Efficacy of Rituximab in Refractory Inflammatory Myopathy Associated With Coexistence of Behçet's Disease and Antiphospholipid Syndrome

  • Verónica Guiomar Internal Medicine Department, Centro Hospitalar de São João, Porto, Portugal
  • Diana Oliveira Internal Medicine Department, Centro Hospitalar de São João, Porto, Portugal
  • Cristina Correia Internal Medicine Department, Centro Hospitalar de São João, Porto, Portugal
  • Edite Pereira Internal Medicine Department, Centro Hospitalar de São João, Porto, Portugal

Keywords

Rituximab, inflammatory myopathy, polymyositis, Behçet's disease, antiphospholipid syndrome

Abstract

A 43-year-old Caucasian male initiated myalgias and loss of muscle strength in the upper and lower limbs, but especially at the shoulder and pelvic girdle. Creatinine phosphokinase was elevated seven-fold above the normal reference value and aldolase was slightly elevated. He had a previous diagnosis of Behçet's disease, antiphospholipid syndrome and hypertriglyceridaemia. At this time, he was on azathioprine 150 mg daily, colchicine 1 mg daily, warfarin and fenofibrate 200 mg daily. Fenofibrate was stopped and creatinine phosphokinase re-evaluated 2 months later, but it was higher, with persistent myalgias. By this time, prednisolone was restarted and the azathioprine dose reduced until it was discontinued. Nevertheless, 2 months after stopping azathioprine, the patient remained symptomatic and creatinine phosphokinase was persistently elevated. At this point, the authors requested myositis antibody testing to exclude overlap with a third autoimmune disorder, and Ro52 antibody was positive. Electromyography was normal. Magnetic resonance imaging of lower limb muscles was compatible with polymyositis. Muscular biopsy of the medial gastrocnemius revealed inflammatory myopathy. The authors proposed treatment with rituximab and after 3 months, the patient had clinically and analytically improved, with reduction of creatinine phosphokinase, without adverse reactions. As we can see in this case, rituximab could be a secure treatment for patients with idiopathic inflammatory myopathy without improvement on glucocorticoids plus another immunosuppressive agent. This patient has a rare overlap syndrome, since this is the first case of an association between inflammatory myopathy, Behçet's disease and antiphospholipid syndrome described in the literature.

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References

  • Mandel DE, Malemud CJ, Askari AD. Idiopathic inflammatory myopathies: a review of the classification and impact of pathogenesis. Int J Mol Sci 2017;18:1084.

  • Sasaki H, Kohsaka H. Current diagnosis and treatment of polymyositis and dermatomyositis. Mod Rheumatol 2018;28:913–921.

  • de Souza FHC, Miossi R, de Moraes JCB, Bonfá E, Shinjo SK. Favorable rituximab response in patients with refractory idiopathic inflammatory myopathies. Adv Rheumatol 2018;58:31.

  • Kim JH, Lee KA, Jung MY, Kim AR, Yoon JM, Shim HJ, et al. Pulmonary thromboembolism in patient with coexistence of Behçet’s disease and antiphospholipid syndrome. Int J Rheum Dis 2018;21:2188–2192.

  • Sherer Y, Livneh A, Levy Y, Shoenfeld Y, Langevitz P. Dermatomyositis and polymyositis associated with the antiphospholipid syndrome-a novel overlap syndrome. Lupus 2000;9:42–46.
  • Published: 2019-11-11

    Issue: Vol 6 Iss11 (view)

    Section: Articles

    How to cite:
    Guiomar, V., Oliveira, D., Correia, C., & Pereira, E. (2019). Efficacy of Rituximab in Refractory Inflammatory Myopathy Associated With Coexistence of Behçet’s Disease and Antiphospholipid Syndrome. European Journal of Case Reports in Internal Medicine, 6(11). https://doi.org/https://doi.org/10.12890/2019_001294

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