Variables associated with mortality in 103 patients with anti-neutrophil cytoplasmic antibodies associated vasculitis Background: Cumulative survival in patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (VAA) is 88 and 78% at 1 and 5 years, respectively. Despite this, mortality continues to be 2.7 times higher than the general population. Differences in the clinical profile of VAA in different ethnicities have been observed. Aim: To identify factors at the time of diagnosis, associated with mortality at one year of follow-up and to describe the clinical characteristics of these patients. Material and Methods: We identified in local databases and reviewed clinical records of patients with VAA with at least one year of follow up in a clinical hospital. Demographic and laboratory parameters and clinical activity scores were analyzed. Results: Of 103 patients with VAA identified, 65 met the inclusion criteria and were analyzed. Their age ranged from 45 to 63 years and 56% were women. Thirty-five patients (54%) were diagnosed as granulomatosis with Polyangiitis (GPA) and 30 patients (46%) with Microscopic Polyangiitis (MPA). The frequency of renal disease was 53% and pulmonary involvement occurred in 72%. At one year of follow-up 11 patients died resulting in a mortality of 17%. Seven patients died within three months after diagnosis. MPO ANCA were more common than PR3 ANCA. In the multivariate analysis, the presence of ophthalmological involvement, lung kidney syndrome and a Five Factor Score (FFS) of 1 or more were independent factors associated with mortality at one year. Conclusions: In these patients, pulmonary manifestations predominate. Lung kidney syndrome, ophthalmological involvement and a FFS score ≥ 1 were associated with mortality.
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
BackgroundAnca Associated Vasculitis (AAV), are a group of necrotizing primary vascultitis, whith multisistemic manifestation, of unknown etiology. The variants are: Microscopic Polyangiitis (MPA), Granulomatosis with polyangiitis (GPA), Granulomatosis whith Polyangiitis and Eosinophilia (GPE) and AAV limited to one organ. Until now, there are no diagnostic criteria for AAV. Therefore definitions, as Chapell Hill consensus Conference Nomenclature, classification criteria and the physician judgement are used for diagnosis. Currently the DCVAS (Diagnosis and Classification Criteria in Vasculitis) proyect is developing diagnostic criteria for AAV, using data-driven methods. The preliminary DCVAS classification criteria for granulomatosis whith polyangiitis has been recently realesead.ObjectivesTo evaluate and compare the acuraccy of ACR/EULAR 2017 provisional Classification Criteria for GPA whith the ACR 1990 Classification Criteria In Chilean patients with AAV.MethodsAll adult patients (>18 yo) with diagnoses of AAV according to their rheumatologist judgment, from 2000–2016 at the University of Chile, Clinical Hospital (UCCH), were included. Clinical variables of interest were extracted form medical chart and AAV database, wich is kept for these patients at the Rheumatology Section of UCCH. Based on that data, the Classification criteria ACR 1990 and 2017preliminary ACR/EULAR (DCVAS) classification criteria for GPA were applied to each individual. Sensibility, especifity, Likehood ratio (LR +/-), predictive values (PPV/NPV) and accuracy were calculated for both sets of Criteria as compared to Clinical diagnosisResults93 patient were included in the study. 59 patients with GPA, 33 with MPA and 1 with GPE (Patients characteristics are described in Table 1). From the 59 patients with GPA according to clinical judgment, 34 fullfilled the ACR 1990 criteria (57,63%) whereas 51 (86,44%) fullfilled the ACR/EULAR 2007 criteria. From the 33 patients diagnosed as having MPA 9 (27,27%) and and 3 (9.09%) fulfilled classification criteria for GPA according to ACR 1990 criteria and 2017 ACR/EULAR preliminary criteria, respectively.The patient with GPE did no classify as GPA by either set of criteria.The values for Sensibility, Espicifity, LR+ LR-, PPV and NPV in our population for both Criteria sets, are described in table 2.ConclusionsIn our population, provisional 2017 ACR/EULAR criteria for classificatiόn of GPA have better acurracy than ACR 1990 classification criteria.References -Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, et al.The American College of Rheumatology 1990 criteria for the classification of Wegener's Granulomatosis. Arthritis Rheum 1990;33:1101–7.-Luqmani, R, Merkel, P. New Clasification Criteria for ANCA-Associated Vasculitis: Implication for Clinical Practice. Lecture, 2016 ACR/ARHP Annual Metting. Disclosure of InterestNone declared
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