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1% vs 30.4%, P=0.05), without any case of rapidly progressive interstitial lung disease. At muscle histology, anti-Mi2+ patients showed more necrotic/degenerative fibres than anti-Mi2- patients [mean 5.3% (5) vs 0.8% (1), P0.01], but similar to IMNM [5.9% (6), P0.05]. In addition, the endomysial macrophage score was similar between anti-Mi2+ and IMNM patients [mean 1.2 (0.9) vs 1.3 (0.5), P0.05], whereas lower macrophage infiltration was found in anti-Mi2- DM [mean 0.4 (0.5), 0.01]. Anti-Mi2+ patients represent a specific