A national survey of 600 people with interstitial lung disease (ILD) commissioned by the Pulmonary Fibrosis Foundation (PFF) revealed that a high percentage of patients are enduring significant diagnostic delays and errors.

The PFF conducted the Interstitial Lung Disease Patient Diagnostic Journey (INTENSITY) survey, with support from molecular diagnostics company Veracyte Inc, to advance understanding of patients’ diagnostic experiences with interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF). Results of the survey will be presented in Washington, DC at the PFF Summit 2015.

An estimated 175,000 to 200,000 patients in the United States and major European countries present with suspected ILDs each year, according to the PFF. Among the known forms of ILD, IPF is among the most common and the most deadly. There are no known cures for IPF and patients have a median survival time of just 3.8 years — worse than most forms of cancer.

The INTENSITY survey assessed the steps and time required for adults living with ILD to receive an accurate diagnosis, specific obstacles hindering timely diagnosis, and the physical and emotional impact of patients’ diagnostic journeys.

Key findings demonstrate diagnostic delays and misdiagnoses, including:

  • 42% of survey respondents endured a year or more between the time they first experienced symptoms and the time they obtained a diagnosis; 25% endured two years or more; and 6% endured six years or more;
  • 55% of survey respondents were misdiagnosed at least once and 38% were misdiagnosed at least twice over the course of diagnosing their disease;
  • Among those who were misdiagnosed, the misdiagnoses persisted for nearly a year.

“The diagnostic delays and misdiagnoses that patients experience are particularly troubling given that we now have effective treatments for both fibrotic and inflammatory forms of ILD,” said David Lederer, MD, MS, PFF senior medical advisor on education and awareness. “The results of the INTENSITY survey clearly signal the need for a change in primary care-provider education.”

Among survey participants, 47% of whom reported having IPF, an accurate diagnosis typically required the involvement of three physicians and multiple diagnostic tests, including (for many) invasive diagnostic procedures.  This diagnostic odyssey is time-consuming and can be clinically risky.

  • Patients saw a median of three physicians before receiving a diagnosis
  • Most ILD diagnoses (88%) were made by a pulmonologist, however multiple PCP visits were the norm: 75% of patients saw their primary care physician more than once and 21% saw their PCP more than four times before seeing a specialist
  • On average, respondents endured six pulmonary lung function texts, five chest X-rays, and two bronchoscopies before receiving a diagnosis
  • Nearly half of survey participants underwent a surgical lung biopsy, an invasive, expensive and potentially risky procedure, as part of their diagnostic process

“IPF is a complicated disease to diagnose because its signs and symptoms mimic a wide range of common medical conditions, and it requires many resources and different specialties,” Lederer said. “These survey results are a wake-up call. We need to raise awareness of the disease and its symptoms, and improve diagnoses to help patients avoid the anxiety, uncertainty, and cost – as well as the potential clinical implications – associated with a protracted diagnostic journey and the invasive diagnostic procedures involved.”