Inflammatory Myopathies

Question 1

Inflammatory myopathy associated with proximal muscle weakness and a heliotrope rash

Answer 1

Dermatomyositis

Question 2

From of dermatomyositis associated with calcinosis (juvenile vs adult)

Answer 2

Juvenile

Question 3

Which autoantibody is associated with calcinosis seen in juvenile dermatomyositis

Answer 3

anti-NXP-2

Question 4

Autoantibody seen in classic dermatomyositis

Answer 4

anti-Mi-2

Question 5

Autoantibodies (2) in dermatomyositis associated with malignancy

Answer 5

Anti-TIF1ɣ

Anti-NXP2

Question 6

Autoantibody in dermatomyositis associated with early skin changes and systemic symptoms in adults

Answer 6

Anti-SAE

Question 7

Autoantibody in dermatomyositis associated with severe skin lesions and interstitial lung disease

Answer 7

Anti-MDA5

Question 8

HLA-DRB1 and HLA-B are associated with which inflammatory myopathy?

Answer 8

Dermatomyositis

Question 9

Inflammatory myopathy classically associated with perifascicular atrophy, vacuolar degeneration and decreased capillaries

Answer 9

Dermatomyositis

Question 10

Inflammatory myopathy associated with aminoacyl transferase synthase antibodies

Answer 10

Antisynthetase syndrome

Question 11

Inflammatory myopathy associated with perifascicular necrosis

Answer 11

Antisynthetase syndrome

Question 12

The most common acquired myopathy in adults >50

Answer 12

Inclusion body myositis

Question 13

Autoantibody associated with inclusion body myositis

Answer 13

anti-cytosolic 5’-nucleotidase 1A

Question 14

Inflammatory myopathy associated with HLA-DR3, HLA-DR52, HLA-B8

Answer 14

Inclusion body myositis

Question 15

Which HLA haplotype is strongly protective against inclusion body myositis

Answer 15

HLA-B4

Question 16

Inflammatory myopathy characterized by the presence of rimmed vacuoles and CD8+ infiltrate of non-necrotic fibers

Answer 16

Inclusion body myositis

Question 17

Rimmed vacuoles seen in inclusion body myositis are derived from which organelle?

Answer 17

Lysosomal origin

Question 18

Which immunohistochemical antibody corresponds to tubulofilamentous inclusions seen in inclusion body myositis

Answer 18

SMI31

Question 19

Inflammatory myopathy associated with markedly elevated CK levels

Answer 19

Immune-mediated necrotizing myopathy

Question 20

Anti-SRP and anti-HMGCR are commonly associated with which inflammatory myopathy?

Answer 20

Immune-mediated necrotizing myopathy

Question 21

Inflammatory myopathy associated with abundant regenerating fibers, pipestem capillaries and lacks lymphocytic inflammation

Answer 21

Immune-mediated necrotizing myopathy

Question 22

Pipestem capillaries seen in immune-mediated necrotizing myopathy are due to what phenomenon?

Answer 22

Complement deposition

Question 23

Which autoimmune disease is granulomatous myositis commonly associated?

Answer 23

Sarcoidosis

Question 24

Which inflammatory myopathy presents as a well-circumscribes intramuscular mass “inflammatory pseudotumor”?

Answer 24

Focal myositis

Question 25

Are the inflammatory cells seen in focal myositis predominantly CD8+ or CD4+?

Answer 25

CD4+

Question 26

Inflammatory myopathy associated with exposure to aluminum in vaccination products

Answer 26

Macrophage myofasciitis