This condition had appeared in Question 13 from the first paper of 2017, in the form of a 56 year old woman dying of hypoxic respiratory failure. Optimise her lung function, the college beckoned to the candidates. And discuss the barriers to weaning.
A brief introduction to this disease is offered by Collard et al (2007), as a review article on acute exacerbations. The authors described acute exacerbations as "clinically significant deteriorations of unidentifiable cause". Generally speaking, this disease is hideous. Median survival from the time of diagnosis is approximately 3 years. Earlier this century the actual ICU and hospital mortality rates for acute exacerbations were 45% and 61%, respectively (Saydain et al, 2002).
For answering management questions, there is probably nothing better than the American Thoracic Society Guidelines (Raghu et al, 2011). Together with the UpToDate page on this topic, these guidelines were summarised into the brief SAQ answer below.
There is not much out there on ICU-specific management of these patients. Juarez et al (2015) had a good article on pharmacotherapy, but it seems to be better suited to an audience who run respiratory outpatient clinics. Whenever one searches for an article to guide the approach to ventilation, one finds a hald dozen articles describing the futility of such ventilation. The only useful contribution was from Fernandez et al (2008), who retrospectively analysed a cohort of 94 mechanically ventilated IPF patients to see what worked and what didn't.
As far as "optimise lung function" goes, there are three main domains: try to control the disease process, get some gas exchange happening and control the pulmonary hypertension.
Specific management of the disease process
Manipulation of gas exchange
Management aimed to control pulmonary hypertension
Supportive management
Long-range thinking
This is specifically to answer Question 13 from the first paper of 2017.
Barriers due to the disease process
Barriers due to side effects of therapy
Sequelae of prolonged ICU stay
Saydain, Ghulam, et al. "Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit." American journal of respiratory and critical care medicine 166.6 (2002): 839-842.
Collard, Harold R., et al. "Acute exacerbations of idiopathic pulmonary fibrosis." American journal of respiratory and critical care medicine 176.7 (2007): 636-643.
Liang, Zhan, et al. "Referral to Palliative Care Infrequent in Patients with Idiopathic Pulmonary Fibrosis Admitted to an Intensive Care Unit." Journal of palliative medicine 20.2 (2017): 134-140.
Moran, J. L., and P. Rangappa. "Outcomes of patients admitted to the intensive care unit with idiopathic pulmonary fibrosis." Critical Care and Resuscitation 11.2 (2009): 102.
Raghu, Ganesh, et al. "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management." American journal of respiratory and critical care medicine 183.6 (2011): 788-824.
Ghofrani, Hossein Ardeschir, et al. The Lancet 360.9337 (2002): 895-900.
Minai, Omar A., et al. "Vaso-active therapy can improve 6-min walk distance in patients with pulmonary hypertension and fibrotic interstitial lung disease." Respiratory medicine 102.7 (2008): 1015-1020.
Fernández-Pérez, Evans R., et al. "Ventilator settings and outcome of respiratory failure in chronic interstitial lung disease." CHEST Journal 133.5 (2008): 1113-1119.
Juarez, Maya M., et al. "Acute exacerbation of idiopathic pulmonary fibrosis—a review of current and novel pharmacotherapies." Journal of thoracic disease 7.3 (2015): 499-519.