Other than referring early to a lung transplant center, there are no strong expert recommendations on how to help patients with idiopathic pulmonary fibrosis (IPF). Trials of therapies have been a trail of tears. So you might think, since there are no treatments, it shouldn’t matter who’s doing the non-treating. But it just might, say Lamas et al from Columbia University.
They report on 129 patients referred to their tertiary academic center for management of IPF (44 biopsy-proven). More than half were referred more than 2 years after the first onset of dyspnea. After controlling for variables (age, sex, forced vital capacity, insurance status, and educational level), a delay in referral to their tertiary center was associated with an increased risk of death. (Hazard ratio of 1.3 for each “doubling of delay” — they arbitrarily defined cohorts who had referral delays of <1, 1-2, 2-4, or 4 years).
It wasn’t only those who were older or with more-advanced IPF at the time of referral that accounted for the mortality difference (neither severity of IPF nor age varied by time of delay). The delays did not affect the rate of lung transplantation, so that was not felt to be the reason. Patients with comorbid conditions including coronary artery disease and diabetes mellitus were more likely to have a delay in referral, which sounds to me like more than enough reason to explain the mortality difference observed.
Although their P-values showed no evidence of race, insurance status, age, education, etc. as interacting with mortality, their sample size was small and it seems it would be hard to rule out an interaction. Also, the findings are from their single center. Still, very interesting. They postulate that later-referred patients may spend some time getting inappropriate therapies like high-dose steroids, or that early referral leads to earlier institution of appropriate therapies. However, they report no data on this. I think their statement, “it may be that those who are referred later are less healthy in ways that are not readily apparent” says a lot. Their delayed-referral patients had more CAD and diabetes … it’s easy to imagine they were sicker and/or took less care of themselves in general in other unmeasured ways.
Lamas DL et al. Delayed Access and Survival in Idiopathic Pulmonary Fibrosis: a cohort study. Am J Respir Crit Care Med 2011;184:842-847.