BackgroundTocilizumab (TCZ) has shown to be effective for large vessel vasculitis including giant cell arteritis (GCA) and Takayasu arteritis (TAK) (1-5). However, LVV-GCA and TAK show different demographic and clinical features that may influence on TCZ therapeutic response.ObjectivesTo compare the effectiveness of TCZ in patients with LVV-GCA and patients with TAK.MethodsObservational multicenter study of patients with LVV-GCA and TAK who received TCZ. Outcome variables were: a) proportion of patients who achieved complete clinical improvement along with normalization of laboratory markers (CRP ≤0.5mg/dL and/or ESR ≤ 20 mm/1st hour) at 12 months b) complete improvement in imaging techniques. A comparative study between patients with LVV-GCA and TAK was performed.ResultsWe evaluated 70 LVV-GCA and 57 TAK patients who received TCZ. Main clinical and demographic characteristic are described in Table 1. Patients with TAK were younger, had longer disease duration, had received more commonly previous biologic therapy and were receiving higher doses of prednisone at baseline. TCZ intravenous administration was more common in TAK patients (80.7% vs 48.6%; p<0.01). Follow-up time after TCZ onset was similar in both groups. At 12 months, about 75% of patients achieved complete clinical improvement and ESR/CRP normalization in both groups. A follow-up imaging technique was performed in 37 LVV-GCA patients after a mean time of 12.9±6.0 months and 38 TAK patients after 9.5±5.0 months. Complete improvement in imaging techniques was only observed in 18.9% and 21.1% of patients with LVV-GCA and TAK, respectively (Figure 1).Table 1.LVV-GCA (n=70)TAK (n=57)pGeneral featuresAge (years), mean ± SD67.2 ± 10.540.5 ± 16.3< 0.01Sex (female), n (%)51 (72.9)49 (86)0.07Disease evolution before TCZ onset (months), median [IQR]5 [2-15]12 [3-37]<0.01Baseline laboratory parametersESR (mm/1st hour), median [IQR]32 [12.5-54.7]31 [10-52]0.82CRP (mg/dL), median [IQR]1.4 [0.5-2.4]1.4 [0.5-3.5]0.41Baseline prednisone dose (mg/day), median [IQR]15 [10-20]30 [15-50]< 0.01Previous therapyConventional DMARDs, n(%)45 (64.3)44(77.2)0.51Biologic therapy, n (%)0(0)12 (21.1)<0.01TCZ therapyIntravenous, n (%)34 (48.6)46 (80.7)< 0.01Combined with MTX, n(%)24 (34.3)24 (42.1)0.37Follow-up time after TCZ onset, median [IQR]20 [10-36]18 [7-41]0.73Complete clinical improvement and ESR/CRP normalization at 12 months, n/N (%)35/47 (74.4)30/39 (76.9)0.79Complete improvement in imaging techniques, n/N(%)7/37 (18.9)8/38 (21.1)0.85CRP: C-reactive protein; DMARDs: Disease-modifying anti-rheumatic drugs ESR: erythrocyte sedimentation rate; GCA: giant cell arteritis; IQR: interquartile range; LVV: large vessel; MTX: methotrexate; n: Number of patients; N: total number of patients: TCZ: tocilizumab; TAK:takayasuFigure 1.ConclusionThe effectiveness of TCZ was similar in patients with LVV-GCA and TAK, despite a more refractory disease in TAK patients. A discordance between clinical and imaging activity improvement was observed in both LVV-GCA and TAK, as reported in previous studies (3).References[1]Calderón-Goercke M, et al. Semin Arthritis Rheum 2019; 49:126-35. https://doi.org/10.1016/j.semarthrit.2019.01.003[2]Prieto-Peña D et al. Ther Adv Musculoskelet Dis. 2021;13:175. PMID: 34211589.[3]Prieto Peña D et al. Clin Exp Rheumatol. 2021;39 Suppl 129:69-75. PMID: 33253103.[4]González-Gay MA, et al. Expert Opin Biol Ther. 2019;19:65-72. doi: 10.1080/14712598.2019.1556256.[5]Prieto-Peña D, et al. Semin Arthritis Rheum. 2019;48(4):720-727. doi: 10.1016/j.semarthrit.2018.05.007Disclosure of InterestsNone declared
