Guida, G

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Institutional Research Information System University of Turin

Results: 273 patients with AAV (131 women, 48%) and 2717 controlsdifferences in the genetic characteristics of MPO- and PR3-ANCAassociated diseases might affect the consequences of triggering theimmune system by different microorganisms.Disclosures: The study was supported by Lund University and RegionSkane.ABSTRACT 159 TABLE 1: Odds ratio of developing AAV after exposure to differentinfections diagnosed  6 months prior to the onset of AAVTypes of infections All OR (95% CI) MPO-ANCAOR (95% CI)PR3-ANCAOR (95% CI)All infections 1.25 (0.93-1.68) 1.70 (1.08-2.68) 0.79 (0.52-1-21)URTI 1.43 (1.01-2.01) 1.81 (1.08-3.03) 0.99 (0.59-1.66)Pneumonia/Influenza 1.70 (1.02-2.24) 2.13 (1.07-4.24) 1.42 (0.56-3.07)LRTI 1.13 (0.64-1.99) 1.02 (0.44-2.37) 1.30 (0.57-2.99)Skin-infections 0.41 (0.19-0.86) 0.32 (0.96-1.06) 0.58 (0.22-0.51)ENT infections 1.54 (0.93-2.54) 2.71 (1.31-5.65) 0.59 (0.20-1.70)UTI 1.15 (0.60 -2.10) 0.70 (0.23-2.09) 1.20 (0.51-2.84)URTI: Upper respiratory tract infections, LRTI: lower respiratory infections, ENT:ear-nose and throat, UTI: urinary tract infectionOF REMISSIONAND SIDEA STUDY PROTOCOLMazzetti3,Secore4, Gordon Guyatt1,Walsh3 on behalf ofand Vasculitis Foundation2Vasculitis FoundationHamilton, ON5University of(AAV) is a multi-systemremitting course. As neware developed and tested inminimally important differ-importance. With patientpartners, we designed a survey instrument called the Vasculitis:Effects of Remission maintenance Therapies on relapse and Sideeffects on patient preferences (VERITAS) to assess the MID from theperspective of patients with vasculitis.Methods: An on-line survey was developed with patient partners andpiloted in a single centre for use with patients with vasculitis throughthe online Vasculitis Patient-Powered Research Network (VPPRN). Thesurvey instrument assesses patients’ acceptance of therapy under arange of baseline risks of relapse, effect sizes of treatment on risk ofrelapse, and risks of serious adverse events. The survey was piloted ina Rheumatology clinic in Hamilton and after three successful iterationswithout any patient-driven changes the survey version was determinedto be final. Survey responses will be summarized as a distribution ofthe proportions of patients that will accept each level of risk reductionfor each level of absolute risk reduction and risk of serious adverseevents. Participants will be randomized to either an unnamed therapyor to naming the therapy prednisone, a treatment with which mostpatients are familiar to further determine whether known treatmentsalter perceptions of acceptability. The effects of naming of therapy aswell as the effects of patient demographics on the MID will beassessed with mixed effects models.Conclusion: Here we report the study protocol for VERITAS, aninstrument designed to assess the distribution of preferences ofpatients with vasculitis for therapies that reduce the risk of relapse.The distribution of preferences will be useful in determining patient-important MID and sample size estimates for future clinical trials.Disclosures: This work was supported by the Vasculitis ClinicalResearch Consortium and the Vasculitis Foundation.161. AUGMENTED IL-6 IMPLIES UN-SEVERE VASCULARA CROSS-SECTIONAL1 and Lindi Jiang1Shanghai, Chinais the most serious complicationoverall stenosis patients remainto discover the VS in the firstis closely associated with TAmarkers are correlated withconducted in the Zhongshan hospitalCohort of Takayasu’s arteritis (ECTA)to the hospital for the first time wererecruited for further analysis (n¼450), and were divided into subgroupsbased on the extent of stenosis (without, mild, moderate, severe). Theoptimal TA patients, defined as naı̈ve patients who were diagnosed andtreated at Zhongshan hospital, were extracted as a subgroup. Weevaluated the symptoms, vascular imaging, serum biomarkers includingIL-6, and drugs. The logistic and ordinal regression models were used. ROCcurve was performed to observe the effect indicators.Results: Among 446 TA patients with vascular imaging, 87.8% werediagnosed with VS. After adjusting age, sex, and course, IL-6 wasnegatively independently associated with both the presence and theextent of VS in both TA patients and optimal TA patients (odds ratio[x5 pg/ml]: 0.84 to 0.87, P<0.05 for all). The cut-off point for IL-6 todiagnose the ‘‘without þ mild’’ VS was 4.45 pg/ml, with the sensitivityof 0.653 and specificity 0.577 respectively.Conclusion: Most TA patients have VS. The level of IL-6 is negativelyassociated with the VS independently. The elevated IL-6 indicates un-severe VS in TA, which deserves effective intervention timely.Disclosures: The investigation was supported by the National NaturalScience Foundation of China [NSFC 81771730 and 81601398]; and theAnimal Research Project of Shanghai Science and TechnologyCommission [grant number 