Detection of Myositis Autoantibodies by Multi-Analytic Immunoassays in a Large Multicenter Cohort of Patients with Definite Idiopathic Inflammatory Myopathies

Objective Our aim was to assess efficacy and safety of Rituximab (RTX) in patients with refractory Idiopathic inflammatory myopathies (IIM) from a monocentric cohort. Thereafter, we evaluated the efficacy of a low-dose RTX regimen as a remission-maintenance therapy. Methods We retrospectively evaluated a cohort of patients affected with IIM treated with RTX. All patients were refractory to glucocorticoids (GC) and at least one immunosuppressant. Two infusions of 1 g two weeks apart were considered as standard cycle of RTX, a single dose of 1 g every six months was deemed as a low-dose RTX regimen. Complete and partial response were defined according to physician’s judgment, laboratory and radiological features. Results Thirty-six patients affected with IIM were enrolled. Eighteen patients (50%) required the use of RTX for muscular involvement, 6 (16.7%) for interstitial lung disease (ILD), 12 (33.3%) for both myositis and ILD. We observed complete response to RTX in 25 patients (69.4%), partial response in 7 (19.4%) and no response in 4 (11.1%), with an overall response of 88.8% (partial and complete response). From the subgroup of twenty-five patients that achieved a complete response, six were treated with a low dose maintenance therapy maintaining a complete response to RTX. Twenty-six patients who achieved a complete or partial response were able to decrease the mean daily GC dose. Infections were the major adverse events detected in our study. Conclusions RTX shows favorable outcomes in refractory patients with IIM. A low-dose regimen of RTX appears to be effective in maintaining remission after induction with standard dose. Key Points• The precise pathogenic mechanism of idiopathic inflammatory myopathies (IIM) remains elusive; however, a growing body of data support the autoimmune hypothesis. In this context, rituximab, a B cell-depleting agent, has emerged as a second-line therapeutic option in IIM.• Several studies have assessed It its effectiveness in refractory IIM patients.• Limited information exists on the use of Rituximab as maintenance therapy in patients who have achieved remission following induction therapy with Rituximab.

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Autoantibody testing in myositis: an update

McMorrow,

Anwyll,

Tansley

2024

Current Opinion in Rheumatology

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Purpose of review This review aims to provide an update on myositis autoantibody testing strategies. We have focussed on the reliability and usefulness of different myositis autoantibody detection methods, including commonly used solid phase immunoassays and newer discovery techniques. Recent findings Several studies have highlighted the limitations of currently available immunoassays, particularly when used in populations with low pretest probability and without supporting clinical evidence. While many autoantibodies, such as anti-Jo1, are detected with high sensitivity and specificity, the low incidence of myositis autoantibodies in tested populations reduces their positive predictive value. The low sensitivity of line immunoassays to detect key myositis autoantibodies, including anti-TIF1γ and rarer antisynthetase autoantibodies, is a concern. Summary Myositis autoantibodies are widely accepted as important clinical tools, and hence, there is a significant demand for reliable, accessible, and affordable detection methods. False positives and negative results have the potential to impact on patient care, particularly for malignancy and lung disease associated autoantibodies. Increased availability of myositis autoantibody testing has led to a rise in requests from a broader range of clinicians. It is critically important that clinicians are aware of specific limitations of tests and interpret results in the context of clinical findings.

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Detection of Myositis Autoantibodies by Multi-Analytic Immunoassays in a Large Multicenter Cohort of Patients with Definite Idiopathic Inflammatory Myopathies

Cited by 7 publications

References 48 publications

Asymmetric darkened knuckles with subclinical myopathy revealed anti-Mi2b positive dermatomyositis

Asymmetric darkened knuckles with subclinical myopathy revealed anti-Mi2b positive dermatomyositis

Effectiveness and safety of low dose Rituximab as remission-maintenance treatment for patients with refractory idiopathic inflammatory myopathies: results of a retrospective study from a monocentric cohort

Autoantibody testing in myositis: an update

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