Polymyalgia Rhuematica, Giant Cell Arteritis and Fibromyalgia

Question 1

Polymyalgia Rheumatica

1. Characterized by what?
2. Occurs most commonly in what age?
3. Usually responds well to what?
4. Is related to what disease?

Answer 1

1. Characterized by aching & stiffness in the shoulder and pelvic girdles and the neck
2. Occurs in people > 50 yrs old
3. Usually responds to low doses of steroids
4. Is related to Giant Cell Arteritis, with biopsy-proven GCA present in about 4-21%

Question 2

PMR
1. Incidence increases with age, peaks at _____yrs

2. Gender? in all age groups (2:1)
3. Higher incidence where?

Answer 2

1. 70-80
2. Females > Males
3. at higher latitudes, Scandinavian countries

Question 3

See Slide 50 for Dx criteria for PMR

Answer 3

50

Question 4

Possible infectious triggers for PMR:

1. Virus? 4
2. Bacteria? 2

Answer 4

1. Viruses:
   - adenovirus,
   - RSV,
   - parvovirus,
   - parainfluenza
2. Bacteria:
   - Mycoplasma,
   - Chlamydia pneumoniae

Question 5

PMR
1. What genes appear to be most associated with susceptibility to PMR? 2

2. Possible etiology?

Answer 5

1. • HLA-DRB1*04 and
   • DRB1*01
2. Possible subclinical vasculitis

Question 6

PMR
1. Clinical Manifestations: Pain for at least how long?

2. Aching & morning stiffness in neck, shoulder and pelvic girdles lasting how long?
3. Discomfort is
   - Unilateral or bilateral?
   - Worse with what?
   - Interferes with what?

Answer 6

1. 1 month
2. 30 min
3. • bilateral,
   • worse with movement, and
   • usually interferes wth ADLs.

Question 7

PMR Clinical Manifestations

1. Most common pain spot?
2. Other places? 2
3. Radiates where?
4. Systemic signs seen in 1/3. What are these? 3

Answer 7

1. Shoulder pain is presenting sign in 70-95%
2. Hips and neck 50-70%
3. Pain usually radiates distally towards elbows and knees
4. Systemic signs seen in 1/3:
   - Fever
   - Malaise/fatigue
   - Anorexia, weight loss

Question 8

PMR
1. Exam reveals little evidence of what?

2. MRI studies have shown what are more prominent than actual joint synovitis?

Answer 8

1. proximal joint swelling or tenderness

2. subdeltoid & subacromial bursitis

Question 9

PMR:
Distal manifestations also seen in ~1/2 cases. What are they?
3

Answer 9

1. Nonerosive, self-limiting, asymmetric peripheral arthritis (knee/wrist)
2. Carpal tunnel syndrome
3. Distal extremity swelling & pitting edema over dorsum of hands and wrists, ankles and feet

Question 10

LABs for PMR? 6

Answer 10

1. ESR > 40mm/hr (nl in up to 20%)
2. CRP less influenced by other factors, may be more sensitive & direct measure
3. Modest anemia of chronic disease in 2/3
4. Mildly abnormal LFTs in ~1/3
5. Rheum factor and ANA usually negative
6. CK and CPK enzymes are normal

Question 11

PMR Differential Diagnosis

1. SLE: Looks for? 2
2. SLE: Labs? 2
3. Check whats antibodies? 2

RA

1. Which joints?
2. Only partially responsive to what?
3. Considerable overlap b/t what? 2

Answer 11

SLE

1. Look for
   - pleuritis or
   - pericarditis
2. • Leukopenia or
   • thrombocytopenia
3. Check
   - anti-dsDNA and
   - anti-ENA antibodies

RA

1. Small joints of hands/feet
2. Only partially responsive to steroids
3. Considerable overlap b/t
   - PMR &
   - seronegative RA

Question 12

PMR Differential Diagnosis
Polymoisitis symptoms to differentiate it from PMR? 6

Other possible DDx? 2

Answer 12

1. Symmetric proximal muscle weakness
2. Pain not prominent
3. Elevated CK,
4. Alk Phos;
5. abnormal EMG,
6. myositis on muscle biopsy

• Fibromyalgia
• Late-onset spondyloarthropathy

Question 13

PMR Differential Diagnosis
1. Malignancy: What kinds? 2

2. Infectious: 1 example?
3. When would you consider these?

Answer 13

1. Malignancy
   -Solid (kidney, ovary, stomach)
   -Hematologic (myeloma, primary amyloidosis)
   Infection
2. Bacterial endocarditis
3. • Lack of adequate response to prednisone and presence of atypical features should make one consider these

Question 14

PMR Treatment & Course

1. DOC?
2. Trial of what if mild?
3. If they dont get better in a few days?
4. Reoccurrence?

Answer 14

1. Corticosteroids are drugs of choice (10-20mg/day) for 2-4 weeks, then taper off.
2. Trial of NSAIDs for 2-4 weeks if mild
3. Complete or nearly complete resolution of sx is seen in a few days - absence of improvement should cause one to question diagnosis.
4. Relapses do occur, more frequent in first 1-2 years.

