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Figure 1.

The algorithm for IPF diagnosis among enrolled patients.

All 111 patients were diagnosed with IPF (definite, n = 104; probable, n = 2; possible, n = 5) according to the algorithm for IPF diagnosis. HRCT, high-resolution computed tomography; SLB, surgical lung biopsy; IPF, idiopathic pulmonary fibrosis; UIP, usual interstitial pneumonia.

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Table 1.

Patient characteristics.

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Table 2.

Characteristics of the patients who had developed CVD at the time of CVD diagnosis.

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Table 3.

Systemic findings and autoantibodies in the patients who developed CVD and in those with IPF at the time of initial diagnosis.

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Table 4.

HRCT and pathological findings in the patients who developed CVD and in those with IPF at the time of initial diagnosis.

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Figure 2.

Pathological findings in the patients who developed CVD (Cases 1, 2, 5, 10).

All patients showed a pathological UIP pattern at the time of the initial diagnosis. Lymphoid aggregates with germinal centers were prominent in the patients who developed CVD.

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Table 5.

Treatment and outcome throughout the course of the patients who developed CVD and those with IPF.

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Figure 3.

Survival curves for the patients who developed CVD and those with IPF.

Patients who developed CVD had a significantly better survival rate than those with IPF (log-rank, P = 0.0433). IPF, idiopathic pulmonary fibrosis; CVD-IP, interstitial pneumonia associated with collagen vascular diseaseG.

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Figure 4.

Cumulative incidence of CVD among patients with IPF.

Cumulative incidences of CVD at 1, 5, and 10 years were 0.91%, 9.85%, and 15.5%, respectively.

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Table 6.

Predictive factors for the development of CVD in patients with IPF, according to univariate Cox proportional hazards analysis.

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