Acquired immune and inflammatory myopathies: Pathologic classification

Purpose of review: We discuss pathology-based characterization and classification of acquired immune and inflammatory myopathies (IIMs). Recent findings:Several types of IIMs do not fit well into the typical IIM subclassifications: dermatomyositis, polymyositis and inclusion body myositis (IBM). Myopathologic features that can provide additional diagnostic clarification in IIM are types of muscle fiber pathology; immune changes (cellular and humoral); and tissues with distinctive involvement (connective tissue, vessels and muscle fibers). Pathologic classification categories include immune myopathies with perimysial pathology (IMPP), a group that can be associated with antisynthetase antibodies; myovasculopathies, including childhood dermatomyositis; immune polymyopathies, active myopathies with little inflammation such as the myopathy with signal recognition particle antibodies; immune myopathies with endomysial pathology (IM-EP), illustrated by brachio-cervical inflammatory myopathy (BCIM); histiocytic inflammatory myopathies, like sarcoid myopathy; and inflammatory myopathies with vacuoles, aggregates and mitochondrial pathology (IM-VAMP), which have inclusion body myositis as a pathologic subtype and are poorly treatable. Some myopathologic features, like B-cell foci and alkaline phosphatase staining of capillaries or perimysium, are more likely to be present in treatable categories of IIM. Summary: Myopathology can be used to classify IIM. Identification of distinctive myopathologic changes in IIM can improve diagnostic and prognostic accuracy and focus treatment, therapeutic trials and studies of pathogenic factors.