Transforming growth factor-beta1 (TGF-beta1) is a cytokine that plays a key role in the development of idiopathic pulmonary fibrosis. There have been reports on the presence of two genetic polymorphisms in the DNA sequence encoding the leader sequence of the TGF-beta1 protein, located in codons 10 and 25. The objective of this study was to investigate the association between TGF-beta1 gene polymorphisms in codons 10 and 25 and the susceptibility to idiopathic pulmonary fibrosis and the progression of the disease. Compared with healthy control subjects (n = 140), patients with idiopathic pulmonary fibrosis (n = 128) showed no significant deviations in genotype or allele frequencies. One hundred and ten patients with idiopathic pulmonary fibrosis were followed up for 30.3 +/- 25 months. The presence of a proline allele at codon 10 was independently associated with a significant increase in alveolar arterial oxygen tension difference during follow-up, after controlling for the effect of treatment (coefficient = 0.59; 95% confidence intervals, 0.23 to 0.96; p = 0.002). These findings suggest that (1) TGF-beta1 gene polymorphisms in codons 10 and 25 do not predispose to the development of idiopathic pulmonary fibrosis; and (2) TGF-beta1 gene polymorphisms may affect disease progression in patients with idiopathic pulmonary fibrosis.

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Patients with idiopathic pulmonary fibrosis (IPF) are at risk for a variety of acute pulmonary complications, including pneumothorax and pneumomediastinum. Our aim was to describe the radiographic and CT findings and to determine the frequency of complicating spontaneous pneumothorax and pneumomediastinum in patients with IPF. A retrospective study was performed including 78 consecutive patients who underwent CT scanning of the chest and who had confirmed IPF. The chest radiographs and CT scans were reviewed by two chest radiologists and classified as showing features of extra-alveolar air collections. The CT scans showed extra-alveolar air in 9 (11.2 %) of 78 patients (six females and three males; age range 26-90 years, mean age 65 years). Pneumothorax was demonstrated in 5 patients and mediastinal air collections in 4 patients. All patients had dyspnea for 1-48 months (mean 14 months). Of the five cases with pneumothorax, four developed acute onset of dyspnea and pleuritic chest pain, whereas 1 patient had a relatively stable functional status. Of the 4 patients with pneumomediastinum, three presented with nonpleuritic chest pain and acute dyspnea. Chest radiographs showed extra-alveolar air in 6 patients. Three cases were predicted to be negative by chest radiographs. Follow-up CT showed that air collections had resolved completely in 5 patients. Two patients died of respiratory failure within 4 months after CT. Extra-alveolar air should be recognized as a relatively common IPF-related complication. Chest CT is a useful imaging method in determining air collections in patients with IPF that become acutely breathless and their chest radiograph fails to reveal the presence of extra-alveolar air.