Usual interstitial pneumonia preceding collagen vascular disease: a retrospective case control study of patients initially diagnosed with idiopathic pulmonary fibrosis

Abstract

Background: The aim of this study was to evaluate the cumulative incidence and the predictive factors for collagen vascular disease (CVD) in patients initially diagnosed with idiopathic pulmonary fibrosis (IPF), and to examine the features of patients who then developed CVD.

Methods: This was a retrospective review of 111 consecutive patients with IPF diagnosed at our institution. None of the patients fulfilled any of the CVD criteria from the American College of Rheumatology (ACR) within 6 months or more after the diagnosis of IPF.

Results: Ten patients (9.0%) developed CVD during the follow-up period: four had rheumatoid arthritis (RA); four had microscopic polyangiitis (MPA); one had systemic sclerosis (SSc); and one had SSc and Sjogren's syndrome (SjS). The mean time until CVD diagnosis was 3.9 years. The cumulative incidences of CVD at 1, 5, and 10 years were 0.91%, 9.85%, and 15.5%, respectively. Patients who developed CVD were significantly younger, more likely to be women and had a better prognosis than those with IPF. Cox proportional hazards regression analysis showed that female sex and the presence of lymphoid aggregates with germinal centers were significantly associated with the occurrence of CVD in patients initially diagnosed with IPF.

Conclusions: CVD is an important underlying condition in IPF, and shows better prognosis. The possibility of the development of CVD should remain a consideration in the follow-up of IPF.

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.

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.

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.

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.