358 Inflammatory Myopathies

Question 1

represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness

Answer 1

inflammatory myopathies

Question 2

Three major groups of inflammatory myopathies

Answer 2

polymyositis, dermatomyositis and inclusion body myositis

Question 3

True or false. These disorders present as a progressive and symmetric muscle weakness except to IBM which can have an asymmetric pattern

Answer 3

True

Question 4

True or false. Falling is common in IBM as the quadriceps are affected early leading to buckling of the knees.

Answer 4

True

Question 5

Muscles spared in inflammatory myopathies even in advance cases

Answer 5

ocular muscles

Question 6

True or False. Ocular and facial muscles are spared in inflammatory myopathies.

Answer 6

False. It is only spared in PM and DM; in IBM it is commonly affected

Question 7

True or false. All types of inflammatory myopathies involve the pharyngeal and neck flexor muscles causing dyspahge and head drop

Answer 7

True

Question 8

Inflammatory myopathis frequently associaed with SLE

Answer 8

Polymyositis

Question 9

Which inflammatory myopathy affects both children and adults

Answer 9

dermatomyositis

Question 10

Which inflammatory myopathy affect older population, those aging more than 50 years old

Answer 10

inclusion body myopathy

Question 11

Connective tissue disorder associated with dermatomyositis

Answer 11

scleroderma and mixed connective tissue disorder (overlap syndrome)

Question 12

Inflammatory myopathy associated with all drugs, virus, parasides and bacteria

Answer 12

polymyositis

Question 13

Inflammatory myopathy associated with malignancy

Answer 13

dermatomyositis

Question 14

inflammatory myopathy associated with virus infection and which one is not

Answer 14

Polymyositis and IBM can be associated with virus while it is not proven in DM

Question 15

distinctive inflammatory myopathy characterized by an accompanying rash which more often precede the muscle weakness

Answer 15

dermatomyositis

Question 16

rash that consists of blue purple discoloration on the upper eyelids of edema; and which inflammatory myopathy is it associated

Answer 16

heliotrope rash of dermatomyositis

Question 17

flat red rash on the face and upper trunk; erythema of the knuckles with raised violaceous scaly eruption

Answer 17

Gottron’s sign of dermatomyositis

Question 18

erythematous rash on the elbows, malleoli, neck and anterior chest

Answer 18

V sign of dermatomyositis

Question 19

erythemous rash in the back and shoulders

Answer 19

shawl sign of dermatomyositis

Question 20

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

Answer 20

mechanic’s hands of dermatomyositis

Question 21

fasciitis and thickening of skin associated with ingestion of contaminated L tryptophan

Answer 21

eosinophilia-myalgia syndrome

Question 22

most common of the inflammatory myopathies in patients more than 50 years of age

Answer 22

inclusion body myopathy (IBM)

Question 23

True or false. weakness and atrophy of the distal muscles esp foot extensors and deep finger flexors occurs in almost all cases IBM

Answer 23

True

Question 24

True or false. Dyphagia is common in IBM occuring at 60%

Answer 24

True

Question 25

Inflammatory myopathy associated with joint contractures

Answer 25

dermatomyositis

Question 26

dyphagia and GI symptoms are associated with which inflammatory myopathies

Answer 26

dermatomyositis and IBM

Question 27

True or false. signs of RA, SLE or Sjogrens syndrome are very rare in patients with DM

Answer 27

True

Question 28

critical early event that triggers release of proinflammatory sytokines and chemokines in dermatomyositis

Answer 28

activation of complement C5b-9

Question 29

immunopathologic characteristic of PM and IBM; its presence can aid in confirming the histologic diagnosis of PM

Answer 29

CD8/MCH-1 complex

Question 30

Viruses associated with myositis

Answer 30

coxsackievirus, influenza, paramyxoviruses, mumps, CMV and EBV

Question 31

Best evidence of viral connection in PM and IBM

Answer 31

retroviruses

Question 32

antiretroviral drug which may present with fatigue, myalgia, mild muscle weakness and mild elevation of creatine kinase

Answer 32

zidovudine (AZT)

Question 33

How does AZT lead to myopathy

Answer 33

inhibits DNA polymerase found i in mitochodrial matrix; seen in long term therapy

Question 34

How is AZT induced myopathy managed

Answer 34

improves when drug is discontinued

Question 35

in Asia, malignancy associated with dermatomyositis

Answer 35

nasopharyngeal malignancy