Differences between ANCA positive and negative lung fibrosis cases without vasculitis
Bildiriler > Özet Bildiri
Yazarlar: Nesrin MOĞULKOÇ BISHOP, Mehmet Nedim Tas, Yasemin KABASAKAL, Paul William Bishop, Simona Littnerová, Karel Hejduk, Martina Vašáková
Idiopathic interstitial pneumonias, , , Uluslararası, Özet Bildiri, ss PA2242-PA2242
DOI: 10.1183/13993003.congress-2019.pa2242
Özet
Aim: Patients with interstitial lung disease of UIP pattern who are ANCA-positive (Anti-neutrophil cytoplasmic antibody, MPO subtype) without signs of vasculitis are not well characterised. We wished to compare this subgroup with the generality of our patients with IPF. Method: The patients were from Ege Hospital and submitted to the EMPIRE Registry, which performed the statistical analysis. We compared 23 patients with UIP who were ANCA-positive at presentation but without signs of vasculitis against 497 patients with IPF who were ANCA-negative, comparing demographics, smoking history, comorbidities, HRCT appearances, anti-fibrotics treatment and survival. Results: The ANCA-positive patients were marginally older (median 70 vs. 67 years, p = 0.03). They were less likely to be diabetic (4.8% vs. 24%, p=0.02) and much less likely to have pulmonary hypertension (0% vs 18%, p = 0.004) but more likely to have COPD (19% vs 5%, p=0.024). They were much more likely to have an HRCT pattern of definite UIP (86% vs 58%, p = 0.007). There were no significant differences in lung function tests. The proportions of patients treated with pirfenidone and nintedanib were no different (48% vs 57%) and the survival outcome did not differ significantly (38% vs 34% at 72 months, p = 0.63). Conclusions: ANCA-positive ILD patients with UIP pattern have some distinct features but show a similar survival curve to the ANCA-negative patients with IPF.