| Type I and III procollagen propeptides in sarcoidosis, fibrosing alveolitis and asbestos-related lung diseases | ||
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The activity of interstitial lung diseases and the prediction of disease progression is difficult to assess and poorly understood. The prognosis of sarcoidosis is good in most cases, and only a small number of sarcoidosis patients develop progressive pulmonary fibrosis. However, no accurate markers for the assessment of the activity of sarcoidosis are available. In contrast, the prognosis of fibrosing alveolitis is poor in most cases, leading to severe impairment of respiratory function and finally to death. Apart from open or thoracoscopic lung biopsies with typical histopathology, assessment of the disease activity and prognosis is difficult. Exposure to asbestos may lead to progressive fibrosis of the lungs, asbestosis, after a latency of 15-20 years with a poor prognosis and no effective cure.
The present study was undertaken to characterise the role of procollagen I and III propeptides in various interstitial lung diseases to examine their role as diagnostic and prognostic tools in these diseases and as markers of fibrogenesis.
The specific aims of the study were as follows:
to apply new tools to investigating collagen synthesis in interstitial lung diseases for purposes of clinical practice.
to evaluate the role of type I and III procollagen markers in serum and BALF in disease activity and the prognosis of sarcoidosis, fibrosing alveolitis and asbestos-related diseases.
to compare procollagen propeptide levels in BALF, serum and in epithelial lining fluid (ELF) to estimate the level of active inflammation.
to assess the accumulation and distribution of type I and III procollagens and the corresponding pN-collagens in histological specimens in fibrosing alveolitis and sarcoidosis.