Is RIG-I staining a reliable biomarker of dermatomyositis?


Dermatomyositis (DM) is inflammatory myopathy or myositis characterised by muscle weakness and skin manifestations. In the differential diagnosis of DM, the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis. Retinoic acid inducible-gene I (RIG-I), a receptor of innate immunity that promotes type I interferon, was observed in perifascicular areas in DM. This study compared the value of RIG-I expression with perifascicular atrophy as a biomarker of DM in 115 coded muscle biopsies: 44 patients with DM, 18 with myositis with overlap, 8 with ASS, 27 with non-DM inflammatory myopathy (16 with polymyositis, 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with muscular dystrophy (4 with dysferlinopathy, 4 with fascioscapulohumeral muscle dystrophy) and 10 healthy controls. The authors found that the perifascicular pattern of RIG-I expression supports the diagnosis of DM. Of importance for clinical and therapeutic studies, the inclusion of RIG-I in the routine pathological staining of samples in inflammatory myopathy will allow to gather more homogeneous subgroups of patients in terms of immunopathogenesis.

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Suárez-Calvet X, Gallardo E, Pinal-Fernandez I, et al. RIG-I expression in perifascicular myofibers is a reliable biomarker of dermatomyositis. Arthritis Res Ther. 2017 Jul 24;19(1):174.