Kaplan-Meier survival curves of all-cause mortality.

<p>Overall cumulative 5-year mortality was 24.4%. (A) Patients with ScAb-ILD (dotted line) had worse survival than those with SSc-ILD (dashed line) (p = 0.011). (B) In patients with ScAb-ILD, those with extensive disease (dashed line) had worse survival than those with limited disease (solid line) (p = 0.015). (C) In patients with SSc-ILD, those with KL-6 ≥ 1000 U/mL (dashed line) had worse survival than those with KL-6 < 1000 U/mL (solid line) (p = 0.049). (D) The survival curves for patients with each type of autoantibody were not significantly different (p = 0.905 for comparison between anti-scleroderma-70 and anti-U1 RNP antibody, p = 0.089 for comparison between anti-scleroderma-70 and anti-centromere antibody, and p = 0.137 for comparison between anti-U1 RNP and anti-centromere antibody).</p

Analysis of factors associated with mortality.

<p>Analysis of factors associated with mortality.</p

Baseline characteristics at the time of diagnosis as interstitial lung disease.

<p>Baseline characteristics at the time of diagnosis as interstitial lung disease.</p

Examples of pathological scoring.

<p>(A-B) Typical imaging in each grade of organizing pneumonia (intra-alveolar polyps) as found in patients with ScAb-ILD (hematoxylin-eosin stain) ([A]: grade 1 and [B]: grade 3). (C-D) Vascular intimal or medial thickening as found in patients SSc-ILD (Elastica van Gieson stain) ([C]: grade 1 and [D] grade 3].</p

Comparison of HRCT and pathological findings between SSc-ILD and ScAb-ILD.

<p>Comparison of HRCT and pathological findings between SSc-ILD and ScAb-ILD.</p

Changes in forced vital capacity (FVC) during follow-up.

<p>(A) SSc-ILD (B) ScAb-ILD (C) Regression lines were calculated by solving the linear mixed-effects model. Baseline FVC (intercept) with ScAb-ILD (mean, 2.574 L [95% CI: 2.313–2.835]) was significantly higher than that with SSc-ILD (mean, 2.191 L [95% CI: 1.974–2.407]) (p = 0.027). The declining slopes of FVC between both groups were not significantly different (SSc-ILD: mean, -0.03979 L year^-1 [95% CI: -0.05449 to -0.02509]; ScAb-ILD: mean, -0.03740 L year^-1 [95% CI: -0.06446 to -0.01034] (p = 0.878).</p

HRCT scans of cyst formation.

<p>(A-B) HRCT scan demonstrates cyst formation (arrowheads) with pulmonary fibrosis, traction bronchiectasis, and architectural distortion in two patients with SSc-ILD. There is no continuity between the cysts and traction bronchiectasis. (C) HRCT scan shows cysts (arrowheads) with opacity separated from the pleura in a patient with MCTD-ILD.</p