Abstract
Among acquired idiopathic inflammatory myopathies necrotizing auto-immune myopathy (NAM) has been recognized as distinct based on morphological features. Anti-SRP and antiHMCR antibody (Ab) are specifically associated with NAM. Data concerning histological features are sparse. We aim to describe the histological pattern of the muscle of a large series ( N = 44) of NAM-seropositive patients. Fiber necrosis and regeneration were defined based on specific histological features. For immohistochemical analysis, a semi-quantitative score (0–5) was used for MHC-I (e.g. 0: no overt expression, 2: focal over-expression more than 50%, 5: diffuse and intense over-expression). ImageJ software on digitally completely scanned slides was used. Forty-four NAM patients were included (SRP + n = 25, HMGCR + n = 19). The percentage of necrotizing fibers was 2.1 ± 1.7%. The percentage of regenerating fibers was 16 ± 12.5%. Percentages of necrosis and regeneration were strongly correlated ( r = 0.61, p 2 , but 20.4% of patients exhibited significant infiltrates (>20 CD3 + T cells/mm 2 ). A correlation between the CD3 + T cells density and CD8 + T cells density with the percentage of fibre necrosis was observed ( r = 0.5, p r = 0.4, p = 0.008). Along that line, a strong correlation between CD8 + T cell density and MHC-I over-expression was seen ( r = 0.6; p
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