Idiopathic Pulmonary Fibrosis (IPF): History
Subjects: Pathology
Contributors:

In idiopathic pulmonary fibrosis (IPF), several factors may have a negative impact on the nutritional status, including an increased respiratory muscles load, release of inflammation mediators, the coexistence of hypoxemia, and physical inactivity. Nutritional abnormalities also have an impact on IPF clinical outcomes. Given the relevance of nutritional status in IPF patients, we sought to focus on some critical issues, highlighting what is known and what should be further learned about these issues. We revised scientific literature published between 1995 and August 2019 by searching on Medline/PubMed and EMBASE databases including observational and interventional studies. We conducted a narrative review on nutritional assessment in IPF, underlining the importance of nutritional evaluation not only in the diagnostic process, but also during follow-up. We also highlighted the need to keep a high level of attention on cardiovascular comorbidities. We also focused on current clinical treatment in IPF with Nintedanib and Pirfenidone and management of gastrointestinal adverse events, such as diarrhea, induced by these antifibrotic drugs. Finally, we concentrated on the importance of pulmonary rehabilitation program, including nutritional assessment, education and behavioral change, and psychological support among its essential components. More attention should be devoted to the assessment of the undernutrition and overnutrition, as well as of muscle strength and physical performance in IPF patients, taking also into account that an adequate clinical management of gastrointestinal complications makes IPF drug treatments more feasible. 

  • idiopathic pulmonary fibrosis
  • nutrition abnormalities
  • nutritional assessment
  • rehabilitation

In recent years, nutritional status has increasingly gained attention in the evaluation of patients with chronic respiratory diseases since their clinical course is often characterized by progressive weight loss and reduction of muscle mass [1–3].

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease of unknown etiology characterized by rapid progression and poor prognosis [4,5].

As shown in Figure 1, in IPF, several factors may have a negative impact on the nutritional status of IPF patients, including an increased respiratory muscles load, release of inflammation mediators, hypoxemia, and physical inactivity [6–9].

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Figure 1. Nutritional disorders in IPF: pathogenesis.

Furthermore, given the rapidly progressive nature of the disease, with a median survival time of only two to five years, all these factors often develop and worsen in a short period of time.

It is worth noting that not only may the “downward spiral” of the underlying pulmonary disease affect the nutritional status, but also nutritional abnormalities may have an impact on clinical outcomes. For instance, lower body mass index (BMI) and body weight loss have been associated with increased mortality [10–12]. Conversely, the effects of obesity and metabolic syndrome are less known.

Given the relevance of evaluating nutritional status in IPF patients, a topic which remains still largely understudied, we sought to focus on some critical issues, highlighting what is known and what should be further learned about these issues.

This entry is adapted from the peer-reviewed paper 10.3390/nu12041131