Idiopathic pulmonary fibrosis. Clinical, histologic, radiographic, physiologic, scintigraphic, cytologic, and biochemical aspects.

Abstract

Idiopathic pulmonary fibrosis is a fatal disorder that starts as an alveolitis and progresses to interstitial fibrosis. Correlative morphologic, physiologic, and biochemical studies in 29 patients have shown that the inflammatory process in best followed by serial bronchoalveolar lavage and 67 Ga citrate scanning, and the fibrotic process is best followed by quantitation of the exercise-induced drop in arterial oxygen tension per unit of oxygen consumed. Although biopsies in idiopathic pulmonary fibrosis seem to show increased amounts of fibrotic tissue, biochemical studies suggest that the disease is probably one of collagen rearrangement rather than collagen increase. Perhaps becasue of this, peripheral lymphocytes of these patients recognize collagen as "non-self" and, when exposed to it in vitro, produce lymphokines and cell lysis. The fibrotic process is probably irreversible, but the inflammatory and immune processes that cause it may be amenable to therapy if diagnosed early.

• Authors: R G Crystal, J D Fulmer, W C Roberts, M L Moss, B R Line, H Y Reynolds
• Journal: Annals of internal medicine (Ann Intern Med) Vol. 85 Issue 6 Pg. 769-88 (Dec 1976) ISSN: 0003-4819 [Print] United States
• PMID: 793466 (Publication Type: Journal Article, Review)

Chemical References

• Collagen
• Oxygen

Topics

• Adult
• Aged
• Child
• Collagen (biosynthesis, immunology)
• Female
• Humans
• Lung (cytology, pathology)
• Lung Volume Measurements
• Male
• Middle Aged
• Oxygen (blood)
• Oxygen Consumption
• Pulmonary Diffusing Capacity
• Pulmonary Fibrosis (diagnosis, pathology, physiopathology)
• Pulmonary Ventilation
• Radiography, Thoracic
• Radionuclide Imaging
• Ventilation-Perfusion Ratio