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A Journal on Diseases of the Respiratory System
Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Minerva Pneumologica 2008 March;47(1):31-7
Management of advanced interstitial lung disease: what are the options?
Chiesa S., Tzani P., Olivieri D., Chetta A.
Section of Respiratory Diseases Department of Clinical Sciences University of Parma, Parma, Italy
Interstitial lung diseases (ILDs) are a heterogeneous group of disorders with different clinical and histological features and different prognosis. They may be considered as inflammatory disorders of the lung interstitium without any extra pulmonary involvement. The cause and pathogenetic mechanisms responsible for ILDs have not been completely elucidated. Accordingly, there is not a therapeutic intervention able to affect the cellular and molecular target responsible for the disease and change its course. The initial pathogenetic theory considering ILDs as inflammatory processes makes reasonable the use of corticosteroids, considered as first-line drugs. Cytotoxic and immunosuppressive agents can be used in combination with corticosteroids. The most recent acquisitions in the pathogenetic field identify the initial phase of the disease in the alveolar epithelial cells injury and destruction of the sub-epithelial basement membrane, leading to abnormal wound healing with a vigorous fibroblastic response and excessive deposition of collagen and extracellular matrix. This new pathogenetic theory suggests a primary role for fibroblasts dysregulation. Thus, fibroproliferative process becomes the therapeutic target and new drugs which arrest the proliferation of fibroblasts and the deposition of extracellular matrix are being testing, such as immunomodulators, antifibrotic agents and antiangiogenic drugs. In ILD patients refractory to medical therapy lung transplantation is the only option which definitely improves survival.