AB0691 Instertitial lung disease and mycroscopic polyangiitis in chilean patients

Abstract

BackgroundMicroscopic Polyangiitis (MPA) is an ANCA associated vasculitis (AAV), associated with p-ANCA (perinuclear) fluorescence pattern and anti- myeloperoxidase (MPO) specificity. Most frequently involved organs are kidney (80%–100%), peripheral nervous system and skin (30%). There is Pulmonary involvement in 25%–35% of patients, being alveolar haemorrhage frequently described. Interstitial lung disease (ILD) has also been recognised.ObjectivesThe aim of our study is to report the characteristics of MPA Chilean patients with ILD and to compare it with other series.MethodsRetrospective study. Patient diagnosed between 2007 and 2016 at the Hospital Clĺnico Universidad de Chile, with ILD, defined as interstitial lung disease on CT scan with Usual Interstitial Pneumonia (UIP) or Non Specific Interstitial Pneumonia (NSIP) pattern, and MPA were included. Demographic, clinical, laboratory and mortality data were plotted. Data from other series were compared with our results. Other causes that could explain the pulmonary involvement were excluded.ResultsFrom 94 patients with AVV, 36,1% were MPA, being 16 patients with ILD. All were Hispanic, median age 65.3 years,32–84 female 62,5% (table 1). Common manifestations were constitutional symptoms (100%), weight loss (68,7%) and fever (68,7%). All patients had anaemia, high ESR (mean 84 mm/hr. range 33–120) and CRP (8–22 times above upper normal limit). All patients were ANCA-p and MPO positive. In 10 cases ILD was diagnosed concomitantly with MPA and in 6 was 0.5 to 15 years before. 4 patients developed pulmonary haemorrhage. Images patterns were 10 UIP and 5 NSIP. All patients received corticosteroid as induction therapy, 15 also received cyclophosphamide. One patient plasmapheresis, and one received Rituximab after a relapsed. Azathioprine was used as Maintenance therapy. Four patients died during follow-up. table 2 shows data from other worldwide region compared with our data.Abstract AB0691 – Table 1Caracteristic of chilean patient with ILD an MPAAbstract AB0691 – Table 2Caracteristic of patient with ild and MPA worldwideConclusionsAmong chilean patients there are more females, have a more NSIP pattern, and less mortality that other worldwide series.References[1] Alba MA, et al. Autoimmun Rev. 2017Jul;16(7):722–729.[2] Flores-Suarez LF, et al. Clin Rheumatol2015;34:1653–4.[3] Fernandez Casares M, et al. Clin Rheumatol2015;34: 1273–7.[4] Huang H, et al. BMC Pulm Med2014;14:8.[5] Kagiyama N, et al. BMJ Open Respir Res. 2015Jan 9;2(1):e000058.[6] Eschun GM, et al. Chest2003;123:297–301.[7] Nada AK, et al. Mayo Clin Proc1990;65:847–56.[8] Comarmond C, et al. Medicine (Baltimore)2014; 93:340–9.[9] Hervier B, et al. Ann Rheum Dis2009;68:404–7.Disclosure of InterestNone declared

Myeloperoxidase and associated lung disease: Review of the latest developments

“…Despite seemingly having the same phenotype, the relevance of identification of MPO‐ANCA is for its potentially significant clinical implications. MPO‐ANCA can pre‐date AAV by up to 5 years (range from 5 to120 months), and ILD can pre‐date diagnosis of an ANCA vasculitis by up to 14 years, 40 so ongoing clinical vigilance is required if there is a suspicion 4,41 . A recent study 42 retrospectively performed autoimmune antibody testing on a cohort of patients diagnosed with ILD, and MPO‐ANCA was identified in 2.5% of patients, suggesting a small population of patients who may be missed if ANCA testing is not performed.…”