Diagnosis and Management of Immune-Mediated Myopathies

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Presentation transcript:

Diagnosis and Management of Immune-Mediated Myopathies Margherita Milone, MD, PhD Mayo Clinic Proceedings Volume 92, Issue 5, Pages 826-837 (May 2017) DOI: 10.1016/j.mayocp.2016.12.025 Copyright © 2017 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 1 Muscle biopsy specimens from a patient with necrotizing autoimmune myopathy and anti–3-hydroxy-3-methylglutaryl coenzyme A reductase antibodies. A, There are numerous scattered necrotic muscle fibers (asterisks), fewer regenerating fibers (arrow), and no inflammatory exudate; few fibers have internalized nuclei (arrowhead) (hematoxylin-eosin). B, Macrophages invading necrotic fibers appear red (acid phosphatase stain). Mayo Clinic Proceedings 2017 92, 826-837DOI: (10.1016/j.mayocp.2016.12.025) Copyright © 2017 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 2 Features of inclusion body myositis. Note weakness of the finger flexors (A) but spared strength of finger extensors (B) in the same patient and weakness of the orbicularis oculi (C), as suggested by inability to close the eyes, in another patient. Mayo Clinic Proceedings 2017 92, 826-837DOI: (10.1016/j.mayocp.2016.12.025) Copyright © 2017 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 3 Muscle biopsy specimens from a patient with inclusion body myositis. A, Autoaggressive inflammatory infiltrates invading a nonnecrotic muscle fiber (arrow) (hematoxylin-eosin, original magnification ×40). B, Rimmed vacuoles (arrow) (trichrome stain, original magnification ×40). C, Congophilic inclusions within a muscle fiber appear bright red (arrow) (Congo red–stained section viewed under rhodamine optics, original magnification ×40). Mayo Clinic Proceedings 2017 92, 826-837DOI: (10.1016/j.mayocp.2016.12.025) Copyright © 2017 Mayo Foundation for Medical Education and Research Terms and Conditions

Figure 4 Muscle biopsy specimens from a patient with dermatomyositis. A and B, Note perifascicular muscle atrophy (arrows), a hallmark of dermatomyositis (A, hematoxylin-eosin; B, ATPase stain, pH 4.3). C, The inflammatory exudate concentrates in the perimysium at perivascular sites (arrow) (hematoxylin-eosin). D, Additional structural abnormalities are present in perifascicular regions: internalized nuclei (blue arrow), necrotic (asterisk) and regenerating (arrowheads) muscle fibers, vacuolar changes (black arrow) (hematoxylin-eosin). Mayo Clinic Proceedings 2017 92, 826-837DOI: (10.1016/j.mayocp.2016.12.025) Copyright © 2017 Mayo Foundation for Medical Education and Research Terms and Conditions