IPF often slow to be diagnosed in US, proper treatment lacking

Study finds 98% of 45K patients initially misdiagnosed, 10% on anti-fibrotic meds

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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Many people with idiopathic pulmonary fibrosis (IPF) in the U.S. are not diagnosed early enough or given treatments helping to low its progression in a timely fashion, a large data study found.

Although more is known about risk factors for IPF, delays are evident in diagnosis and the start of treatment.

“We hope to reduce the delay to IPF diagnosis and treatment initiation by increasing the awareness of care of IPF patients and by enhancing education for providers on the appropriate diagnostic approach,” its researchers wrote.

The study, “Idiopathic pulmonary fibrosis in the United States: time to diagnosis and treatment,” was published in the journal BMC Pulmonary Medicine.

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IPF diagnosis ‘challenging’ due to similarities with other lung conditions

Pulmonary fibrosis causes lung tissue to thicken and stiffen, and to form scars over time. Scarring in the lungs makes it more difficult for patients to breathe, which can lead to shortness of breath and a dry, hacking cough.

Why this happens often is unknown, with the disease considered to be idiopathic.

Because the symptoms of pulmonary fibrosis are similar to those of other lung diseases, “the diagnosis of IPF can be challenging,” the researchers wrote. However, a proper diagnosis and early start with disease treatments are crucial for patients’ outcomes.

Researchers with the Mayo Clinic, in Minnesota, traced a timeline showing how IPF is diagnosed and treated in a real-world clinical setting in the U.S.

They looked at patient data from January 2014 through December 2019 in Medicare’s fee-for-service program, which includes over 50,000 people with IPF across the U.S.

Researchers identified 44,891 adults, age 50 or older, with IPF and “five years of consecutive [program] enrollment and data.” This group had a mean age of 78.8, was majority white (92.8%), and included about as many women as men (50.7% vs. 49.3%).

Before being diagnosed with IPF, more than half of these patients (56.6%) had a history of tobacco use.

Most patients (98.2%) were diagnosed with another respiratory condition before IPF was confirmed. The most common were upper respiratory infections (46.6%) or acute bronchitis (12.6%), a sudden inflammation of the large airways leading to the lungs.

On average, it took about 2.7 years from the time patients were first diagnosed with a breathing problem to when they were diagnosed with IPF. About 55% were hospitalized at least twice for breathing problems before a correct diagnosis.

‘Sobering reality’ of diagnostic delays, therapy underutilization

Around 1 in 10 patients (4,649 people) received treatment with anti-fibrotic medications that help to slow disease progression. For 1,205 (25.9%) of these patients, treatment began an average of 7.9 months before their IPF diagnosis, while for 3,444 others (74.1%), it started an  average of 6.8 months after diagnosis.

“While widely believed that patients with IPF experience significant diagnostic delays, this observational study provides insights among patients with IPF across all care settings,” the researchers wrote. “Unfortunately, our observations support the sobering reality of significant diagnostic delay of IPF and underutilization of available therapies for this disorder.”

Men were more likely than for women to receive anti-fibrotic medications, though such treatment was low for both groups (13.2% vs. 7.6%).

Anti-fibrotic medication also was most commonly prescribed patients ages 65 to 74 (15.1%), followed by those ages 75 to 84 (10.3%). Such treatment was more likely in white patients (10.5%, a majority study group) than Blacks (6.5%; 2% of study group) or Hispanics (8.9%; 2.9% of study group).

But one-quarter of these people did not refill an initial prescription, the study found, possibly due to out-of-pocket treatment costs in the U.S., side effects of anti-fibrotic medications, or advanced disease at diagnosis leading doctors to consider such treatment “futile.”

Over half (56.1%) of the patients used oxygen therapy to help with breathing at some point during the disease course, with 16,808 patients (37.4%) starting oxygen therapy before being diagnosed with IPF.

The average time between first use of oxygen therapy and an IPF diagnosis was 2.2 years; after diagnosis, oxygen therapy typically started within one year (283 days).

Survival chances among patients hospitalized for breathing problems fell significantly each year, with about half living for two years after a first breathing-related hospitalization. Overall, patients lived a median of 2.8 years after being diagnosed with IPF.

Very few patients — 124 or 0.28% — received a lung transplant for IPF after its diagnosis, which “may in part reflect the overall age of the individuals in the study population,” the researchers wrote.

“Despite increasing knowledge regarding the risk factors and available treatments for IPF, delays in the diagnosis of this disorder and the initiation of therapy persist,” the researchers wrote, adding that “prognosis remains poor following diagnosis.”

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