358 Inflammatory Myopathies Flashcards
represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness
inflammatory myopathies
Three major groups of inflammatory myopathies
polymyositis, dermatomyositis and inclusion body myositis
True or false. These disorders present as a progressive and symmetric muscle weakness except to IBM which can have an asymmetric pattern
True
True or false. Falling is common in IBM as the quadriceps are affected early leading to buckling of the knees.
True
Muscles spared in inflammatory myopathies even in advance cases
ocular muscles
True or False. Ocular and facial muscles are spared in inflammatory myopathies.
False. It is only spared in PM and DM; in IBM it is commonly affected
True or false. All types of inflammatory myopathies involve the pharyngeal and neck flexor muscles causing dyspahge and head drop
True
Inflammatory myopathis frequently associaed with SLE
Polymyositis
Which inflammatory myopathy affects both children and adults
dermatomyositis
Which inflammatory myopathy affect older population, those aging more than 50 years old
inclusion body myopathy
Connective tissue disorder associated with dermatomyositis
scleroderma and mixed connective tissue disorder (overlap syndrome)
Inflammatory myopathy associated with all drugs, virus, parasides and bacteria
polymyositis
Inflammatory myopathy associated with malignancy
dermatomyositis
inflammatory myopathy associated with virus infection and which one is not
Polymyositis and IBM can be associated with virus while it is not proven in DM
distinctive inflammatory myopathy characterized by an accompanying rash which more often precede the muscle weakness
dermatomyositis
rash that consists of blue purple discoloration on the upper eyelids of edema; and which inflammatory myopathy is it associated
heliotrope rash of dermatomyositis
flat red rash on the face and upper trunk; erythema of the knuckles with raised violaceous scaly eruption
Gottron’s sign of dermatomyositis
erythematous rash on the elbows, malleoli, neck and anterior chest
V sign of dermatomyositis
erythemous rash in the back and shoulders
shawl sign of dermatomyositis
cuticles may ne irregular, thickened, distorted and the lateral and palmar areas of the fingers may become rough and cracked with irregular “dirty” horizontal lines
mechanic’s hands of dermatomyositis
fasciitis and thickening of skin associated with ingestion of contaminated L tryptophan
eosinophilia-myalgia syndrome
most common of the inflammatory myopathies in patients more than 50 years of age
inclusion body myopathy (IBM)
True or false. weakness and atrophy of the distal muscles esp foot extensors and deep finger flexors occurs in almost all cases IBM
True
True or false. Dyphagia is common in IBM occuring at 60%
True
Inflammatory myopathy associated with joint contractures
dermatomyositis
dyphagia and GI symptoms are associated with which inflammatory myopathies
dermatomyositis and IBM
True or false. signs of RA, SLE or Sjogrens syndrome are very rare in patients with DM
True
critical early event that triggers release of proinflammatory sytokines and chemokines in dermatomyositis
activation of complement C5b-9
immunopathologic characteristic of PM and IBM; its presence can aid in confirming the histologic diagnosis of PM
CD8/MCH-1 complex
Viruses associated with myositis
coxsackievirus, influenza, paramyxoviruses, mumps, CMV and EBV
Best evidence of viral connection in PM and IBM
retroviruses
antiretroviral drug which may present with fatigue, myalgia, mild muscle weakness and mild elevation of creatine kinase
zidovudine (AZT)
How does AZT lead to myopathy
inhibits DNA polymerase found i in mitochodrial matrix; seen in long term therapy
How is AZT induced myopathy managed
improves when drug is discontinued
in Asia, malignancy associated with dermatomyositis
nasopharyngeal malignancy
