Fibrosi polmonare idiopatica

The pulmonary interstitial disease denominated Idiopathic Pulmonary Fibrosis (or Cryptogenetic Fibrosing Alveolitis) is a specific form of chronic interstial pneumonitis, mostly associated with hystologic features defining the Usual Interstitial Pneumonitis. Between the other Idiopathic Interstitial Pneumonites this is the first by frequency and also the one characterised by the worst prognosis. A generally lethal disease (except in the unfrequent cases where a gastroesophageal reflux can be recognized as a pathogenetic determinant) which shows a fast, evident, unexplained increase worldwide, more frequent in males and in presenile age although diffuse enough in the feminine gender as well as in different ages. Potential risks and pathogenetic factors are described; the numerous acute and chronic conditions needing a differential diagnosis (in particular the other forms of idiopathic interstitial pneumonitis) are examined; the symptoms, both clinical (particularly the Velcro-like crepitations) and instrumental, such as cellular and biochemical information obtained by the bronchoalveolar lavage, hystopathology obtained by lung biopsy but specially imaging coming from high resolution computerized tomography are described. The last features, to be substantially distinguished in "ground glass" and "honeycombing", lead to the interesting observation of a better response to the specific medical treatment (essentially corticosteroids associated or not with azathioprine or cyclophosphamide) in those patient (often positively responding to the therapy, in some way) who are characterized by prevalence of "ground glass" lung images, compared with the patients characterized by "honeycombing" images, who undergo a more rapidly lethal, prognosis. Other pharmacologic agents have been submitted to trials, namely colchicine, pirphenidone, interferon, anti-TNFalpha, bosentan, but the results are temporary or ambiguous; although a very recent observation with an antioxidant (NAC in relatively high doses) leads to some interesting new interpretations. Good enough, anthough obviously "numerically" limited, are the results of the lung transplantation, with several kinds of overall advantages, at least in the experience of our Hospital, for the monolateral transplant.