High risk of cancer in autoimmune necrotizing myopathies: usefulness of myositis specific antibody

Yves Allenbach, Jeremy Keraen, Anne-Marie Bouvier, Valérie Jooste, Nicolas Champtiaux, Baptiste Hervier, Yoland Schoindre, Aude Rigolet, Laurent Gilardin, Lucile Musset, Jean-Luc Charuel, Olivier Boyer, Fabienne Jouen, Laurent Drouot, Jeremie Martinet, Tanya Stojkovic, Bruno Eymard, Pascal Laforêt, Antony Behin, Emmanuelle Salort-Campana, Olivier Fain, Alain Meyer, Nicolas Schleinitz, Kuberaka Mariampillai, Aurelie Grados, Olivier Benveniste
Brain 2016, 139 (Pt 8): 2131-5
Cancer can occur in patients with inflammatory myopathies. This association is mainly observed in dermatomyositis, and myositis-specific antibodies have allowed us to delineate patients at an increased risk. Malignancy is also reported in patients with necrotizing autoimmune myopathies, but the risk remains elusive. Anti-signal recognition particle or anti-HMGCR antibodies have been specifically associated with necrotizing autoimmune myopathies. We aimed at screening the incidence of cancer in necrotizing autoimmune myopathies. A group of patients (n = 115) with necrotizing autoimmune myopathies with or without myositis-specific antibodies was analysed. Malignancy occurred more frequently in seronegative necrotizing autoimmune myopathies patients and in HMGCR-positive patients compared to anti-signal recognition particle positive patients. Synchronous malignancy was diagnosed in 21.4% and 11.5% of cases, respectively, and incidence of cancer was higher compared to the general population in both groups. No specific type of cancer was predominant. Patients suffering from a synchronous cancer had a decreased median survival time. Cancer screening is necessary in seronegative necrotizing autoimmune myopathies and in HMGCR-positive patients but not in anti-signal recognition particle-positive patients.

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