This volume describes the pathogenesis and pathophysiology of several pulmonary diseases as
well as their treatment. It also discusses the underlying genetic and molecular biological
basis which opens the way for new treatments for these conditions. It focuses on the treatment
of cystic fibrosis including CFTR (cystic fibrosis transmembrane-conductance regulator)
modulator therapies drug therapies that augment airway surface liquid as well as
anti-inflammatory and anti-infective therapies. Further topics include long-term low-dose
macrolide therapy for diffuse panbronchiolitis novel agents for previously untreatable
idiopathic pulmonary fibrosis possible new treatments for pulmonary alveolar proteinosis (PAP)
and multiple novel therapeutic targets for treating lymphangiomyomatosis. Research into these
conditions has led to major advances in our understanding of the underlying genetic and
molecular basis of this disease and to dramatic improvements in survival and quality of life
for affected individuals.
