formal designation of criteria for an HRCT pattern of usual interstitial pneumonia. In the correct clinical context, a usual interstitial pneumonia pattern on HRCT is indicative of a definite diagnosis of IPF and negates the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of the guideline recommendations by clinicians has identified limitations that should be addressed in future statements. Key problems include: 1) HRCT misdiagnosis, particularly by less experienced radiologists; 2) lack of management recommendations for the highly prevalent clinical scenarios of probable or possible IPF; 3) ongoing confusion concerning the diagnostic role of bronchoalveolar lavage; and 4) the lack of integration of clinical data in the designation of the diagnostic likelihood of IPF, including the treated course of disease. These issues become evident as the recommendations are applied and highlight the need for continued guideline adjustments. © ERS 2013. Source

Staitieh B.S.,Emory University | Renzoni E.A.,Interstitial Lung Disease Unit | Veeraraghavan S.,Emory University
Annals of Medicine | Year: 2015

Idiopathic pulmonary fibrosis (IPF) is a severe, progressive fibrotic disease of the lung of unknown etiology that affects approximately 150,000 patients in the United States. It carries a median survival of two to three years, but clinical course can vary markedly from patient to patient. There has been no established treatment for IPF, but recent advances in coordinated clinical trials through groups such as IPFnet and academia-industry partnerships have allowed this relatively rare disease to be studied in much greater depth. Historically, the default therapy for IPF was a combination of prednisone, N-acetylcysteine, and azathioprine, but recent trials have shown that this regimen actually increases mortality. An enormous body of work in recent years, spanning the bench to the bedside, has radically altered our understanding of the molecular mechanisms underlying IPF. Newer modalities, particularly those involving monoclonal antibodies targeted at specific pathways known to contribute to the fibrotic process, have generated a great deal of excitement in the field, and recent clinical trials on therapies such as pirfenidone and nintedanib herald a new era in targeted IPF therapies. © 2015 Informa UK, Ltd. Source

Maher T.M.,Interstitial Lung Disease Unit | Maher T.M.,University College London | Maher T.M.,Imperial College London
European Respiratory Review | Year: 2013

Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF), diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints) study is a currently enrolling, prospective cohort study designed to tackle these issues. © ERS 2013. Source

Maher T.M.,Interstitial Lung Disease Unit | Maher T.M.,Imperial College London | Maher T.M.,University College London
Clinics in Chest Medicine | Year: 2012

Idiopathic pulmonary fibrosis (IPF) is a progressive disease of unknown cause that conveys a dismal prognosis. In the United States there are currently no licensed therapies for treatment of IPF. The development of effective IPF clinical trials networks across the United States and Europe, however, has led to key developments in the treatment of IPF. Advances in understanding of the pathogenetic processes involved in the development of pulmonary fibrosis have led to novel therapeutic targets. These developments offer hope that there may, in the near future, be therapeutic options available for treatment of this devastating disease. © 2012 Elsevier Inc. Source

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