Gender differences at presentation of idiopathic pulmonary fibrosis in Sweden

Abstract

BackgroundIdiopathic pulmonary fibrosis (IPF) is a disease with poor prognosis mainly affecting males. Differences in clinical presentation between genders may be important both for the diagnostic work-up and for follow-up. In the present study, we therefore explored potential gender differences at presentation in a Swedish cohort of IPF-patients.MethodsWe studied patients included in the Swedish IPF- registry over a three-year period from its launch in 2014. A cross-sectional analysis was performed for data concerning demographics, lung function, 6- min walking test (6MWT) and quality of life (QoL) (King’s Brief Interstitial Lung Disease (K-BILD) score).ResultsThree hundred forty- eight patients (250 (72%) males, 98 (28%) females, median age 72 years in both genders) were included in the registry during the study period. Smoking history (N = 169 (68%) vs. N = 53 (54%), p < 0.05), baseline lung function (Forced vital capacity, % of predicted (FVC%): 68.9% ± 14.4 vs. 73.0% ± 17.7, p < 0.05; Total lung capacity, % of predicted (TLC%): 62.2% ± 11.8 vs. 68.6% ± 11.3%, p < 0.001) were significantly different at presentation between males and females, respectively. Comorbidities such as coronary artery disease (OR: 3.5–95% CI: 1.6–7.6) and other cardiovascular diseases (including atrial fibrillation and heart failure) (OR: 3.8–95% CI: 1.9–7.8) also showed significant differences between the genders. The K- BILD showed poor quality of life, but no difference was found between genders in total score (54 ± 11 vs. 54 ± 10, p = 0.61 in males vs. females, respectively).ConclusionsThis study shows that female patients with IPF have a more preserved lung function than males at inclusion, while males have a significant burden of cardiovascular comorbidities. However, QoL and results on the 6MWT did not differ between the groups. These gender differences may be of importance both at diagnosis and follow- up of patients with IPF.

Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the Swedish IPF Registry

“…Thus, approximately 65% of the patients received anti-fibrotic treatment in our study, which is considerably more than in Germany (44%) [ 9 ], Finland (26%) [ 10 ], and Australia (23%) [ 6 ]. The present study shows that patients on anti-fibrotic therapy appear to survive longer than untreated patients, a result similar to what other registries have reported [ 6 , 8 , 20 ]. In order to avoid a potential bias in mortality analysis, we showed that there were no significant differences in baseline lung function between anti-fibrotic treated and untreated groups.…”

“…The Swedish IPF Registry (SIPFR) is a nationwide registry collecting comprehensive longitudinal data of IPF patients and implemented in 22 respiratory medicine units across Sweden [ 13 , 19 , 20 ]. The SIPFR also includes patients diagnosed before the registry was launched in 2014.…”

“…However, the impact of these physiological variables on disease progression and mortality in patients with mild or more advanced disease is largely unknown. Furthermore, we have previously indicated potential gender differences in patients with IPF [ 20 ]. Thus, an unsupervised cluster analysis may provide novel insights into the phenotypes of IPF with potential prognostic significance.…”

COVID-19 Associated Interstitial Lung Disease - An Emerging Entity

“…The majority of our patients who developed COVID-associated ILD were males, which has also been previously reported in literature with certain ILDs like idiopathic pulmonary brosis [13,14]. COVID associated brosis is one of the lung insults already described with previous Coronavirus infections [2,3], and there are emerging studies now reporting COVID-19 associated early pulmonary brosis.…”

Respiratory Sex Differences in Response to Smoke Exposure

“…Other substantial respiratory outcomes are also associated with smoke exposure, though evidence is more limited. For example, percent of predicted forced vital capacity (FVC%) and percent of predicted total lung capacity (TLC%) were lower in males compared to females in a study of idiopathic pulmonary fibrosis (IPF) in Sweden (Kalafatis et al 2019 ). When smoking history was considered, male ex-smokers showed lower FVC%, TLC%, and percent of predicted diffusing capacity for carbon monoxide (DLCO%), compared to female ex-smokers.…”