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

RATIONALE AND OBJECTIVES: To establish a quantitative CT threshold for radiological disease progression of progressive pulmonary fibrosis (PPF) and evaluate its feasibility in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). MATERIALS AND METHODS: Between April 2007 and October 2022, patients diagnosed with CTD-ILD retrospectively evaluated. CT quantification was conducted using a commercial software by summing the percentages of ground-glass opacity, consolidation, reticular opacity, and honeycombing. The quantitative threshold for radiological progression was determined based on the highest discrimination on overall survival (OS). Two thoracic radiologists independently evaluated visual radiological progression, and the senior radiologist's assessment was used as the final result. Cox regression was used to assess prognosis of PPF based on the visual assessment and quantitative threshold. RESULTS: 97 patients were included and followed up for a median of 30.3 months (range, 4.7-198.1 months). For defining radiological disease progression, the optimal quantitative CT threshold was 4%. Using this threshold, 12 patients were diagnosed with PPF, while 14 patients were diagnosed with PPF based on the visual assessment, with an agreement rate of 97.9% (95/97). Worsening respiratory symptoms (hazard ratio [HR], 12.73; P < .001), PPF based on the visual assessment (HR, 8.86; P = .002) and based on the quantitative threshold (HR, 6.72; P = .009) were independent risk factors for poor OS. CONCLUSION: The quantitative CT threshold for radiological disease progression (4%) was feasible in defining PPF in terms of its agreement with PPF grouping and prognostic performance when compared to visual assessment.

The Association Between Sputum Culture Conversion and Mortality in Cavitary Mycobacterium avium Complex Pulmonary Disease.

BACKGROUND: The association between treatment outcome and the mortality of Mycobacterium avium complex pulmonary disease (MAC-PD) with cavitary lesions is unclear. This article assessed the impact of culture conversion on mortality in patients with cavitary MAC-PD. RESEARCH QUESTION: Is the achievement of sputum culture conversion in MAC-PD with cavitary lesions associated with the prognosis? STUDY DESIGN AND METHODS: From 2002 to 2020, a total of 351 patients with cavitary MAC-PD (105 with the fibrocavitary type and 246 with the cavitary nodular bronchiectatic type), who had been treated with a ≥ 6-month macrolide-containing regimen at a tertiary referral center in the Republic of Korea, were retrospectively enrolled in this study. All-cause mortality during the follow-up period was analyzed based on culture conversion at the time of treatment completion. RESULTS: The cohort had a median treatment duration of 14.7 months (interquartile range [IQR], 13.4-16.8 months). Of the 351 patients, 69.8% (245 of 351) achieved culture conversion, and 30.2% (106 of 351) did not. The median follow-up was 4.4 years (IQR, 2.3-8.3 years) in patients with culture conversion and 3.1 years (IQR, 2.1-4.8 years) in those without. For the patients with and without culture conversion, all-cause mortality was 5.3% vs 35.8% (P < .001), and the 5-year cumulative mortality was 20.0% vs 38.4%, respectively. Cox analysis found that a lack of culture conversion was significantly associated with higher mortality (adjusted hazard ratio, 5.73; 95% CI, 2.86-11.50). Moreover, the 2-year landmark analysis revealed a distinct impact of treatment outcome on mortality. INTERPRETATION: The mortality rate of patients with cavitary MAC-PD who did not achieve culture conversion was significantly higher than that of those with culture conversion.

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.

Clinical impact of weight loss on mortality in patients with idiopathic pulmonary fibrosis: a retrospective cohort study.

Patients with idiopathic pulmonary fibrosis (IPF) often experience weight loss during the follow-up period. However, the prevalence and clinical impact of weight loss in these patients still need to be elucidated. This retrospective single-center study reviewed 134 consecutive patients diagnosed with IPF. Weight loss of 5% or more over 1 year was defined as significant weight loss. Clinical data of patients were compared according to the significant weight loss. We analyzed whether the clinical impact of significant weight loss differed regarding the pirfenidone dose. The median follow-up period was 22.1 months. The mean age of patients was 67.3 years, and 92.5% were men. Of the 134 patients, 42 (31.3%) showed significant weight loss. Multivariate cox regression analysis revealed that significant weight loss was independently associated with mortality (hazard ratio [HR]; 2.670; 95% confidence interval [CI] 1.099-6.484; p = 0.030) after adjusting for lung function and other significant risk factors (6-min walk test distance: HR, 0.993; 95% CI 0.987-0.998; p = 0.005). The median survival of patients with significant weight loss (n = 22) was relevantly shorter than that of those without significant weight loss (n = 43) in the reduced dose pirfenidone group (28.2 ± 3.3 vs. 43.3 ± 3.2 months, p = 0.013). Compared with patients without significant weight loss (n = 38), patients with significant weight loss (n = 15) also showed a marginally-significant shorter survival in the full-dose pirfenidone group (28.9 ± 3.1 vs. 39.8 ± 2.6 months, p = 0.085). Significant weight loss is a prognostic factor in patients with IPF regardless of pirfenidone dose. Vigilant monitoring might be necessary to detect weight loss during the clinical course in these patients.