Across the world, a fair amount of variation exists in medical practices to diagnose and treat acute exacerbations of idiopathic pulmonary fibrosis (IPF), reflecting a lack of evidence-based guidelines, a study based on surveys of pulmonologists reports.
The study, “Acute exacerbation of idiopathic pulmonary fibrosis: international survey and call for harmonisation,” was published in the European Respiratory Journal.
An acute exacerbation of IPF (AE-IPF) is a sudden worsening of lung function, posing a substantial health risk. It is estimated that nearly half of deaths (46%) in people with IPF are associated with AE-IPF. Despite this, there are no international guidelines regarding how an AE-IPF should be diagnosed or treated.
An international research team conducted a survey among lung-specialist physicians worldwide. The survey was designed after a review of available scientific literature. Questions were asked regarding practices used to diagnose, treat, and prevent AE-IPF, as well as opinions on future directions for research.
In total, 509 pulmonologists from 66 countries responded: 217 (42.6%) from Europe, 136 (26.7%) from Asia, 57 (11.2%) from North America, 50 (9.8%) from South America, 25 (4.9%) from Australia and New Zealand, 5 (1%) from Africa, and 19 (3.7%) with unspecified locations.
Some similarities were evident in how physicians approached the treatment of AE-IPF. For example, nearly all respondents reported using corticosteroids (a class of medicines that limits the activity of the immune system) in treating these patients, followed by a slow tapering.
However, other treatment approaches were not so universally agreed upon. Some respondents reported using immune-suppressing therapies besides corticosteroids, but their use varied significantly based on the respondents’ geographical location.
As an example, “cyclophosphamide was used by 28% in Asia, and never in North America,” the researchers wrote.
Very few pulmonologists, 4%, reported never using an immune-suppressing therapy to treat AE-IPF.
Use of anti-fibrotic therapies was also mixed: while a majority (67%) recommended the approved IPF therapies Esbriet (pirfenidone) or Ofev (nintedanib) as AE-IPF treatments, a third (33%) did not consider these therapies to be helpful in an acute setting.
To diagnose AE-IPF, most respondents (76%) reported using high-resolution computerized tomography (HRCT) scans, a widely accepted diagnostic tool. But its rates of use varied from place-to-place (from 91% in Asia to 67% in Europe).
The use of other diagnostic tools, such as blood markers, also varied. For example, KL-6 was measured by over half of doctors from Asia (54%), but by no other respondents.
Infections may play a role in some AE-IPF cases. Rates of testing for infections varied from place-to-place, and these pulmonologists reported testing for different specific infections.
The use of antibiotics and other medicines meant to control infections also varied substantially. About half (56%) of respondents reported commonly using broad-spectrum antibiotics (even in the absence of a confirmed infection), while about a quarter (23%) said they would only use antibiotics if there was a confirmed bacterial infection.
Regarding prevention, 93% of respondents agreed that vaccinations (which can lower the risk of specific infections) were a useful way to prevent AE-IPF. Anti-fibrotic therapies were also deemed good prevention strategies by most (86%) doctors.
As for future directions in the field, many respondents noted a need for more research into treatments (86%), better understanding of the biology underlying AE-IPF (83%), and generating consensus guidelines for AE-IPF (79%). A need for better collaboration between different lung disease specialists in general was expressed by 60% of respondents.
These findings suggest a large variability in how AE-IPF is managed around the globe, and highlight a need for clear guidelines to standardize care of IPF patients experiencing acute exacerbations.
“In conclusion, the heterogeneity of management of AE-IPF as found in this international survey reflects the lack of evidence and focused guidelines on important aspects of the management of AE-IPF,” the researchers wrote.
“This strongly calls for research, education, and collaborations between ILD [interstitial lung disease]-specialist around the world to find new ways to approach this deadly complication of IPF,” they added.
We are sorry that this post was not useful for you!
Let us improve this post!
Tell us how we can improve this post?