No abstract

MDC care model for IPF was associated with reduced health care use in the last year of life and more home deaths.

Idiopathic pulmonary fibrosis is a progressive disease, with a high mortality within the first 3-5 years from diagnosis and a poor quality of life mainly because of the burden of symptoms, such as dyspnea and cough, occurring usually many months before the diagnosis. Although available antifibrotic therapies slow down disease progression, they have no impact on quality of life. Moreover, health care around idiopathic pulmonary fibrosis patients is often "disease-centered" and relies on clinical surrogate outcomes that are poorly related to patients' quality of life and disease experience. Therefore, patients with idiopathic pulmonary fibrosis have several unmet needs in all domains of health that they wish to see recognized and addressed in the context of the treatment of their disease and its complications. In this review, we summarize the care pathway from the patients' perspective, identifying current gaps in care, education, support, and communication among patients with IPF, their caregivers, and care teams during the patient journey. The role of patient-reported outcomes (PROs), PRO measures (PROMs), and patient-reported experience measures (PREMs) in their care is discussed, as well as the need of diseasespecific PROs, PROMs, and PREMs.

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disorder with an estimated median survival time of 3–5 years after diagnosis. This condition occurs primarily in elderly subjects, and epidemiological studies suggest that the main risk factors, ageing and exposure to cigarette smoke, are associated with both pulmonary and extrapulmonary comorbidities (defined as the occurrence of two or more disorders in a single individual). Ageing and senescence, through interactions with environmental factors, may contribute to the pathogenesis of IPF by various mechanisms, causing lung epithelium damage and increasing the resistance of myofibroblasts to apoptosis, eventually resulting in extracellular matrix accumulation and pulmonary fibrosis. As a paradigm, syndromes featuring short telomeres represent archetypal premature ageing syndromes and are often associated with pulmonary fibrosis. The pathophysiological features induced by ageing and senescence in patients with IPF may translate to pulmonary and extrapulmonary features, including emphysema, pulmonary hypertension, lung cancer, coronary artery disease, gastro-oesophageal reflux, diabetes mellitus and many other chronic diseases, which may lead to substantial negative consequences in terms of various outcome parameters in IPF. Therefore, the careful diagnosis and treatment of comorbidities may represent an outstanding chance to improve quality of life and survival, and it is necessary to contemplate all possible management options for IPF, including early identification and treatment of comorbidities.