Idiopathic Pulmonary Fibrosis and the Elderly: Diagnosis and Management Considerations

Abstract

Idiopathic pulmonary fibrosis (IPF) is a severe and progressive fibrosing interstitial lung disease, which ultimately results in respiratory failure and death. The median age at diagnosis is 66 years, and the incidence increases with age, making this a disease that predominantly affects the elderly population. IPF can often be difficult to diagnose, as its symptoms--cough, dyspnoea and fatigue--are non-specific and can often be attributed to co-morbidities such as heart failure and chronic obstructive pulmonary disease. Making an accurate diagnosis of IPF is imperative, as new treatments that appear to slow the progression of IPF have recently become available. Pirfenidone and nintedanib are two such treatments, which have shown efficacy in randomised controlled trials. As with all new treatments, caution must be advocated in the elderly, as these patients often lie outside the narrow clinical trial cohorts that are studied, and the benefits of therapy must be weighed against potential toxicities. Both medications, while relatively safe, have been associated with adverse effects, particularly gastrointestinal symptoms such as nausea, diarrhoea and anorexia. In this review, we highlight measures to improve recognition and accurate diagnosis of IPF, as well as co-morbidities that often affect the diagnosis and disease course. The gold standard for IPF diagnosis is a multidisciplinary meeting whereby clinicians, radiologists and histopathologists reach a consensus after interactive discussion. In many cases, a lung biopsy may not be available because of high risk or patient choice, particularly in the elderly. In these cases, there is debate as to whether a biopsy is required, given the high rates of IPF in patients over the age of 70 years with interstitial changes on computed tomography. We also discuss the management of IPF, drawing particular attention to specific issues affecting the elderly population, especially with regard to polypharmacy and end-of-life care. Through this article, we endeavour to improve awareness of this devastating disease and thus improve recognition of the disease and its outcomes in elderly patients.

Fatty acid synthase is required for profibrotic TGF‐β signaling

“…Our identification of FASN as a TGF-b-regulated profibrotic target has the potential to redefine the paradigm by which fibroproliferative diseases are investigated and subsequently treated. This is of particular importance for diseases such as IPF, as whereas Pirfenidone and Nintedanib are 2 recently approved therapies, both have been associated with adverse effects, two-thirds of individuals with IPF still die within 5 yr of diagnosis, and it has recently been reported that "neither Pirfenidone nor Nintedanib has a clear advantage on mortality outcomes" (65)(66)(67).…”

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

“…Smoking cessation is also important in that regard and also has implications regarding the use of long‐term oxygen therapy (LTOT). Smoking has also been shown to decrease systemic exposure to both nintedanib and pirfenidone and smoking cessation should be strongly advised when initiating either of these medications …”

“…Smoking has also been shown to decrease systemic exposure to both nintedanib and pirfenidone and smoking cessation should be strongly advised when initiating either of these medications. 5 Depression and anxiety are other common but often overlooked co-morbidities in IPF patients, associated with a poorer quality of life and poorer outcomes. 6 Underlying depression and anxiety are often correlated with the presence of debilitating symptoms such as dyspnoea, a symptom affecting up to 90% of patients at diagnosis.…”

Clinical characteristics and factors associated with mortality in idiopathic pulmonary fibrosis: An experience from a tertiary care center in Pakistan

“…18 Older age has been established in the literature as an IPF risk factor, but the mechanism behind aging and the development of IPF remains elusive. The median age of patients at diagnosis with IPF is 66 years.…”

“…The median age of patients at diagnosis with IPF is 66 years. 18 Older age has been established in the literature as an IPF risk factor, but the mechanism behind aging and the development of IPF remains elusive. The diagnosis and management of the disease is complicated in the elderly population where the non-specific symptoms of cough and dyspnea can be attributed to other comorbidities like heart failure and chronic obstructive pulmonary disease.…”