17140902000].162. CLUSTER ANALYSIS TO EXPLORE CLINICALSUBCLASSIFICATION OF EOSINOPHILIC GRANULOMATOSISWITH POLYANGIITIS (CHURG–STRAUSS)Emma Rubenstein1, Carla Maldini1, Augusto Vaglio2, Paolo Bottero3,Phillip Bremer4, Giacomo Emmi5, Paolo Fraticelli6, Andrea Gioffredi2,Gina Gregorini7, Julian Grosskreutz8, Giuseppe Guida9,Bernhard Hellmich10, Claus Kroegel8, Federica Maritati11,Alberto Pesci12, Matthieu Resche-Rigon1, David Saadoun13,Renato Sinico11, Barbara Sokolowska14, Wojciech Szczeklik14,Jochen Zwerina15, Frank Moosig16, Thomas Neumann17 andAlfred Mahr11Saint Louis Hospital Paris, France, 2University Hospital of Parma,Italy, 3Ospedale di Magenta Milano, Italy, 4Clinical Center BadBramstedt Bad Bramstedt, Germany, 5University of Firenze,Florence, Italy, 6Ospedali Riuniti Ancona, Italy, 7Spedali Civili BresciaBrescia, Italy, 8University Hospital of Jena Jena, Germany, 9Birago diVische Hospital Torino, Italy, 10Vasculitis Center Tübingen-KirchheimKirchheim unter Teck, Germany, 11University of Parma Parma, Italy,12University of Milano Bicocca Milan, Italy, 13Pitié-SalpêtrièreUniversity Hospital Paris, France, 14Jagiellonian University MedicalCollege Krakow, Poland, 15Hanusch Hospital Vienna, Austria,16Rheumazentrum Schleswig- Holstein Mitte Neumünster, Germany,17Kantonsspital St. Gallen St Gallen, SwitzerlandBackground: Previous studies of eosinophilic granulomatosis withpolyangiitis (Churg–Strauss, EGPA) suggested that EGPA may involveclinically distinct phenotypes. In particular, clinical manifestationsmight be determined by the presence or absence of antineutrophilcytoplasmic antibodies (ANCAs) and reflect more prominent under-lying vasculitic than eosinophilic disease processes. This studyexplored whether EGPA involves several subcategories by usinghierarchical cluster analysis.Methods: This study used clinical data for patients diagnosed with EGPAin 16 centers in Austria, France, Germany, Italy and Poland collected witha standardized case report form. The analyses pertained to patientsreported to have asthma and who fulfilled the Lanham and AmericanCollege of Rheumatology criteria, with ANCA positivity (by immunofluor-escence or ELISA test) as an additional item. We used a multiplecorrespondence analysis followed by hierarchical cluster analysis with theWard method. The cluster model included 10 clinical variables assessedat diagnosis (arthromuscular, mucocutaneous, ophthalmological, ENT,cardiovascular, pulmonary, gastrointestinal, renal, central or peripheralneurological involvement); a second model added ANCA positivity as anii70 EPIDEMIOLOGYDownloaded from https://academic.oup.com/rheumatology/article/58/Supplement_2/kez059.039/5421994 by guest on 07 February 2023input variable. Clinical relevance of the generated clusters was assessedby describing each cluster’s clinical characteristics.Results: We had data for 482 patients (46.3% males; mean age atdiagnosis: 51 years [SD 14.9]) with a diagnosis mostly from 1990 to2015. The cluster analysis generated 4 clusters of 34, 371, 39 and 38patients. No distinctive cluster characteristic could be identified andwe found no mutually exclusive subgroups, even after adding ANCApositivity to the model. ANCAs were detected in 37.1% of patients andwere mostly P-ANCA (85.2%) or anti-myeloperoxidase (87.5%)according to the technique used. As compared with ANCA-negativeEGPA patients, those with ANCA-positive EGPA were more often male(P¼0.01), significantly younger (P¼0.002), with more renal(P<0.0001) and peripheral neurological involvement (P< 0.0001) andtissue findings of vasculitis (P¼0.01), and fewer cardiovascular signs(P<0.0001) and tissue findings of eosinophilic infiltration (P<0.0001).Conclusion: Although reinforcing the known associations of ANCAstatus with clinical manifestations, our data do not provide evidencefor clearly distinct subclasses within EGPA. EGPA may be a pheno-typic continuum rather than a phenotypic dichotomy, with entangledrather than distinct mechanistic pathways.Disclosures: None163. A RETROSPECTIVE COHORT STUDY TO INVESTIGATESEX-SPECIFIC DIFFERENCES IN ANCA-ASSOCIATEDperformed within the Irish Clinical Academic Training (ICAT)Programme, supported by the Wellcome Trust and the HealthResearch Board (Grant Number 203930/B/16/Z), the Health ServiceNational Doctors Training and Planning and the Health andSocial Care, Research and Development Division, Northern Ireland.164. INCIDENCE AND SEASONAL VARIATION OF BIOPSY-Pavlos1LundMethoof 1,undeidentifiperiodof theseasoEPIDEMIOLOGY ii71Downloaded from https://academic.oup.com/rheumatology/article/58/Supplement_2/kez059.039/5421994 by guest on 07 February 2023

CLUSTER ANALYSIS TO EXPLORE CLINICAL SUBCLASSIFICATION OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS)

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CLUSTER ANALYSIS TO EXPLORE CLINICAL SUBCLASSIFICATION OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS)

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