Clinical effect of progressive pulmonary fibrosis on patients with connective tissue disease-associated interstitial lung disease: a single center retrospective cohort study
-
Published:2023-10-13
Issue:8
Volume:23
Page:4797-4807
-
ISSN:1591-9528
-
Container-title:Clinical and Experimental Medicine
-
language:en
-
Short-container-title:Clin Exp Med
Author:
Lee Ju Kwang,Ahn Yura,Noh Han Na,Lee Sang Min,Yoo Bin,Lee Chang-Keun,Kim Yong-Gil,Hong Seokchan,Ahn Soo Min,Kim Ho Cheol
Abstract
AbstractThe 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.
Funder
National Research Foundation of Korea
Publisher
Springer Science and Business Media LLC
Subject
General Biochemistry, Genetics and Molecular Biology,General Medicine
Reference46 articles.
1. Garen T, Lerang K, Hoffmann-Vold A-M, Andersson H, Midtvedt Ø, Brunborg C, Kilian K, Gudbrandsson B, Gunnarsson R, Norby G, et al. Mortality and causes of death across the systemic connective tissue diseases and the primary systemic vasculitides. Rheumatology. 2018;58:313–20. 2. Li L, Zuo X, Luo H, Li Y, You Y, Duan L, Zhang W, Zhao H, Li T, Ning W, et al. Mortality trend of inpatients with connective tissue diseases: 2005–2014. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2017;42:927–33. 3. Oldham JM, Adegunsoye A, Valenzi E, Lee C, Witt L, Chen L, Husain AN, Montner S, Chung JH, Cottin V, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47:1767–75. 4. Yunt ZX, Chung JH, Hobbs S, Fernandez-Perez ER, Olson AL, Huie TJ, Keith RC, Janssen WJ, Goldstein BL, Lynch DA, et al. High resolution computed tomography pattern of usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease: Relationship to survival. Respir Med. 2017;126:100–4. 5. Park JH, Kim DS, Park IN, Jang SJ, Kitaichi M, Nicholson AG, Colby TV. Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes. Am J Respir Crit Care Med. 2007;175:705–11.
|
|