Sophisticated people can hardly understand how vague experience is at bottom, and how truly that vagueness supports whatever clearness is afterwards attained.
George Santayana The Life of Reason
De morbis artificium diatriba, one of the most influential documents on occupational medicine, written by Bernardino Ramazzini da Capri (1633–1714), (Figure 1.1), surfaced in Padua in 1700. In the chapter “Diseases of Sifters, Measurers, and Handlers of Grain,”
the author described the occurrence of dry cough, cachexia, asthma, and dropsy in these workers. In addition, he commented on the role of humidity, and suggested that “small worms” not visible to the eye present in the wheat dust might be responsible for the illness. This was the first clinical description of an interstitial lung disease caused by occupational exposure.
In 1868, Austin Flint described a vague, nondescript lung disease that he called chronic pneumonitis. It was characterized by florid inflammatory exudation without pus, and it caused solidification and fibrosis of the lungs. Flint alluded that Carl Freiherr von Rokitansky had described a similar condition in which exudation had occurred into the interlobular and intervesicular areolar tissue. Furthermore, he asserted that the illness was different from florid tuberculosis. A few years earlier, Dominic Corrigan, an Irish cardiologist, called the similar entity cirrhosis of the lung because it was analogous, not identical, to cirrhosis of the liver. Although Flint had thought that the condition was rare;
two significant observations were made during the period. First, Corrigan described the occurrence of traction bronchiectasis in interstitial pneumonitis. Second, Flint observed that the fingertips of one patient with interstitial pneumonitis had assumed a bulbous appearance, and he therefore became the first to notice the association of clubbing and interstitial pneumonitis/fibrosis.
Two decades later, Wilson Fox, professor of pathological anatomy at the University College, London, recorded the microscopic changes of capillary edema; accumulation of pigmented epithelium in the alveoli; and thickening of the walls of the alveoli and veins in lungs with interstitial pneumonitis. Fox also credited Rokitansky for recognizing the fibrous thickening of the alveoli in this condition (Figure 1.2a, b, c).
In 1892, William Osler, while at Johns Hopkins Hospital, Baltimore, observed, “In one of Charcot’s cases . . . death occurred about three months after the onset of the acute disease and the lung was two thirds of the normal size, grayish in color, hard as carti- lage. In only case of the kind that has come under my observation, the patient died about a month from the onset of the chill. The lung was uniformly solid and grayish in color.
Microscopically these areas showed advanced fibrotic changes and great thickening of the alveolar walls.” Osler recommended that the term cirrhosis should be applied only to the cases in which a lung was densely fibrosed, whether fibrosis had originated in the parenchyma or the pleura. Additionally, like Flint, Osler advised that this new entity be distinguished from the fibrosis caused by tuberculosis (Figure 1.3).
In 1944, Louis Hamman and Arnold Rich (Figure 1.4), both at the Johns Hopkins Uni- versity School of Medicine, described four young patients who died of progressive
1
Interstitial Lung Diseases:
A Historical Note
1
Figure 1.1. Bernardino Ramazzini (1633–1714).
Figure 1.2. (continued)
Figure 1.2. Title page: An Atlas of the Pathological Anatomy (a). Chronic pneumonitis caused by syphilis (b). Various stages in the development of fibrosis in chronic phthisis (c).
(continued below)
Interstitial Lung Diseases: A Historical Note 3
Figure 1.3. Sir William Osler (1849–1919).
Figure 1.4. Louis Hamman and Arnold Rich at Johns Hopkins Medical Center, Baltimore, Maryland.
dyspnea within 6 months of onset. The clinical profile of the illness was similar to that described earlier by Flint, Corrigan, Fox, Charcot, and Osler. The term Hamman-Rich syndrome became a synonym for an interstitial pneumonia of unknown cause followed by fulminating pulmonary fibrosis. It was soon apparent that the course of this new disease was not always acute, progressive, or fatal. In 1957, Rubin and Lubliner reviewed 48 cases of the Hamman-Rich syndrome and added 15 cases of their own. From that point on, diffuse interstitial pneumonitis/fibrosis could no longer be considered a rare disease.
