Inflammatory Myopathy/Myositis

Question 1

Inflammatory myopathy/Myositis: Definition

Answer 1

Autoimmune disease causing inflammation and degeneration of muscle (unknown cause)

Question 2

5 types of Myopathy

Answer 2

1. Adult polymyositis (PM)
2. Peddermatomyositis
3. Adult dermatomyositis (DM)- muscle + skin issues
4. Overlap syndrome- PM or DM + 1 other autoimmune dis.
5. Inclusion body myositis- early distal muscle weakness

Question 3

Who makes the diagnosis

Answer 3

Neurologist and rheumatologist

Question 4

Items involved in making the diagnosis

Answer 4

1. CPK- elevated (in the 1000s) –> degradation of muscle
2. Troponin levels (breakdown of heart muscle)
3. Muscle biopsy- seeing inflammatory cells (WBC) and muscle degradation
4. EMG- nerve conduction velocity is fine, but no muscle response (cannot recruit)
5. type specific antibodies (ANA in PM)

Question 5

Medical management

Answer 5

1. IVIG
2. high dose steroids
3. clinical drug trials: remicade
4. respiratory care
5. speech and swallowing

Question 6

PT impairments

Answer 6

1. strength
2. muscle pain (fatigued and broken down)
3. fatigue (unable to recruit all muscle fibers)
4. ROM limitations (hip + knee flexor tightness if non-ambulatory)
5. endurance

Question 7

Muscle fiber regeneration

Answer 7

the muscles do regenerate, but it ends up having a lot of scar tissue; loss of some muscles you cannot get back

Question 8

Particular Activity limitation

Answer 8

1. bed mobility
2. transfers
3. ambulation
   * all have to do with proximal weakness

Question 9

Clinical Features

Answer 9

1. symmetrical, proximal muscle weakness (myopathic, may include respiratory muscles)
2. Distal muscle weakness occurs late in dx (except in IBM)
   * if hand weakness within a couple weeks of dx, thinking IBM
3. Distinctive rashes (DM)
4. Cardiac involvement (CHF)- look at troponin levels

Question 10

Labs to check before seeing the Myositis pt

Answer 10

1. Troponin- make sure there is no cardiac ischemia
2. CPK- want these to be declining; if levels are elevating do not see them
3. WBC- clueing you in to if there is a new infection
4. PFTs- what is their respiratory status?

Question 11

Recovery rate (PM)

Answer 11

only 30% achieve full recovery (most have lingering functional deficits)

Question 12

5yr survival rate (PM and DM)

Answer 12

90% (other 10% die from respiratory issues)

Question 13

Long term complications of Corticosteroids

Answer 13

1. osteoporosis
2. avascular necrosis of the hip
3. Steroid myopathy

Question 14

Problems with the literature

Answer 14

Groups all neuromuscular dx together
comes from MD population
small number of pts included

Question 15

Strengthening: evidence from the literature

Answer 15

small numbers of sets and reps (3 sets of 10 at 5 R)

Rotate the muscles you’re working- avoid CPK elevations

Question 16

Aerobic Conditioning: evidence from the literature

Answer 16

exercise at 60% HRmax
no on had increased CPK levels
5 min bouts up to 30min 3x/wk
don’t do specific aerobics with pt in crisis (everything will be aerobic for them)

Question 17

Clinical suggestions for this population

Answer 17

1. Positioning devices/PROM exercises (maintain resting muscle length)
2. monitor blood markers for injury/inflammation
3. Target proximal musculature and respiration
4. Keep pts on program forever (lifestyle change)