A nationwide patient survey highlights the lengthy and often inaccurate diagnosis of interstitial lung disease (ILD), as well as the emotional stress it causes in patients.

The research, “Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey,” was published in the journal BMC Pulmonary Medicine.

The Interstitial Lung Disease Patient Diagnostic Journey (INTENSITY) survey quantified the challenges of diagnosing ILD and was conducted through a collaboration between the Pulmonary Fibrosis Foundation and Veracyte, a genomic diagnostics company.

Idiopathic pulmonary fibrosis (IPF) is a chronic type of ILD that causes progressive scarring of the lungs. Diagnosis of IPF is currently done with high-resolution computed tomography (HRCT), which frequently leads to inconclusive diagnosis. Because of that, patients often need to undergo risky surgeries to confirm their disease. Others are too frail for such an invasive approach, leading to potential misdiagnosis and ineffective treatment.

Researchers conducted the online INTENSITY survey to evaluate the challenges involved in ILD diagnosis, such as the obstacles and time needed for accurate diagnosis, as well as the physical and emotional toll on patients.

INTENSITY included 600 ILD patients — 300 men (median age 69 years) and 300 women (median age 63 years); 279 (47 percent) of whom had IPF. Most patients had been diagnosed in the prior two to five years.

Results showed that 43 percent of patients had to wait more than a year from the onset of the first symptoms, mainly shortness of breath and cough, until diagnosis. Almost one in every five (19 percent) waited three or more years for a correct diagnosis.

Furthermore, more than half of the patients (55 percent) reported at least one misdiagnosis before receiving a correct diagnosis, with 38 percent receiving at least two misdiagnoses.

Importantly, 21 percent of the respondents with IPF reported receiving treatment with systemic corticosteroids prior to diagnosis. Such therapy can be harmful for IPF patients.

“Our results show that the typical diagnostic experience for patients with ILD, including IPF, is characterized by considerable delays, frequent misdiagnosis, exposure to costly and invasive diagnostic procedures and substantial use of healthcare resources,” Gregory P. Cosgrove, MD, the study’s lead author, said in a press release. Cosgrove is chief medical officer at the Pulmonary Fibrosis Foundation.

The results also showed that although an accurate diagnosis of ILD was primarily done by a pulmonologist (88 percent of the cases), multiple prior visits to a primary care physician were frequent, with 30 percent of patients making four or more visits before referral to a specialist.

“These findings underscore the need for physician education, practical clinical guidelines, and improved diagnostic tools that increase the speed and accuracy of diagnosis and facilitate early therapeutic intervention,” the researchers wrote.

Concerning examinations conducted before diagnosis, patients underwent an average of six lung function tests, five chest X-rays, and two bronchoscopies. Furthermore, 61 percent of ILD patients underwent invasive procedures, including surgical lung biopsy.

More than 80 percent of patients had emotional stress associated with the difficulty in obtaining an accurate diagnosis. Also, 28 percent revealed that the time spent on medical appointments and doing diagnostic procedures contributed to their decision to apply for disability benefits or retirement.

“The publication of these findings underscores the tremendous need for patients with suspected IPF to get clearer answers faster so that they can receive the treatment they need and avoid potentially harmful diagnostic procedures and treatment,” said Bonnie Anderson, chairman and CEO at Veracyte.

Aiming to improve IPF diagnosis without requiring surgery and to reduce healthcare costs, Veracyte introduced its Envisia Genomic Classifier in October 2016. This technology uses samples collected from less-invasive bronchoscopies.

Veracyte previously reported the validity of the Classifier tool in the diagnosis of IPF and lung cancer.