In 1960, interstitial pneumonias acquired two new terms: idiopathic pulmonary fibrosis and cryptogenic fibrosing alveolitis. Scadding and Hinson, working at Brompton Chest Hospital, London, preferred the latter term to define the inflammatory and fibrotic changes in the lung parenchyma. They advised that the word idiopathic be dropped in favor of the term cryptogenic in describing the illness of unknown cause. Cryptogenic fibrosing alveolitis is an accepted term in Europe; whereas, in North America, the usual interstitial pneumonia remains a popular description.
Averill Liebow (Figure 1.5), on the basis of his extensive clinical and pathologic sci- ences, classified interstitial pneumonitis into five different histologic types: usual inter- stitial pneumonitis (UIP), desquamative interstitial pneumonia (DIP), bronchiolitis obliterans interstitial pneumonia (BIP), lymphoid interstitial pneumonia (LIP), and giant cell interstitial pneumonia (GIP).
The classification of idiopathic or primary interstitial lung disease was further
simplified by removing BIP, LIP, and GIP, and by adding nonspecific interstitial
pneumonia (NSIP). The new classification of idiopathic interstitial pneumonia includes UIP and DIP from Liebow’s original classification and two new entities, acute intersti- tial pneumonia (AIP; or Hamman-Rich syndrome) and NSIP. Bronchiolitis obliterans organizing pneumonia (BOOP) was not initially included because it is primarily an intraluminal disease, but later the American Thoracic Society/European Respiratory Society (ATS/ERS) consensus included it again for its similar diagnostic algorithm and clinical behavior as other IIPs. Lymphoid interstitial pneumonias (LIP) were also added to the list. Clinical recognition of these disorders is easier today than it used to be in the early days.
Bibliography
1. Ramazzini B. De morbis artificium diatriba. Modena, Italy 1700.
2. Flint A. A Treatise on the Principles and Practice of Medicine. Philadelphia: Henry C. Lea; 1868:193.
3. Fox W. An Atlas of the Pathological Anatomy of the Lungs. London: J & A; 1888:52.
4. Osler W. Practice of Medicine. New York: Appleton & Co.; 1892:533.
5. Hamman L, Rich A. Acute diffuse interstitial fibrosis of the lungs. Bull Johns Hopkins Hosp 1944;74:177–212.
6. Rubin E, Lubliner H. The Hamman-Rich syndrome: Review of the literature and analysis of 15 cases. Med- icine (Baltimore) 1957;36:397–405.
7. Scadding G, Hinson K. Diffuse fibrosing alveolitis (diffuse interstitial fibrosis of the lungs): Correlation of histology at biopsy and prognosis. Thorax 1967;22:291–303.
8. Liebow A. Definition and classification of interstitial pneumonias in human pathology. Prog Respir Dis 1975;8:1–39.
9. Katzenstein A-L, Myers J. Idiopahtic pulmonary fibrosis: A clinical relevance of pathologic classification.
Am J Respir Crit Care Med 1998;157:1301–1318.
Figure 1.5. Averill Liebow in San Diego, California.
Interstitial Lung Diseases: A Historical Note 5 10. Nagai S, Kitaichi M, Izumi T. Classification and recent advances in idiopathic interstitial pneumonia. Curr
Opin Pulm Med 1998;4:256–260.
11. Myers J. NSIP, UIP and the ABC of idiopathic interstitial pneumonias. Eur Respir J 1998;12:1003–
1014.
12. Ryu J, Colby T, Hartman T. Idiopathic pulmonary fibrosis: Current concepts. Mayo Clin Proc 1998;73:1085–1101.
13. ATS/ERS. International multidisciplinary consensus classification of the idiopathic interstitial pneumo- nias. Am J Respir Crit Care Med 2002;165:277–304.
