ABSTRACT
Fibrosis of the pulmonary interstitium is observed in a wide variety of disorders, including infectious diseases, autoimmune disorders of connective tissue, and disorders where the etiology is unknown, such as idiopathic pulmonary fibrosis (1,2). The clinical manifestations of interstitial pulmonary fibrosis are likely the consequences of an initial immune/inflammatory response to a persistent antigen, leading to continued tissue injury and progressive fibrosis. The underlying mechanisms which promote the development of a fibrotic pathological response, as compared to a normal reparative response, remains one of the many different enigmas of pulmonary fibrosis. The clinical management of these disorders is frequently difficult, requiring the use of potent and often cytotoxic and immunosuppressive therapies (3,4); however, these therapeutic approaches often fail and organ transplant is the last resort.
