[HTML][HTML] Pulmonary fibrosis: patterns and perpetrators

PW Noble, CE Barkauskas… - The Journal of clinical …, 2012 - Am Soc Clin Investig
PW Noble, CE Barkauskas, D Jiang
The Journal of clinical investigation, 2012Am Soc Clin Investig
Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of
morbidity and mortality, and represents an enormous unmet medical need. However, the
disease is heterogeneous, and the failure to accurately discern between forms of fibrosing
lung diseases leads to inaccurate treatments. Pulmonary fibrosis occurring in the context of
connective tissue diseases is often characterized by a distinct pattern of tissue pathology
and may be amenable to immunosuppressive therapies. In contrast, idiopathic pulmonary …
Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need. However, the disease is heterogeneous, and the failure to accurately discern between forms of fibrosing lung diseases leads to inaccurate treatments. Pulmonary fibrosis occurring in the context of connective tissue diseases is often characterized by a distinct pattern of tissue pathology and may be amenable to immunosuppressive therapies. In contrast, idiopathic pulmonary fibrosis (IPF) is a progressive and lethal form of fibrosing lung disease that is recalcitrant to therapies that target the immune system. Although animal models of fibrosis imperfectly recapitulate IPF, they have yielded numerous targets for therapeutic intervention. Understanding the heterogeneity of these diseases and elucidating the final common pathways of fibrogenesis are critical for the development of efficacious therapies for severe fibrosing lung diseases.
The Journal of Clinical Investigation