We still have significant unmet needs in the pharmacological personalized treatment of difficult-to-control asthma, chronic obstructive pulmonary disease (COPD), lung cancer, idiopathic pulmonary fibrosis (IPF) and the respiratory tract involvement in neuromuscular diseases. Collectively these lung diseases represent a major cause of morbidity and/or mortality worldwide.
Lung cancer is the most frequent cause of cancer-related deaths worldwide. Every year, 1.8 million people are diagnosed with lung cancer, and 1.6 million people die as a result of the disease. 5-year survival rates vary from 4-17% depending on stage and regional differences. In the last years, the introduction of immune checkpoint inhibitors is quickly changing lung cancer management and this is a rapidly evolving field.
In addition there are molecular links, still largely under investigated, between COPD, lung, cancer and IPF, all tobacco smoking-related diseases but with different clinical-pathological expressions. It is particularly relevant that in a large longitudinal study of asymptomatic patients with mild-to-moderate COPD, 33% of subjects died of lung cancer over 15 years suggesting that lung cancer is a leading cause of morbidity and mortality in COPD patients. These patients share a common environmental risk factor (tobacco smoking) and a genetic predisposition represented by the incidence of these diseases in only a fraction of smokers. COPD is also a major independent risk factor for lung carcinoma, among smokers.
IPF occurs worldwide. The prevalence of the disease appears to be increasing, although it is unclear whether this reflects increased recognition or a true increase in incidence. IPF is of particular clinical interest because it is often misdiagnosed and managed inappropriately with immunosuppressive therapy, it is associated with a high mortality rate, and therapies that slow disease progression are now available.
Severe asthma accounts for only a small proportion of the patients with difficult-to-control asthma, but use a disproportionately high amount of health resources and has an high morbidity. It is an heterogeneous entity and requires a step-wise, evidence-based approach for its evaluation and management in specialised Centres.
Management of severe asthma may include many biologic drugs as well as new emerging therapies based on the individual patient characteristics.
Neuromuscular diseases represent an heterogeneous group of disorders of the muscle, nerve or neuromuscular junction. Involvement of respiratory muscles is a nearly constant feature of neuromuscular disorders, leading to severe respiratory failure and increasing the risk of pneumonias. Diagnosis and treatment of their respiratory tract involvement is crucial because of the importance of respiratory morbidity and mortality.
We also would like to provide an overview of emerging and/or changing concepts in several areas of Paediatric Pulmonology rather than a comprehensive review of the field.
For all these reasons the aim of this Meeting is to gather together Italian and International experts in the field of these lung diseases to discuss in depth the cutting edge clinical and translational research performed in these different diseases and to coordinate global research network efforts to promote a better knowledge in their pathogenesis and to obtain new pharmacological targets for rationale drug development.
Prof. Giuseppe Girbino
President of the Meeting