Survey Finds Global Differences in Diagnosing, Treating Acute IPF Flares

Marisa Wexler MS avatar

by Marisa Wexler MS |

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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.