By Joseph P. Lynch III
A dialogue of the epidemiology, scientific beneficial properties, and differential diagnoses of idiopathic pulmonary fibrosis (IPF). Key issues contain the position of polymorphonuclear leukocytes within the pathogenesis of pulmonary fibrosis, and present healing procedures, together with clinical treatment and lung transplantation.
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Additional resources for Lung Biology in Health and Disease Volume 185 Idiopathic Pulmonary Fibrosis
D. Histological Diagnosis Only a minority of patients with IPF undergo surgical lung biopsy (4,86). Often these patients are younger (11), and the clinical diagnosis is not certain. Therefore, prognostic results based on histological ﬁndings must be interpreted cautiously, since the process of patient selection for surgical lung biopsy may bias results on the relationship between histological ﬁndings and prognosis in patients with IPF. In early case series, a consistent ﬁnding of these studies was that patients with a histological diagnosis of desquamative interstitial pneumonitis (DIP) had a much better prognosis than those with a diagnosis of UIP (37,38).
1). Third, the occurrence of IPF varies widely both within and between countries. These observations provide a basis for generating hypotheses on potential etiological factors in IPF and are discussed in the next section. IV. Etiological Studies Although the diagnosis of IPF requires ‘‘exclusion of other known causes of ILD such as certain drug toxicities, environmental exposures, and connective tissue diseases’’ (see Table 1), experimental evidence, clinical observations, and epidemiological investigations suggest that a number of factors may have an etiological role in IPF.
26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 25 Uydebrouck M and the working group on ILD, VRGT, Brussel. Epidemiology of interstitial lung disease (ILD) in Flanders: registration by pneumologists in 1992–1994. Acta Clin. Belg. 1995; 50–5:260–268. Hubbard R, Johnston I, Coultas DB, Britton J. Mortality rates from cryptogenic fibrosing alveolitis in seven countries. Thorax 1996; 51:711–716. Mannino DM, Etzel RA, Parrish RG. Pulmonary fibrosis deaths in the United States, 1979–1991. Am J Respir Crit Care Med 1996; 153:1548–1552.