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BackgroundThe 2012 EULAR recommendations gave equal weight to intravenous cyclophosphamide (IVCY) and mycophenolate mofetil (MMF) as the induction therapy for class III/IV lupus nephritis (LN). However, there are no effective parameters that could inform the choice the induction therapy (IVCY or MMF) in individual cases.ObjectivesThis study examined the patient characteristics that determine the most appropriate treatment for LN: IVCY or MMF.MethodsWe retrospectively examined 29 patients with LN who received induction therapy with IVCY (n=16) or MMF (n=13) between January 1994 and December 2015. Their baseline characteristics and the complete response (CR) rate at week 24 were analysed. CR was defined as a urine protein:creatinine ratio <0.5 g/gCre with normal urine sediment.ResultsAt baseline, the time since diagnosis of systemic lupus erythematosus (SLE) was longer in the IVCY group than the MMF group (4.8±6.4 vs. 1.3±2.5 years, p=0.06) and the IVCY group had more frequent flares (1.9±2.4 vs. 0.7±1.1 times, p=0.08); however, the differences were not significant. Moreover, there was no difference in age, sex, complement levels, anti-dsDNA antibody titers, anti-Sm/RNP antibody positivity rates, proteinuria, or rate of abnormality in urine sediment at baseline between the two groups. CR was achieved at week 24 in 11/16 patients (69%) in the IVCY group and 9/13 patients (69%) in the MMF group. Considering the 20 patients who achieved CR at week 24, univariate analyses revealed that in addition to a longer time since diagnosis of SLE (4.5±6.6 vs. 1.0±1.7 years, p=0.12) and more frequent flares (1.9±2.8 vs. 0.6±1.0 times, p=0.16), the anti-RNP antibody positivity rate was higher (OR 8.15; p=0.07) in the IVCY group. Furthermore, the positivity rate of anti-RNP antibody differed significantly (OR 12.9; p=0.03) in the multivariate analysis.Abstract AB0525 – Table 1Univariate analyses of patients with CR at week 24 IVCY (n=11) MMF (n=9) p value age (years) 31.2±10.9 39.6±18.8 0.23 sex (male,%) 9.1 22.2 0.57, OR=2.71 time since diagnosis of SLE (years) 4.5±6.6 1.0±1.7 0.12 flares (times) 1.9±2.8 0.6±1.0 0.16 complement C3 level (mg/dL) 48.3±29.3 47.6±21.5 0.95 anti-dsDNA antibody (IU/mL) 161.2±187.4 128.8±154.2 0.68 anti-Sm antibody (%) 63.6 33.3 0.37, OR=3.27 anti-RNP antibody (%) 72.7 22.2 0.07, OR=8.15Abstract AB0525 – Table 2Multivariate analyses of patients with CR at week 24Model 1Model 2 OR (95% CI) p value OR (95% CI) p value age − − 0.9 (0.8–1.0) 0.15 sex (female) − − 1.2 (0.04–38.8) 0.92 time since diagnosis of SLE 1.1 (0.7–1.7) 0.77 1.0 (0.6–1.6) 0.91 flares 1.5 (0.5–4.2) 0.44 1.7 (0.6–4.4) 0.30 anti-RNP antibody 12.9 (1.3–132.3) 0.03* 31.8 (1.1–892.9) 0.04*Model 1 was adjusted by all of the characteristics which showed p<0.20 in the univariate analysis; model 2 was adjusted by age and sex in addition to model 1.dsDNA, double strand DNA; anti-Sm antibody, anti-smith antibody; anti-RNP antibody, anti-ribonucleoprotein antibody; OR, odds ratio; CI, confidence interval. (*p<0.05)ConclusionsAlthough IVCY and MMF ...