BackgroundAnti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) immune-mediated necrotizing myopathy (IMNM) is an entity of growing interest. However, data on epidemiology and clinical spectrum are still scarce and there is a need for the identification of its potential risk factors.ObjectivesTo characterize the demographic, genetic, clinical, and serological features of patients with anti-HMGCR IMNM in a region of northern Spain.MethodsStudy of all patients diagnosed with anti-HMGCR IMNM during a 5-year period at a reference hospital in Northern Spain. Besides clinical and laboratory data, we analyzed the genetic influence of HLA genes and the rs4149056 (c.521T>C) single nucleotide polymorphism (SNP) in the SLCO1B1 gene.Results8 patients (5 women, 3 men) with a mean ± SD age of 64.9±7.3 years, fulfilled the criteria for anti-HMGCR IMNM. The incidence rate was 0.6 per 100.000 person-years and the prevalence 3 per 100.000 population. All patients had dyslipidemia and had been exposed to statins. Seven of the 8 of cases complained of myalgia. All of them had predominant lower limb proximal and symmetric muscle weakness that was severe in 2 of them. None of the patients had extra-muscular involvement. No evidence of malignancy was found. All patients had elevated serum CK levels with a median [IQR] of 4488 [2538-9194] IU/L. Serum 25-hydroxy vitamin D levels were decreased in all patients in whom it was determined. The 3 patients with a previous diagnosis of hypothyroidism had abnormal levels of TSH at the time of diagnosis. All patients experienced improvement with different schemes of immunosuppressive therapy. Noteworthy, 7 of 8 patients carried the HLA-DRB1*11 allele. The frequency of the rs4149056 C allele in the SLCO1B1 gene (12.5%) was similar to that of the general population.ConclusionIn northern Spain, the IMNM anti-HMGCR preferentially affects people over 50 years of age who are carriers of the HLA-DRB1*11 allele and take statins. Both low vitamin D levels and hypothyroidism may play a potential predisposing role in the development of this diseaseTable 1.PatientAge/SexHLA DRB1*11rs4149056 genotypeMRC at the weakest muscle group*DysphagiaCK (IU/L) at diagnosisAnti-HMGCR titer (CU)Induction therapy*Maintenance therapyClinical improvement**CK (IU/L) at last follow-up visit156/MYesTT2No8963277.8GC. IVIG.MTXGC. IVIG. MTX. RTXMarked134269/FYesTT0Yes9271235.9GC iv bolus. IVIG.GC. MTX. RTX.Marked890364/FYesTT3No4000242.6IVIG.IVIG.RTXMarked1284479/MYesTT4No4977145.6GC. IVIG.GC. IVIG.Complete92562/FNoTT3No2116210.0GCGC.MTX.Marked236657/FYesTC4No2294259.3IGIVIGIVComplete235768/FYesTT3No3273236.0GC. IGIV. AZA.GC. AZAComplete249864/MYesTC4Yes11000179.0GC iv bolus. AZA.GC. AZAComplete161AZA: azathioprine; CK: creatinine kinase; CU: chemiluminescence units; F: female; GC: glucocorticoids; IVIG: intravenous immunoglobulins; M: male; MRC: medical research council scale; MTX: methotrexate; RTX: rituximab; ** Induction therapy initiated within 3 months of diagnosis. **Clinical improvement: no improvement (no improvement in MRC grade), mild improvement (improvement of MRC grade but still requiring assistance for activities of daily living), marked improvement (persistence of mild weakness without functional limitation), and complete improvement (return to baseline with no symptoms or signs of weakness).Disclosure of InterestsNone declared
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