ROMA (ITALPRESS) – Lack of breath, initially during physical effort, which with the progression of the disease can also appear in the minimum movements, dry cough and a general feeling of fatigue. They are some of the main alarm bells of idiopathic pulmonary fibrosis (Ipf), a rare, chronic and progressive disease. Diagnosis, taken into account and perspectives of care were at the center of the fourth national congress Fimarp, Italian Federation Ipf and rare lung diseases, which was held at the Higher Institute of Health. “Rare diseases – says Iss President, Rocco Bellantone – cannot be dealt with from one point of view. They require research, clinical skills, data, health planning and, above all, listening to people directly involved.”.
In Italy, the data of the National Register of Rare Diseases report 3.517 people diagnosed with idiopathic pulmonary fibrosis in the period 2017-2023. About 60% of patients are male and with an average age diagnosed about 70 years. However, it is a partial photograph, because the Ipf is recomposed in the register within the larger group of lung interstitiopathies.
The Italian Register for Idiopathic Pulmonary Fibrosis (Registro Ipf), active at the Higher Institute of Health and coordinated by the National Centre for Rare Diseases, was founded by the need to improve diagnosis, therapy and patient care. “The Registry – says Maria Luisa Scattoni, director of the National Centre for Rare Diseases of the Iss – currently in the enlistment phase, includes to date information regarding 425 patients taken into charge by 17 Centres who joined the project.
Through a structured collection of clinical data, the goal is to build a deeper knowledge of Ipf and support research: from the definition of the natural history of the disease to the integration of clinical data with radiographic images, to the evaluation of the effectiveness of the available therapies and those in the experimental phase”. In addition to the need to know the disease better, the research on idiopathic pulmonary fibrosis is going through a phase of particular interest on the therapeutic front. Among the most important novelties figure nerondomilast, protagonist of two important studies published in the New England Journal of Medicine. The studies were led by the Catholic University of the Sacred Heart and by the Uoc Pneumology of the Political Foundation Gemelli, directed by Luca Richeldi.
The results of the research on nerondomilast are an element of particular interest in the development of new therapeutic strategies for pulmonary fibrosis and could help to broaden the panorama of the available options, at a distance of years from the introduction of the main anti-fibrotic therapies today used. “Research – says Marco Salvatore, of the National Centre for Rare Diseases of Iss – does not only look to therapies. Another priority objective is to anticipate the diagnosis, identifying early signs of the disease and people more at risk.”.
In this direction, the Genesis study, conducted by the Fondazione Policlinico Gemelli together with the University of Catania, which examines the genetic mutations of patients with Ipf and previews the screening of family members through tools such as chest Tac and electronic stethoscopes. The objective is to identify characteristics and signals that can indicate a greater predisposition to the disease, progressively moving the diagnosis to a more early stage of the pathological path. The Congress at Iss was the occasion to celebrate Fimarp’s ten years. “The ten years of Fimarp – says President Matteo Buccioli – do not represent a point of arrival for us, but the beginning of a new phase. The Federation was born from the vision of Alessandro Giordani, who understood the importance of joining associations and giving patients a national voice. Today we want to collect that legacy and be an active part of the confrontation with scientific communities and institutions, bringing people’s needs to the places where research, assistance and health policies are built. The Registry and our projects are born from this vision: transforming patient listening into concrete actions that improve their lives.”.
– Photo Pexels.com –
(ITALPRESS).
