AutoImmune Myopathies

Question 1

Heliotrope Rash/ Gottron Sign are pathognomonic for what condition..

Answer 1

Dermatomyositis

Question 2

What is the major DDx for Dermatomyositis and Polymyositis

Answer 2

Dermatomyositis is typically distinguished from polymyositis by the presence of a distinct rash

Question 3

What must be present to Dx a pt with autoimmune myopathies

Answer 3

PROXIMAL muscle weakness must be present

Question 4

What are Gattrons Papules

Answer 4

Gattron’s papules: raised, violaceous lesions on the extensor surfaces
MC on MCP, PIP & DIP

KEY FINDING IN Dermatomyositis

Question 5

What is Gatrrons Sign

Answer 5

Gattron’s sign: erythematous rash involving extensor surfaces

Key finding in Dermatomyositis

Question 6

What is Heliotrope rash

Answer 6

Heliotrope rash: red or purplish discoloration of eyelids
This may appear as hyperpigmentation in dark skinned patients

KEY FINDING in Dermatomyositis

Question 7

Anti Jo-1 Antibodies are most prevalent in pts with myopathies and what other condition

Answer 7

20-30% of patients
Most likely in patients with interstitial lung disease
-ILD, Raynauds, non-erosive arthritis, mechanics hands: Antisynthetase syndrome

Question 8

What is the most sensitive lab finding in Autoimmune Myopathies

Answer 8

ANA

Question 9

What is the Bx requirement for all pts with an autoimmune myopathy

Answer 9

All patients in whom autoimmune myopathy suspected (unless pathognomonic findings of dermatomyositis found).
Deltoid or biceps with 4/5 strength.

Question 10

What is the 1st line treatment for pts with autoimmune myopathies

Answer 10

Prednisone 1-2 mg/kg/day for 3-4 weeks then tapper

May need rx for up to a year

Question 11

What is the threshold for immunosuppressants in the treatment of Autoimmune myopathies

Answer 11

(1) patients are experiencing complications from long-term glucocorticoid use
(2) the inability to taper glucocorticoid dose without precipitating a myositis flare
(3) ineffectiveness after 2–3 months of therapy!!
(4) rapidly progressive disease with respiratory failure

Question 12

What steroid sparing agent has shown benefit in pts with Dermatomyositis skin conditions

Answer 12

Mycophenolate mofetil

Question 13

What is the Rx for pts with myopathies and ILD

Answer 13

Clycophosphamide

Question 14

What is the oppurtunistic infection risk with long term steroid use

Answer 14

P jiroveci pneumonia