BACKGROUND AND AIM: Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease of unknown etiology. Acute exacerbation of IPF (AE-IPF) can cause sudden deterioration in the prognosis of the disease and is responsible for a significant proportion of deaths due to IPF. The aim of our study is to examine the medical histories, symptoms, clinical findings, laboratory tests, and radiological images of cases hospitalized in our clinic due to AE-IPF, and to determine mortality rates during and after the AE.

METHODS: We retrospectively examined the records of patients hospitalized with a diagnosis of AE-IPF. We recorded their demographic data, comorbidities, physical examination and laboratory findings, radiological findings, spirometry results, treatment status, intensive care needs, and mortality-related data. Statistical analyses were performed using Chi-square and Mann-Whitney U tests.

RESULTS: Out of 28 cases, 19 (67.9%) were male with a mean age of 67.1±11.4 years. The most common comorbidities were hypertension (39.3%), chronic obstructive pulmonary disease (35.7%), diabetes (32.1%), chronic renal failure (14.3%), and atrial fibrillation (14.3%). In respiratory function tests, mean forced vital capacity and carbon monoxide diffusion capacity was 65±17.1% and 47 47±14.2%, respectively. All patients had newly developed ground-glass opacity and/or consolidation areas in their chest computed tomography images. It was observed that the mean duration of steroid use initiated due to an AE was 7.1 days. Of the cases, 23 (82.1%) were discharged home, while the remaining 5 (17.9%) were transferred to the intensive care unit, where, unfortunately, all of them lost their lives. Among the discharged patients, 6 succumbed within three months. Consequently, the mortality rate within the hospital stay and the subsequent three months for patients hospitalized with AE-IPF was determined to be 39.3%. Notably, the mortality rate was significantly higher in patients with chronic renal failure compared to those without (p=0.016).

CONCLUSIONS: It was observed that the in-hospital and early post-discharge mortality rate for patients hospitalized with AE-IPF was approximately 40%. This finding underscores the serious negative impact of AEs on the prognosis of the disease.