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Table 195-1

Features Associated with Inflammatory Myopathies

CharacteristicPolymyositisDermatomyositisInclusion Body Myositis
Age at onset>18 yearsAdulthood and childhood>50 years
Familial associationNoNoYes, in some rare cases
Extramuscular manifestationsYesYesYes
Associated conditions
Connective tissue diseasesYesaScleroderma and mixed connective tissue disease (overlap syndromes)Yes, in up to 20% of casesa
Systemic autoimmune diseasesbFrequentInfrequentInfrequent
MalignancyNoYes, in up to 15% of casesNo
VirusesYescUnprovenYesc
DrugsdYesYes, rarelyNo
Parasites and bacteriaeYesNoNo

aSystemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, systemic sclerosis, mixed connective tissue disease.

bCrohn's disease, vasculitis, sarcoidosis, primary biliary cirrhosis, adult celiac disease, chronic graft-versus-host disease, discoid lupus, ankylosing spondylitis, Behçet's syndrome, myasthenia gravis, acne fulminans, dermatitis herpetiformis, psoriasis, Hashimoto's disease, granulomatous diseases, agammaglobulinemia, monoclonal gammopathy, hypereosinophilic syndrome, Lyme disease, Kawasaki disease, autoimmune thrombocytopenia, hypergammaglobulinemic purpura, hereditary complement deficiency, IgA deficiency.

cHIV (human immunodeficiency virus) and HTLV-1 (human T cell lymphotropic virus type 1).

dDrugs include penicillamine (dermatomyositis and polymyositis), zidovudine (polymyositis), statins (necrotizing, toxic, or autoimmune myositis), and contaminated tryptophan (dermatomyositis-like illness). Other myotoxic drugs may cause myopathy but not an inflammatory myopathy (see text for details).

eParasites (protozoa, cestodes, nematodes), tropical and bacterial myositis (pyomyositis).