Autoantibodies in Myositis nect.com
Autoantibodies in Myositis
Pennsylvania Rheumatology Society Annual Meeting
September 23, 2018
Chester V. Oddis, MD Director, Myositis Center University of Pittsburgh
Disclosures
? Genentech: Clinical trial support ? Bristol Myers Squibb: Clinical trial support ? Mallinckrodt: Clinical trial support ? Octapharma: Clinical trial support
Lecture Objectives
? Discuss clinical features of myositis classification ? Discuss spectrum of autoantibodies seen in
patients with myositis
Classification of Myositis
? Adult polymyositis ? Adult dermatomyositis ? Juvenile myositis (DM >> PM) ? Malignancy-associated myositis ? Myositis in overlap with another AI disease ? Inclusion body myositis (IBM)
IIM: Diagnostic Criteria
Bohan and Peter (1975)
? Symmetric proximal muscle weakness ? Elevation of serum muscle enzymes: CK, aldolase, AST, ALT, LDH ? Myopathic electromyographic abnormalities: sharp waves,
fibrillations, polyphasic motor units, high frequency repetitive discharges ? Characteristic muscle pathology: myofiber degeneration/regeneration, MNC infiltrates, perifascicular atrophy ? Cutaneous features (heliotrope, Gottron sign/papules)
IIM: Diagnostic Criteria
Bohan and Peter (1975) ? Symmetric proximal muscle weakness ? Elevation of serum muscle enzymes: CK, aldolase, AST, ALT, LDH ? Myopathic electromyographic abnormalities: sharp waves,
fibrillations, polyphasic motor units, high frequency repetitive discharges ? Characteristic muscle pathology: myofiber degeneration/regeneration, MNC infiltrates, perifascicular atrophy ? Cutaneous features (heliotrope, Gottron sign/papules)
Problems with B&P Criteria ? No good way to exclude other myopathies ? Misclassification of IBM as PM ? Each criterion not explicitly defined
Lundberg et al, Ann RD, 2018
? Candidate variables assembled from published criteria/expert opinion
? Data collected from rheum/derm/neuro/peds clinics worldwide
? New criteria were derived and each item was assigned a `weighted' score
? Total score corresponds to a probability of having IIM
Lundberg et al, Ann RD, 2018
Lundberg et al, Ann RD, 2018
? For patients without classic DM rashes, do a muscle biopsy.
? For DM patients without muscle involvement do a skin biopsy.
? These criteria provide a score and probability for having IIM (for clinical trial purposes)
Rashes of DDeerrmmaattoommyyoossiittiiss Rashes
Classification of Myositis
? Adult polymyositis ? Adult dermatomyositis ? Juvenile myositis (DM >> PM) ? Malignancy-associated myositis ? Myositis in overlap with another AI disease ? Inclusion body myositis (IBM)
Classification of Myositis
? Adult polymyositis ? Adult dermatomyositis ? Juvenile myositis (DM >> PM) ? Malignancy-associated myositis ? Myositis in overlap with another AI disease ? Inclusion body myositis (IBM)
Polymyositis Mimics
? Endocrine myopathies
hyper/hypothyroid
? Drug or toxic myopathies ? Metabolic myopathies ? Mitochondrial myopathies ? Muscular dystrophies ? Infectious myositis ? Neuropathies/neurologic syndromes ? Paraneoplastic syndromes ? Other connective tissue disorders ? Miscellaneous
amyloid, sarcoid
Muscle Biopsy is a Must in "Polymyositis "
(unlike classic DM)
Muscle Biopsy is a Must in "Polymyositis "
(unlike classic DM)
And maybe pathologic classification may even be better
than "clinical" classification
Pathologic Myositis Classification
Pestronk, Curr Opin Rheum, 2011
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