New Guidelines Released for IPF Diagnosis

A panel of international experts have released updated criteria for physicians to diagnose idiopathic pulmonary fibrosis (IPF). 

Members of the American Thoracic Society, the European Respiratory Society, Japanese Respiratory Society and the Latin American Thoracic Society all took part in developing the new guidelines. Twenty-nine people, including pulmonologists, radiologists, pathologists, methodologists and an IPF patient, made up the committee. 

Idiopathic pulmonary fibrosis is a rare and usually fatal lung disease. It’s the deadliest and most common form of a combination of over 200 illnesses referred to as interstitial lung disease. It’s not clear what causes IPF, but according to Cleveland Clinic, risk factors include working near dust or fumes, older age (most diagnoses are made after age 60), smoking, male sex, and having a family member with the disease. Symptoms may develop overtime and could include dry cough, shortness of breath, fatigue, weight loss, widening and rounding around the fingertips, and swollen legs. Treatment is limited and there is currently no cure. Median survival following diagnosis is between three and five years. 

It’s #PFMonth – a time to educate others about the impact of the devastating rare lung disease #IPF. Help us raise awareness by sharing these resources: https://t.co/qmay311wu3 #BreathlessIPF pic.twitter.com/PZ3i2MJ5aV

— Boehringer Ingelheim (@boehringerus) September 1, 2018

“Diagnosing IPF is challenging because these symptoms are non-specific: they occur with all other interstitial lung diseases and with other respiratory problems,” said Dr. Ganesh Raghu, director at UW Medicine’s Center for Interstitial Lung Disease and chair of the committee. 

Early diagnosis of the disease is critical, Raghu said, because drugs may slow the progression of the disease. 

RT @davidlederer: New @atscommunity @ERSTalk @ALATIntersticio JRS guidelines for #IPF diagnosis. Easier to dx IPF without a biopsy… a step forward i think @ATSBlueEditor @PFFORG @BillS_PFF https://t.co/Nt3a2mJELs pic.twitter.com/QDiocJFRBK

— American Thoracic Society (ATS) (@atscommunity) September 7, 2018

One of the changes included an update to usual interstitial pneumonia (UIP) pattern definitions, which were refined to patterns of UIP, probable UIP, indeterminate, and alternate diagnosis. The experts also provided recommendations on performing surgical lung biopsy, transbronchial lung biopsy, lung cryobiopsy, and BAL depending on results from CT scans. 

“Our hope is that this new guideline will bridge the gap between the experienced IPF experts and general pulmonologists in making a prompt and accurate diagnosis of IPF for the individual unfortunately confronted with the disease,” Raghu said. “This will allow patients to make well-informed decisions about treatment options and participation in clinical trials.” 

The updated guidelines are available online as well as the American Thoracic Society’s Sept. 1 issue of the American Journal of Respiratory and Critical Care Medicine. 

Sources: UW Medicine, American Journal of Respiratory and Critical Care Medicine, Cleveland Clinic