Is It Idiopathic Pulmonary Fibrosis or Not?

Mary Salvatore, MD; Genta Ishikawa, MD; Maria Padilla, MD


J Am Board Fam Med. 2018;31(1):151-162. 

In This Article


UIP/IPF has a poor prognosis with a mean life expectancy of 3.8 years. The American Thoracic Society (ATS) has provided guidelines for the accurate diagnosis of IPF. This is a diagnosis of exclusion. There must be no known cause for a patient's lung fibrosis. The CT scan must show a UIP pattern with subpleural basilar-predominant fibrosis and honeycombing and absence of features that would suggest alternative diagnoses. A "possible UIP pattern" by ATS criteria includes the same criteria except honeycombing. If the patient does not have HC a lung biopsy may be considered to make the definitive diagnosis.[8]

In 2014, 2 antifibrotic medications, nintedanib and pirfenidone, were approved in the United States for the treatment of IPF. These daily administered medications are expensive but are often covered by insurance if patient has a diagnosis of UIP/IPF. These target multiple pathways of UIP[51] and slow the decline of the forced vital capacity (FVC). They may be helpful in other fibrotic lung disease but his has not been studied. Therefore, it is now critical to diagnose IPF early and accurately. Early referral to a pulmonologist with expertise in lung fibrosis may be beneficial. The early and correct diagnosis is challenged by mimickers which have similarities to UIP/IPF and include NSIP, CHP, and sarcoidosis. Fortunately, careful clinical history, serologic testing, CT inspection, and multidisciplinary discussion can establish the correct diagnosis without an invasive procedure (Table 2 and Table 3). It has been the goal of this article to share with primary care clinicians the most important aspects of the clinical and radiology presentation of IPF and its mimickers so that you will be able to correctly and noninvasively diagnosis UIP/IPF.