Collard Harold R., Richeldi Luca. Interstitial Lung Disease

Elsevier, 2018. — 187 p. — ISBN: 978-0-323-48024-6.The interstitial lung diseases (ILDs) are a large and heterogeneous group of disease entities that differ significantly with respect to presentation, cause, prevention, therapy, and prognosis. Many of them have no clear etiology. However, in recent years considerable progress has been made in our understanding of these entities. In Interstitial Lung Disease, we discuss the most important of the ILDs and update the reader about their management.
Arriving at the correct diagnosis often requires correlation with clinical, radiologic, and pathologic findings.
The diagnostic strategy that should be employed when faced with a patient with ILD is described. Given the important role of high-resolution computed tomography (HRCT) in the diagnosis of ILD, we introduce pulmonologists and clinicians to the imaging appearances of ILDs by providing a pattern approach to the evaluation of HRCT. In addition, a focused discussion of the HRCT findings in the common ILDs is presented.
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Over the past decade, our thinking about IPF has been reshaped and guidelines have been revised using an evidence-based approach. A number of epidemiologic studies have identified potential risk factors for IPF. We summarize the approach to diagnosing IPF and review its epidemiology. With improved understanding of IPF, we now recognize that there may also be distinct phenotypes among this group of patients. Furthermore, we describe how the identification and management of comorbidities may improve the overall quality of life and well-being of these patients. It has become clear that the clinical course of individual patients with IPF is variable and that the sudden deterioration of a patient’s respiratory condition during a relatively stable course is possible. This deterioration can result from wellknown causes such as infection, pulmonary embolism, congestive heart failure, pneumothorax, or drugs. However, in many cases the etiology is not certain despite rigorous searches. The current state of our understanding of this important manifestation of IPF is discussed.
Also, the mechanisms of action of current and potential IPF therapies are reviewed, with particular reference to current disease understanding and to highlight areas of IPF disease biology that afford attractive targets for the development of the new treatments. The general management of a patient with IPF is described, especially the management of important comorbidities (e.g., pulmonary hypertension and gastroesophageal reflux) and symptoms (e.g., dyspnea, exercise limitation, fatigue, anxiety, mood disturbance, sleep disorders) that dramatically affect IPF patients’ lives. Furthermore, the increasingly important role of lung transplantation in the treatment of patients with ILD is discussed, especially the evidence suggesting that patients with pulmonary fibrosis undergoing lung transplantation have a favorable long-term survival compared with other disease indications.
Finally, several chapters review important individual ILD entities, including inherited fibrotic lung diseases, nonspecific interstitial pneumonia, ILD associated with connective tissue diseases, chronic hypersensitivity pneumonitis, and smoking-related ILDs.