Poster Session C
Vasculitis
Elif Ediboglu, M.D.
Mayo Clinic
Rochester, Minnesota, United States
No financial relationships with ineligible companies to disclose
Fifteen patients with DAH caused by MPA had pre-existing ILD with presenting characteristics and manifestations are shown in table 1. Median (IQR) time between ILD and DAH diagnosis was 9 (3-31) months. Ten patients (67%) had a UIP pattern on high-resolution CT scans of the chest, 5 had a non-UIP pattern. All patients had pulmonary function tests during their evaluation for ILD with evidence of a restrictive pattern. Chest imaging studies were performed in all patients, in addition, 12 patients had bronchoscopy to diagnose DAH. Overall survival was 80% at 1 year and 53% at 5 years in the MPA-ILD-DAH group. We compared the overall mortality between the 3 groups: although the patients in the MPA-ILD-DAH group had a higher mortality than matched patients in the MPA-ILD group, the difference of mortality of patients in the 3 groups was not statistically significant (p= 0.17, by the log-rank test) (Figure 1).
Conclusion: DAH may be a risk factor for hospital mortality and overall mortality in patients with MPA and ILD. In contrast, in patients with DAH, having a history of pre-existing ILD does not seem to affect mortality.
Disease related features and outcomes of patients with MPA -ILD and DAH, MPA-ILD, MPA-DAH
Survival assessed using the log-rank test as univariate analysis. Although there were significant differences among the three patient groups, patients in the MPA-ILD-DAH and the MPA-DAH groups did not have a significantly different mortality (p=0.17).
High-resolution computed tomography (HRCT) images of a patient with MPA-ILD-DAH. HRCT images at baseline with MPA-associated ILD are shown in axial (Figure 1A) and coronal (Figure 1C) views. 1 year later, this patient presented with acute onset dyspnea and HRCT images at that time demonstrated new peribronchovascular nodularity and ground glass opacities after which he underwent bronchoscopy with confirmed a diagnosis of DAH on axial (Figure 1B) and coronal (Figure 1D) views.
E. Ediboglu: None; S. Falde: None; M. Baqir: None; R. Cartin-Ceba: None; U. Specks: Amgen, 2, 5, Argenx, 2, AstraZeneca, 1, 2, 5, Boehringer-Ingelheim, 1, 2, Bristol-Myers Squibb(BMS), 5, CSL Vifor, 1, Genentech, 5, GlaxoSmithKline(GSK), 5, Northstar Medical Radioisotopes, 5, Novartis, 5, NS Pharma, 5.