BackgroundPrevious studies indicate that malignancies in polymyositis (PM) or dermatomyositis (DM) patients are associated with high mortality. Hence, it becomes important clinically to identify patients who are at high risk of developing malignancy. However, previous studies to characterise such risk factors for malignancy in patients with PM and DM were small size or the findings ware not conclusive.ObjectivesThis study investigated factors predictive of malignancy and prognosis in patients with PM and DM.MethodsWe conducted a retrospective study of PM and DM patients who were inpatients at our hospital between January 1992 and September 2016. The diagnosis of PM or DM was made according to Bohan and Peter criteria. The collected data included gender, age at onset, laboratory test results at presentation, clinical features and complications in all progress. Past history of hypertension or diabetes mellitus was determined upon diagnosis of myositis. All patients in this study were followed from the baseline visit until loss of follow-up or death or otherwise until censor date 1 September 2017. Fisher exact test and Mann-Whitney U test were used for group comparisons. Univariate and multivariate analysis of the predictors of malignancy associated with PM or DM were performed by logistic regression to identify independent risk factors. Patient survival was analysed by using Kaplan-Meier curve and log-rank test.ResultsAmong 134 patients, 29 had cancer diagnosed between 2 years prior and 3 years following identification of PM or DM. Esophageal cancer (n=5, 14.7%) and lung cancer (n=5, 14.7%) were the most frequent. Univariate analysis showed that male sex (55.2% vs 23.8%, p=0.003), older age (64.6±11.3 vs 53.4±16.5, p<0.001), past history of diabetes mellitus (28.6% vs 2.9%, p<0.001), dysphagia (41.4% vs 17.3%, p=0.01) and absence of interstitial lung disease (37.9% vs 64.8%, p=0.01), arthralgia (17.2% vs 47.1%, p=0.005) and the Raynaud phenomenon (3.4% vs 23.1%, p=0.02) were associated with increased malignancy. Multivariate analysis showed that independent factors included male sex (OR=3.65, p=0.03), older age (OR=1.05, p=0.02), past history of diabetes mellitus (OR=10.4, p=0.005) and absence of interstitial lung disease (OR=0.25, p=0.03) (table 1). Survival was significantly lower in patients with malignancy than in patients without malignancy (p<0.001).Abstract AB0778 – Table 1Multivariate analysis of PM/DM with and without cancerVariablesAdjusted OR95% CIP value Male, n(%)3.651.17–11.300.03Onset age, years, mean±SD1.051.01–1.100.02Interstitial lung disease, n(%)0.250.08–0.840.03Diabetes mellitus10.42.00–54.30.005Arthralgia, n(%)0.550.15–2.040.37Dysphagia, n(%)2.820.82–9.710.10Raynaud phenomenon, n(%)0.250.03–2.290.22Abbreviation: OR, odds ratio; CI, confidence intervals.ConclusionsThis study is the first to associate a past history of diabetes mellitus with malignancy; 28.6% of PM or DM patients with malignancy had diabetes mellitus whereas 7.3 percent of cancer patients had diabetes mellitus.Reference[1] J.wang...

BackgroundThe 2012 Chapel Hill Consensus Conference (CHCC) definitions and the 2007 European Medicines Agency (EMA) algorithm have been commonly used for the diagnosis of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) as a conventional and reliable method. Recently the 2022 American College of Rheumatology (ACR)-European Alliance of Associations for Rheumatology (EULAR) classification criteria for AAV have been released. It is well-known that patients with granulomatosis with polyangiitis (GPA) are predominantly proteinase 3 (PR3)-ANCA-positive in the U.S. and Europe, but myeloperoxidase (MPO)-ANCA-positive patients with GPA are more prevalent in Japan. However, very few studies have been available on the evaluation of the applicability of the new criteria to an Asian population.ObjectivesThis study aimed to assess the applicability of the 2022 ACR/EULAR classification criteria to AAV patients from an Asian background.MethodsThis study included a total of 415 patients (mean age = 64.2 years, standard deviation [SD] = 16.3 years) diagnosed with AAV by the conventional method at three medical institutions in Japan between 2000 and 2022. Only Asians accounted for the whole population comprising 139, 128, and 148 patients (mean age = 65.2 years, SD = 15.7 years; mean age = 70.8 years, SD = 13.8 years; mean age = 56.1 years, SD = 15.8 years) diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA), GPA, and microscopic polyangiitis (MPA) by the conventional method, respectively. The applicability of the new criteria to AAV patients in Japan was assessed through the verification of the consistency with the conventional diagnoses. Additionally, phenotypes of organ involvement were analyzed in each reclassified group from the conventionally-diagnosed GPA population.ResultsThe patients diagnosed with EGPA and MPA by the conventional method were significantly more likely to be reclassified into the same AAV subphenotype (138 out of 139 patients [99.3%] and 145 out of 148 patients [98.0%], respectively). On the other hand, the patients diagnosed with GPA by the conventional method were less likely to be reclassified into the same AAV subphenotype (91 out of 128 patients [71.1%]). In the conventionally-diagnosed GPA population, 71 patients were reclassified as GPA, not as MPA, 20 were reclassified not only as GPA but also as MPA, and 36 were reclassified not as GPA but rather as MPA. In the group reclassified only as GPA, there were 59 patients with sinusitis, including ten patients with orbital mass lesions. In the group reclassified both as GPA and as MPA, there were 20 patients with severe organ involvement, including 15 patients with pauci-immune glomerulonephritis (PIGN).ConclusionThe new criteria gave more weight to ANCA serology than histology and surrogate markers compared with the conventional method, which kept diagnostic consistency in the conventionally-diagnosed EGPA and MPA populations. At the same time, the new criteria occasionally led to a big diagnostic contradiction in the conventionally-diagnosed GPA population from an Asian background. In the conventionally-diagnosed GPA population, the new criteria were likely to reclassify MPO-ANCA-positive patients as MPA. However, it is noteworthy that the new criteria were still beneficial in extracting GPA patients with recurrent or severe organ involvement, including orbital masses and PIGN.Figure.Reclassification of the conventionally-diagnosed AAV patients according to the 2022 ACR/EULAR classification criteria for AAV, including the distribution of ANCA serotypes. AAV, antineutrophil cytoplasmic antibody-associated vasculitis; ANCA, antineutrophil cytoplasmic antibody; MPO, myeloperoxidase; PR3, proteinase 3; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.