Adult bronchiectasis, asthma therapy, frailty in ILD

Interstitial and Diffuse Lung Disease

Frailty as a measure of disease activity in ILD

Frailty is a systemic geriatric syndrome characterized by age-related accumulation of physiologic deficits across several systems with an attenuated response to biological stress. Considering that interstitial lung disease (ILD), particularly, idiopathic pulmonary fibrosis (IPF), is a disease of the aging population, frailty is an emerging area of clinical interest. The biological pathways driving the association of frailty with worse prognosis are complex but hinge on cellular senescence, systemic inflammation, and sarcopenia.

There is a high prevalence of frailty in adults with chronic lung diseases and is associated with worse prognosis. The current literature, though, is mostly derived from patients with COPD. Frailty measured using the 42-item patient-reported frailty index is associated with dyspnea severity in patients with fibrotic ILD (Milne et al. Respirology. 2017;22[4]:728) and systemic sclerosis-associated ILD (Guler et al. Respir Med. 2017 Aug;129:1-7. doi: 10.1016/j.rmed.2017.05.012. Epub 2017 May 25.). The SHARE-Frailty and the Edmonton Frail Scale instruments utilized to measure frailty in the University of Alabama at Birmingham IPF cohort detected a high-percentage of frail and pre-frail patients (Luckhardt et al. Am J Respir Crit Care Med. 2017;195:A7012). However, there are differences in targeted domains between the various frailty instruments, and this could affect the identification of the frailty syndrome in patients.

Frailty as a measure of disease activity and progression is not currently employed in clinical trials for ILD, primarily due to lack of standardized tools for this patient population. Future studies designed to utilize the frailty syndrome as outcome measures may further our understanding of the clinical manifestations and underlying mechanisms, as well as identify potential therapeutic interventions for patients with ILD.

Tejaswini Kulkarni MD, MPH
Fellow-in-Training Member