Comparison of the diagnostic criteria for progressive pulmonary fibrosis in connective tissue disease related interstitial lung disease

Lee, Jongmin; Kim, Kyuhwan; Jo, Yong Suk

doi:10.1016/j.rmed.2023.107242

Cited by 4 publications

(5 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A few years ago, the ILD guideline from the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society suggested that a decline ⩾10% in FVC or ⩾15% in DLco in the first 6–12 months was a risk factor for adverse outcome. 23 Our modified criteria for PPF were in line with previous study 24 and the prevalence of PPF in CTD-ILD cohort was 24.5%. Accompanied ILD is a leading cause of death in CTD patients.…”

Section: Discussionsupporting

confidence: 76%

Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease

Kim,

Lee,

2023

Ther Adv Respir Dis

Self Cite

View full text Add to dashboard Cite

Background: Progressive fibrosis can occur in connective tissue disease (CTD)-related interstitial lung disease (ILD) and make the prognosis worse. Objectives: This study aimed to investigate factors related to progressive pulmonary fibrosis (PPF) phenotype in CTD-ILDs. Design: Medical records of patients diagnosed as CTD and ILD at a single, tertiary hospital in South Korea were retrospectively reviewed. Methods: Patients whose lung functions were followed up for more than a year were included in analysis. PPF was defined as forced vital capacity (FVC) declined ⩾10% or diffusion capacity of carbon monoxide (DLco) ⩾15%. Results: Of 110 patients with CTD-ILD, 24.5% progressed into PPF. Rheumatoid arthritis (RA) and Sjogren’s disease accounted for more than 63% of PPF. Compositions of CTD type were similar between PPF and non-PPF. Clinical characteristics and proportion of usual interstitial pneumonia (UIP) pattern on chest images were also similar between PPF and non-PPF. Approximately 10% of patients in both groups were treated with anti-fibrotic agents. Use of systemic steroids and/or other immunomodulating agents lowered the risk of developing PPF in CTD-ILD patients after adjusting for gender-age-physiology score and smoking status (adjusted odds ratio: 0.25, 95% confidence interval: 0.07–0.85). Conclusion: About a quarter of CTD-ILD progressed into PPF. The use of immunomodulating agents lowered the risk of developing PPF. To improve outcomes of patients, future studies need to detect patients at higher risk for PPF earlier and set up clinical guidelines for treatment strategies in the process of PPF.

show abstract

Section: Discussionsupporting

confidence: 76%

Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease

Kim,

Lee,

2023

Ther Adv Respir Dis

Self Cite

View full text Add to dashboard Cite

show abstract

“…The lower prevalence of PPF in the present study than in previous studies may be attributed to several factors. First, the shorter follow-up period in the present study than in previous studies might have contributed to the lower observed prevalence (median follow-up duration: 17.4, IQR; 7.4–29.4 months) when compared with the median follow-up duration of 49.9 ± 36.5 months reported by Lee et al and of 6 (IQR 6–9) years by Chiu et al [ 14 , 15 ]. In addition, the application of stricter criteria for radiologic deterioration, assessed by two radiologists, could have resulted in a more stringent evaluation.…”

Section: Discussioncontrasting

confidence: 66%

“…According to previous studies, the prevalence of PPF in patients with CTD-ILD ranged from 23 to 38% [ 14 – 16 ]. In the present study, 37 (18.8%) patients met the diagnostic criteria of PPF, which is relatively lower than those in previous studies.…”

Section: Discussionmentioning

confidence: 99%

“…These results might be attributed to the difference in the study design. Lee et al [ 14 ] analyzed 107 patients with CTD and identified PPF in 38.3% of these patients. In addition, Chiu et al [ 15 ] reported that 53 (23%) of their 230 patients with CTD-ILD had a PPF with median follow-up of 6 years.…”

Section: Discussionmentioning

confidence: 99%

“…According to previous studies, the prevalence of PPF in patients with CTD-ILD ranged from 23 to 38% [ 14 – 16 ]. However, to our knowledge, there is currently no large-scale, well-designed study available to investigate the clinical impacts of PPF in patients with CTD.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical effect of progressive pulmonary fibrosis on patients with connective tissue disease-associated interstitial lung disease: a single center retrospective cohort study

Lee,

Ahn,

Noh

et al. 2023

Clin Exp Med

View full text Add to dashboard Cite

The concept of progressive pulmonary fibrosis (PPF) has been introduced to predict the diverse prognosis of interstitial lung disease (ILD). However, the incidence and effect of PPF on outcomes in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) need to be elucidated. This study reviewed 197 patients with CTD-ILD. Symptomatic worsening, pulmonary function decline, and radiological deterioration were investigated to assess the fulfillment of PPF diagnostic criteria. Clinical outcomes, including mortality, were compared based on the presence or absence of PPF. The median follow-up duration was 17.4 months. The mean age of the patients was 64.0 years, and 60.9% were female. Among the underlying CTDs, rheumatoid arthritis (42.1%), inflammatory myositis (19.8%), and systemic sclerosis (13.2%) were the most common. Of the 197 patients, 37 (18.8%) met the diagnostic criteria for PPF during the follow-up period. Even after adjusting for other significant risk factors, PPF was independently associated with mortality [hazard ratio (HR) 3.856; 95% confidence interval (CI) 1.387–10.715; P = 0.010] and baseline albumin was marginally significantly associated with mortality (HR 0.549; CI 0.298–1.010; P = 0.054). The median survival was also significantly shorter in the PPF group than in the non-PPF group (72.3 ± 12.9 vs. 126.8 ± 15.5 months, P < 0.001). Baseline KL-6 ≥ 1000 (U/mL) was a significant risk factor for PPF (HR 2.885; CI 1.165–7.144; P = 0.022). In addition to increased mortality, the PPF group had significantly higher rates of respiratory-related hospitalizations, pneumonia, acute exacerbations, and weight loss than the non-PPF group. PPF is a significant prognostic indicator in patients with CTD-ILD. Thus, healthcare professionals should know that patients with CTD-ILD are at risk of PPF.

show abstract

Usefulness of CT Quantification-Based Assessment in Defining Progressive Pulmonary Fibrosis

Ahn,

Kim,

Lee

et al. 2024

Academic Radiology

View full text Add to dashboard Cite

Comparison of the diagnostic criteria for progressive pulmonary fibrosis in connective tissue disease related interstitial lung disease

Cited by 4 publications

References 36 publications

Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease

Factors for progressive pulmonary fibrosis in connective tissue disease-related interstitial lung disease

Clinical effect of progressive pulmonary fibrosis on patients with connective tissue disease-associated interstitial lung disease: a single center retrospective cohort study

Usefulness of CT Quantification-Based Assessment in Defining Progressive Pulmonary Fibrosis

Contact Info

Product

Resources

About