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- W2784299924 abstract "Immune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP.This is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP).Compared with DM-VP, IMPP patients more commonly had interstitial lung disease (ILD) (p < 0.01), Raynaud phenomenon (p < 0.05), mechanic's hands (p < 0.05), arthralgias (p < 0.001), and a sustained response to immunomodulatory therapy (p < 0.05), and less frequently had a concurrent malignancy (p < 0.01). IMPP patients had higher serum creatine kinase values (p < 0.05), more frequent serum Jo-1 (p < 0.03) or SSA/SSA52 autoantibodies (p < 0.05), and less frequent antinuclear antibodies (p < 0.01). IMPP patients with serum Jo-1/antisynthetase antibodies were more likely to have ILD (p < 0.05) and inflammatory arthritis (p < 0.05) than IMPP patients without these antibodies.IMPP myopathology is associated with an increased risk of ILD, Raynaud phenomenon, mechanic's hands, and inflammatory arthritis when compared with another immune myopathy (DM-VP). IMPP patients require regular screening for ILD, particularly those with antisynthetase antibodies. The absence of myositis-specific autoantibodies in a large percentage of IMPP patients emphasizes the important role for myopathology in identifying patients at higher risk of severe comorbid conditions such as ILD." @default.
- W2784299924 created "2018-01-26" @default.
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- W2784299924 date "2018-01-18" @default.
- W2784299924 modified "2023-10-13" @default.
- W2784299924 title "Immune myopathies with perimysial pathology" @default.
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- W2784299924 doi "https://doi.org/10.1212/nxi.0000000000000434" @default.
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