Interstitial Lung Disease in 2020: A History of Progress.

Interstitial lung diseases (ILDs) are heterogenous and complex chronic lung diseases that even today are challenging to diagnose and classify. The terminology and mechanistic understanding of specific ILDs have evolved substantially over the last centuries and decades, and clinicians, pathologists, radiologists, and researchers are continuously working to untangle the various ILDs of differing causes. Despite many drawbacks and negative clinical trials, the unremitting work of ILD researchers have resulted in great therapeutic successes over the last decade. In this chapter, the authors present historical aspects of ILD and build a foundation to understand current and emerging concepts in ILD.

20 años en enfermedad intersticial pulmonar / 20 years in pulmonary interstitial disease

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[Interstitial lung diseases - historical development, current status, future prospects]. / Interstitielle Lungenerkrankungen - historische Entwicklung, Status quo und Ausblick.

Sarcoidosis and lung fibrosis were first described by histopathology in the 19th century. Since then a large number of different forms of diffuse parenchymal lung diseases has been identified. Although all these diseases manifest in the lung interstitium, there is a wide range of predominanthy inflammatory to purely fibrotic disease processes. Accordingly, anti-inflammatory treatment is successful in the former, whereas for the latter an effective medical therapy is lacking. Gene technology has recently led to results which have fundamentally changed our understanding of the pathophysiology of fibrosing lung disease. Early and differential diagnosis has much improved with the help of high-resolution computed tomography. Recent clinical trials in patients with idiopathic pulmonary fibrosis have shown at least some effectiveness for the antioxidant treatment approach using high-dosed N-acetylcysteine as well as for the use of pirfenidone. Importantly, these studies have proven that well-designed treatment trials are feasible in this patient population. This has kindled the hope that, based on a better understanding of the pathophysiology, new targeted therapies will prove to be successful in the future.

What caused the epidemic of Pneumocystis pneumonia in European premature infants in the mid-20th century?

An epidemic of interstitial pneumonia principally involving premature infants occurred in Germany and nearby European countries between the 1920s and 1960s. Fatalities were due to Pneumocystis. Because the principal defenses against Pneumocystis are T cells, an acquired T-cell deficiency was postulated. A number of potential causes including malnutrition were considered. All were implausible except for a retrovirus that was benign in adults but virulent in premature infants. Furthermore, we suspect that the virus was imported into Germany from former German African colonies. Premature infants were vulnerable because of the developmental status of their T cells. Given the practices in that part of Europe at that time, the virus was most likely transmitted by contaminated blood transfusions and subsequent contamination of reusable needles and syringes used in injections. Although the epidemic ended 4 decades ago, a search for the postulated retrovirus can be conducted if tissues from affected infants are available.