Predictors for Progressive Fibrosis in Patients with Connective Tissue Disease Associated Interstitial Lung Diseases

Yu-Hsiang Chiu¹, Julia Spierings², Pim de Jong¹, Firdaus Mohamed Hoesein¹, Jan Grutters³, Jacob van Laar¹ and Mareye Voortman¹, ¹University Medical Centre Utrecht, Utrecht, Netherlands, ²University Medical Center Utrecht, Utrecht, Netherlands, ³St. Antonius Hospital, Nieuwegein, Netherlands

Meeting: ACR Convergence 2021

Keywords: autoimmune diseases, interstitial lung disease

Session Information

Date: Saturday, November 6, 2021

Title: Systemic Sclerosis & Related Disorders – Clinical Poster I (0387–0413)

Session Type: Poster Session A

Session Time: 8:30AM-10:30AM

Background/Purpose: Connective tissue disease associated interstitial lung disease (CTD-ILD) is associated with decreased quality of life and high mortality risk. Outcome and treatment response is unpredictable. This study aimed to identify clinical predictors for CTD-ILD with poor outcome.

Methods: We performed a retrospective single center cohort study in outpatients with CTD-ILD seen between 2004 and 2018. Clinical, biochemical data as well as pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results were recorded. The ILD patterns were classified according to the classification for idiopathic interstitial pneumonia and categorised into fibrotic or inflammatory. Overall survival and progressive fibrosing ILD (PF-ILD, defined as ≥ 10% decline in FVC, ≥ 15% decline in DLCO, or progression of the fibrosis on HRCT within two years) were assessed.

Results: In total, 150 patients with CTD-ILD were included. Table 1 shows the baseline patient characteristics. Thirty (20%) deaths occurred during a median follow-up of 40 months (IQR 27.3–60.8), which were attributed to pulmonary infection in six (4%), respiratory failure due to PF-ILD in ten (7%) and due to other causes in fourteen patients. PF-ILD occurred in 74 (49.3%) patients and was associated with poor overall survival. (Figure 1) Age, smoking, C-reactive protein, and steroid-use were independently associated with increased mortality risk as well. (Table 2) Furthermore, patients with diabetes mellitus (adjusted OR 4.2, 95%CI 1.05–16.78), steroid-use (adjusted OR 2.38, 95%CI 1.09–5.18), and a fibrotic HRCT pattern at baseline (adjusted OR 3.02, 95%CI 1.11–8.24) had a higher risk of PF-ILD.

Conclusion: PF-ILD is associated with increased mortality in patients with CTD-ILD. Patients with a fibrotic HRCT pattern at baseline, diabetes mellitus and steroid-use have a higher risk of developing PF-ILD.

Table 1. Baseline patient characteristics. Abbreviations: CTD, connective tissue disease; MCTD, mixed connective tissue disease; SLE, systemic lupus erythematosus; UCTD, undifferentiated connective tissue disease; IVIG, intravenous immunoglobulin; HSCT, Hematopoietic stem-cell transplantation; TNFi, tumor necrosis factor alfa inhibitor; UIP, usual interstitial pneumonia; NSIP, non-specific interstitial pneumonia; PPFE, pleuroparenchymal fibro-elastosis; OP, organising pneumonia; LIP, lymphocytic interstitial pneumonia.

Table 2. Predictors of mortality with multivariable adjustment in Cox regression. MMF, mycophenolate mofetil; HR, hazard ratio; 95%CI, 95% confidence interval; CRP, c-reactive protein; PF-ILD, progressive fibrosing interstitial lung diseases; AMA, Anti-mitochondrial antibody.

Figure 1. The Kaplan-Meier plots for survival analysis among predicted forced vital capacity (FVC), predicted single-breath diffusing capacity for carbon monoxide (DLCO), and progressive fibrosing interstitial lung diseases (PFILD).

Disclosures: Y. Chiu, None; J. Spierings, Boehringer Ingelheim, 5, Miltenyi, 5; P. de Jong, Sanifit, 2, Inozyme, 2; F. Mohamed Hoesein, None; J. Grutters, None; J. van Laar, Abbvie, 6, Arxx Tx, 6, Galapagos, 6, Gesyntha, 6, Leadiant, 6, Roche, 6, Boehringer Ingelheim, 5, Astra Zeneca, 5, MSD, 5, Roche, 5; M. Voortman, None.

To cite this abstract in AMA style:

Chiu Y, Spierings J, de Jong P, Mohamed Hoesein F, Grutters J, van Laar J, Voortman M. Predictors for Progressive Fibrosis in Patients with Connective Tissue Disease Associated Interstitial Lung Diseases [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/predictors-for-progressive-fibrosis-in-patients-with-connective-tissue-disease-associated-interstitial-lung-diseases/. Accessed .

ACR Meeting Abstracts - https://acrabstracts.org/abstract/predictors-for-progressive-fibrosis-in-patients-with-connective-tissue-disease-associated-interstitial-lung-diseases/