Successful treatment of cardiac involvement in dermatomyositis with rituximab

doi:10.1016/j.jbspin.2007.05.011

Joint Bone Spine

Volume 75, Issue 3, May 2008, Pages 334-337

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Abstract

Polymyositis and dermatomyositis are autoimmune inflammatory myopathies characterized by muscle weakness and inflammation. Current recommended therapy includes corticosteroids as mainstay treatment in addition to immunosuppressant. We present herein a 25 year-old female with dermatomyositis and cardiac involvement resistant to disease modifying anti-rheumatic drugs and anti-tumor necrosis factor-α. She was treated with anti-CD20 monoclonal antibody, rituximab. The patient demonstrated a remarkable clinical and laboratory response. B-cell depletion therapy with rituximab may be a viable option in patients with dermatomyositis and heart disease.

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Response to cyclophosphamide and rituximab therapy in idiopathic inflammatory myopathies: A single center experience
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Citation Excerpt :
B lymphocytes play an important role in the pathogenesis of IIM, proved by the identification of circulating autoantibodies in 80% of patients, and by B cells demonstration in inflammatory muscle fibers in DM and PM patients, and in perivascular regions of muscles in DM [9,12]. A number of case reports, case series, and very few large treatment trials recommended RTX to be efficient in refractory IIM treatment [5,13–19]. However, no standardized therapeutic guidelines for treatment of IIM have been established, perhaps due to the insufficient randomized controlled trials and the rarity of such diseases.
To evaluate the clinical response to cyclophosphamide (CYC), and rituximab (RTX) following CYC regarding muscle power and creatine kinase (CK) level changes, and the ability to manage cardiac and bulbar affection in dermatomyositis (DM) and polymyositis (PM) patients.
Twenty two idiopathic inflammatory myopathies (IIM) patients: 14 (63.6%) DM and 8 (36.4%) PM were categorized into CYC group (n = 17) received only CYC, and RTX group received RTX following refractory CYC (n = 5). History taking and clinical examination were done, and Medical Research Council Sum Score (MRC-SS) and CK were evaluated at baseline visit, by end of monthly CYC, and 18 months after CYC or RTX. Improvement of cardiac affection or bulbar manifestations was checked.
Patients were 16 females and 6 males (F:M 2.7:1) and mean age was 34.9 ± 14.9 years. The patients were followed up for 7.7 ± 4.3 years. MRC–SS increased in (CYC group) and in (RTX group) (p < 0.001, and p = 0.018 respectively). CK levels decreased significantly in CYC group (p < 0.001) but not in RTX group. MRC-SS changes in males, females, DM, PM patients were (p = 0.007, p < 0.001, p < 0.001, p = 0.006 respectively), and CK changes (p = 0.17, p < 0.001, p < 0.001, p = 0.006 respectively). The two DM cardiac patients and the three PM patients with bulbar manifestations in (RTX group) showed improvement in their extra-muscular manifestations.
CYC can be an effective drug in the treatment of IIM muscle affection. RTX may be promising for refractory myositis, cardiac and bulbar manifestations, so these patients should be a target for its early administration.
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Idiopathic inflammatory myopathies (IIMs), collectively called myositis, are a heterogeneous group of diseases affecting adults and children. The IIMs are characterized by symmetrical, proximal muscle weakness and by inflammatory infiltrates in skeletal muscles. Based on differences in clinical and histopathological features, they can be divided into different subgroups, the most common forms being dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis (sIBM), and in children juvenile dermatomyositis (JDM). Other organs are frequently involved in patients with IIM eg, lungs, gastrointestinal tract, and the heart, emphasizing that these are systemic inflammatory diseases. IIMs are associated with increased mortality, and cardiovascular involvement has been recognized as the main prognostic factor for death. Processes affecting the heart in IIM include coronary artery disease and cardiac affection caused by inflammation. In this chapter, we focus on adult PM and DM and JDM as little is known about cardiac involvement in the other subsets.
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Case reportSuccessful treatment of cardiac involvement in dermatomyositis with rituximab

Abstract

Clin Chim Acta

Rheum Dis Clin North Am

Semin Arthritis Rheum

Inflammatory muscle disease: clinical features

Inflammatory diseases of muscle and other myopathies

Cardiac involvement in autoimmune myositis and mixed connective tissue disease

Lupus

Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging

Ann Rheum Dis

Serum skeletal troponin I in inflammatory muscle disease: relation to creatine kinase, CKMB and cardiac troponin I

Ann Rheum Dis

Case report
Successful treatment of cardiac involvement in dermatomyositis with rituximab