Demographic, clinical, radiological characteristics of patients with idiopathic pulmonary fibrosis and relationship of these characteristics with acute exacerbation

Introduction and Aim: Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease, the etiology of which is still unknown, which has a bad prognosis and is characterized by advanced fibrosis. It is the most common group among idiopathic interstitiel pneumonias. A limited number of studies have been carried out relating to the epidemiological characteristics of the disease. IPF acute exacerbation is a significant cause of mortality and morbidity. In our thesis, we aimed to investigate the demographic, clinical and radiological characteristics of patients with idiopathic pulmonary fibrosis and the relationship of these characteristics with acute exacerbation. Material and Method: 65 patients who applied to Çukurova University Medical Faculty Department of Chest Diseases between the years 2015-2020 and were diagnosed with IPF clinically/radiologically and/or histopathologically were evaluated retrospectively in our study. Age, genders, smoking histories, jobs, BMI, symptoms and family histories of the patients were recorded. Methods of IPF diagnosis, radiological and if presents pathological findings, respiratory function test measurements at the time of diagnosis, vaccination histories, 6-minute walking test, oxygen saturation values, and information relating to antifibrotic drug use and term of antifibrotic drug use were obtained from patient's files and recorded. Patients who were included in the study and had acute exacerbation were identified. Laboratory values and radiological characteristics during acute exacerbation and treatment modalities were recorded. Patients were divided into two groups based on the gender variable and presence of defenite/possible Usual Interstitial Pneumonia (UIP). The results were evaluated statistically. Findings: Fifty-two (80%) of the patients who were included in our study were male and 13 (20%) were female. The age average of the patients was identified as 67.86±7.17 (54-84). Forty-three patients (66.2%) had a smoking history. Average BMI was identified as 26.21±4.04. Familial IPF was identified in 4 (6.2%) patients. Dyspnea was identified in 63 (96.9%) patients, cough was identified in 62 (95.4%) patients and finger clubbing was identified in 35 (53.8%) patients. Average Forced Vital Capacity (FVC) value was identified as 2.36±0.65 (expected value in terms of percentages was 73.23±14.99), Diffusing Capacity of Lung for Carbon Monoxide (DLCO) value was identified as 3.26±1.34 (expected value in terms of percentages was 43.16±14.89). Sixty-one patients were observed to be diagnosed clincially and radiologically (6.2%) and 4 (6.2%) by surgical biopsy. Acute exacerbation history was identified in 7 (10.8%) patients. Cigarette use ratio's being higher in male patients than in female patients was found to be statistically significant (p<0,05). Age average of female patients was identified to be statistically significantly higher than the age average of male patients (p<0,05). Average FVC and DLCO values' being lower in female patients was found to be statistically significant (p<0,05). The fact that the presence of exacerbation history is more common in patients with a possible UIP pattern was found to be statistically significant (p<0,05). Conclusion: Based on these findings, we have observed that the most of the patients were middle-old aged, average age of female patients was higher and the patients had a smoking history to a large extent. We observed that radiological diagnosis comprises a basis for IPF diagnosis. IPF acute exacerbation was observed to be more common in the possible UIP pattern group. Broader, multicenter studies with more participants are needed to determine the demographic, clinical and radiological characteristics of IPF patients during stable and acute exacerbations. Keywords: Acute exacerbation, Demographic characteristics, IPF, Treatment