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

Progressive pulmonary fibrosis (PPF) is possible among patients with connective tissue disease (CTD) related interstitial lung disease (ILD). Our aim herein was to compare the prevalence and clinical characteristics of patients with CTD-ILD, with and without PPF, according to the different diagnostic criteria currently used in practice. This retrospective study included patients diagnosed with CTD-ILD, with a ≥1-year follow-up of their lung function, at a single tertiary hospital in South Korea. Diagnostic criteria from two clinical trials (RELIEF and TRAIL1) and from a recently updated guideline (ATS/ERS/JRS/ALAT) were applied. Of the 107 patients included, 80% tested positive for Sjogren's disease, rheumatoid arthritis, and systemic sclerosis. The prevalence of CTD-ILD with PPF for the different diagnostic criteria was as follows: RELIEF, 25.2%; TRAIL1, 20.6%; and ATS/ERS/JRS/ALAT, 38.3%. More previous history of pulmonary tuberculosis and less positivity for antinuclear antibodies were identified in the PPF group. The radiologic pattern of ILD did not differ between patients with and without PPF, with a usual interstitial pneumonia pattern identified in 34.6% of the patients. Systemic steroids and immunomodulatory agents were used in about 80% of patients with PPF and 50% without PPF, irrespective of the diagnostic criteria used. Antifibrotic therapy was used in a limited number of patients in both groups. The proportion of patients with CTD-ILD and PPF was higher for the ATS/ERS/JRS/ALAT guideline criteria, without a between-group difference in clinical characteristics.

Lee J ，Kim K ，Jo YS 《-》

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

Kim K ，Lee J ，Jo YS 《-》

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

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.

Lee JK ，Ahn Y ，Noh HN ，Lee SM ，Yoo B ，Lee CK ，Kim YG ，Hong S ，Ahn SM ，Kim HC ... -  《-》

Comparison of characteristics of connective tissue disease-associated interstitial lung diseases, undifferentiated connective tissue disease-associated interstitial lung diseases, and idiopathic pulmonary fibrosis in Chinese Han population: a retrospectiv

Pan L ，Liu Y ，Sun R ，Fan M ，Shi G ... -  《-》

Clinical Characterization and Predictive Factors for Progression in a Cohort of Patients with Interstitial Lung Disease and Features of Autoimmunity: The Need for a Revision of IPAF Classification Criteria.

Bozzao F ，Tomietto P ，Baratella E ，Kodric M ，Cifaldi R ，Della Porta R ，Prearo I ，Pirronello SMG ，Confalonieri P ，Ruaro B ，Fischetti F ，Fabris B ... -  《-》