Background and Aims?

Several registries provide records for patients with idiopathic pulmonary fibrosis (IPF), yet few prospective cohort studies have examined patients with non-IPF idiopathic interstitial pneumonias (IIPs). We aimed to assess the disease progression and cause of death (COD) for IPF and non-IPF IIPs patients.?

Methods:

We analyzed data from a prospective multi-institutional cohort of 528 IIPs patients enrolled in Japan between September 2013 and April 2016. Central multidisciplinary discussion (MDD) was performed, and follow-up surveillance was executed up to 5 years after registration.

Results:

Table1 shows the base line characteristics. IPF was the most common diagnosis (58.1%), followed by unclassifiable IIPs (35.8%) and others (6.1%). The 5-year survival rate in IPF and non-IPF IIPs groups was 53.6% and 73.5%, respectively; chronic respiratory failure was the primary COD in both groups (Figure2). Acute exacerbation (AE) was the second most common COD in non-IPF IIPs patients (14.8%), as well as IPF patients (18.9%).

Conclusion:

This study demonstrated that the prognosis of non-IPF patients was better than that of IPF patients, but AE is an important COD for both groups.