NP17-938 D.P. Bx: 9/8/2017.

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Presentation transcript:

NP17-938 D.P. Bx: 9/8/2017

CD3 CD68

MHC class I

A-SRP antibody(+) necrotizing myopathy Normal immunohistochemical staining: dystrophin; a-sarcoglycan; merosin; dysferlin; caveolin 3; a-dystroglycan; emerin. Pathologic diagnosis: Muscle, left thigh, biopsy: Myopathy with necrotizing features, early chronic, active. “a-SRP Ab found!!” A-SRP antibody(+) necrotizing myopathy

Advances in the diagnosis & Treatment of Neuromuscular Disorders 2012 Pathology of inflammatory myopathy Kurenai Tanji, M.D., Ph.D. Neuromuscular Pathology Laboratory Division of Neuropathology Columbia University Medical Center

Inflammatory myopathy (IM): heterogeneous group of disorders Infectious Non-infectious Immune-mediated (idiopathic) IM macrophagic myofasciitis granulomatous or eosinophilic myo-fasciitis focal myositis (nodular myositis) sarcoidosis vasculitis paraneoplastic involvement graft vs. host disease muscle involvement in collagen vascular disease (e.g. MCTD, PR, RA, Sjogren, SLE, SS) secondary inflammation in other myopathy (e.g. FSH, LGMDs) Fix the content

Idiopathic IM Dermatomyositis (DM) Polymyositis (PM) Inclusion body myositis (sIBM), sIBM-like syndrome Immune-mediated necrotizing myopathies (IMNM)

Myositis- specific (related) Abs : ever-expanding list Laboratory data EMG Myositis- specific (related) Abs : ever-expanding list Abs a/w a-synthetase syndrome a- Jo-1 >> a- PL-7, a- PL-12, a- EJ, a-OJ, a- KS, a-Zo Abs a/w DM a- Mi-2, a- p155/140, a- MJ (a-NXP2), a- MDA5, a- SUMO-1, a-TIFg (w/malignancy) Abs a/w IMNM a- SRP, a- HMGCR Abs a/w MCTD, overlap syndrome a- PM/Scl, a- U1RNP, a-Ku Abs a/w sIBM a-cN1A (specificity being questioned as of 2018) Others : a- mito. Abs (myositis w/ or w/0 primary biliary cirrhosis); a-FHL1 (“severe inflam. myopath myopathies”); a-AQP 4 (myelitis optica spectrum) Others : muscle biopsy; ??? biomarker (? IP-10)

a + Degenerating/Necrotic fibers Regenerating fibers Myofiber atrophy Tissue diagnosis Degenerating/Necrotic fibers Regenerating fibers Myofiber atrophy Evidence of inflammatory infiltration + a

a + Morphological hallmark of autoimmune myopathies Degenerating/Necrotic fibers Regenerating fibers Myofiber atrophy Evidence of inflammatory infiltration + Distinctive histological features suggesting different pathophysiological mechanisms underlying each disease a

Dermatomyositis perifascicular pattern of involvement For instance: Dermatomyositis perifascicular pattern of involvement perimysial > endomysial inflammation Polymyositis CD8 (+) cytotoxic T-cell infiltration perimysial < endomysial inflammation IBM rimmed vacuoles mitochondrial abnormality (secondary)

DM - Perifascicular pattern

DM, IMMUNE COMPLEXES (C5b-9) IN BLOOD VESSEL WALL