Question 15

PMR: Treatment & Course

1. Follow what labs?
2. Treatment for how long?
3. Watch for what during tx?
4. Use what for refractory cases?

Answer 15

1. Follow ESR or CRP
2. Treatment for 1-2 years is often required, sometimes longer
3. Watch for corticosteroid adverse effects!
4. Methotrexate being used in refractory cases

Methylprednisolone had similar efficacy & fewer adverse effects

Question 16

GCA – Giant Cell Arteritis

1. What is it?
2. More common in what age?
3. Gender?

Answer 16

1. Chronic vasculitis of medium and large vessels
2. More common at age > 50, incidence peaks at 70-80
3. Affects women more often than men, 2:1

Question 17

PP of GCA: 4 steps

Symptoms are due to?

Answer 17

1. Vasculitis of extracranial branches of aorta, spares intracranial branches
2. Transmural inflammation->
3. intimal hyperplasia->
4. luminal occlusion

Symptoms are due to end-organ ischemia

Question 18

Possible etiology of GCA?

Answer 18

Cellular immune response involving T-cells, APCs, macrophages

Question 19

GCA Presentation

3

Answer 19

1. Onset usually gradual, but may be abrupt
2. Most frequent symptom: headache (2/3)
3. Usually accompanied by syndrome of systemic inflammation: fever, malaise, weight loss, anorexia (40%)

Question 20

GCA symptoms in order of prevalence? 8

Answer 20

1. Headache 68%
2. Jaw claudication 45%
3. Transient visual symptoms 16%
4. Fixed visual symptoms 14%
5. CNS Abnormalities 15%
6. Swallowing claudication/ dysphagia 8%
7. Tongue claudication 6%
8. Limb claudication 4%

Question 21

GCA Clinical Signs in order of prevalence? 7

Answer 21

1. Weight loss or anorexia 50%
2. Decreased temporal artery pulsations 46%
3. Fever 42%
4. Artery tenderness 27%
5. Erythematous or swollen scalp arteries 23%
6. Large artery bruits 21 %
7. Fundoscopic abnormalities 18%

Question 22

GCA: Complications

3

Answer 22

1. Blindness
2. Aortic aneurysms
3. Stroke

Question 23

Laboratory Findings
for GCA?
3

Answer 23

1. Erythrocyte sedimentation rate > 50
   - 22% of patients with biopsy-proven GCA have normal ESR
   - Therefore, normal ESR does NOT rule out GCA
2. Mild-moderate anemia of chronic disease
3. Elevated LFTS (1/3)

Question 24

Diagnosis for GCA:
1. What performed in all GCA?

2. Sensitivity?
3. NPV?
4. What increases the sensitivity?

Answer 24

1. Temporal artery biopsy should be performed in all patients with suspected GCA
2. Sensitivity of unilateral biopsy: 88%
3. Negative Predictive Value: 91%
4. Bilateral biopsies increase sensitivity, may be prudent

Question 25

GCA Diagnosis: Imaging 1. What could be used in large vessel GCA? 2. What else may it help? 3. Other possible imaging? 3

Answer 25

1. MRI/MRA may be used to diagnose large-vessel GCA. 2. Also may held guide biopsy by localizing inflammation. 3. Other possible imaging modalities include - arteriography, - ultrasound, and - PET.

Question 26

1. Treatment of GCA? 2. Prolonged treatment often required for what? 2. Vision loss present how do we treat? 3. What is reccommened to reduce ischemic events?

Answer 26

1. Glucocorticoids are the established treatment Initial dose: 40-60 mg prednisone daily Slow taper after 2-4 weeks if symptoms controlled 2. Prolonged treatment often required to avoid relapse 3. IV pulse methylprednisolone (1000 mg IV daily x 3 days) if vision loss present 4. Anti-platelet agents reduce ischemic events-> low dose aspirin is recommended

Question 27

1. If clinical suspicion is high, treatment should not be what? 2. Temporal artery biopsies should be performed as soon as possible after treatment is initiated, but biopsy results should not be affected for what?

Answer 27

1. NOT be delayed for biopsy | 2. several weeks

Question 28

What is Fibromyalgia Syndrome (FMS)?

Answer 28

A clinical syndrome characterized by widespread muscular pain (usually chronic), fatigue and muscle tenderness.

Question 29

FMS 1. Not associated with? 2. Caused by? 3. Usually extremely sensitive to what?

Answer 29

1. Not an inflammatory condition. 2. Caused by abnormal sensory processing in the central nervous system. 3. People with fibromyalgia may be extremely sensitive to pain and other unpleasant sensations.

Question 30

FMS: Additional symptoms are common and include? 11 Symptoms including pain may wax and wane over time

Answer 30

1. poor sleep (almost always) 2. headaches 3. irritable bowel syndrome 4. cognitive and memory problems 5. numbness and tingling in fingers and toes 6. irritable bladder 7. temporomandibular joint (TMJ) disorder 8. restless leg syndrome 9. dry eyes and dry mouth 10. morning stiffness 11. anxiety and depression Symptoms including pain may wax and wane over time

Question 31

FMS 1. Abnormally high levels of __________ in spinal fluid in some patients 2. __________ important in transmission and amplification of pain signals to and from brain 3. "Volume control" is turned up too high in what?

Answer 31

1. Substance P 2. Substance P 3. brain’s pain centers

Question 32

FMS 1. Genetic? 2. Can be triggered by physical, emotional or environmental stressors such as what? 3 3. Patients with what are more likely to develop FMS? 2

Answer 32

1. Familial tendency to develop FMS suggests genetic role 2. - car accidents, - repetitive injuries and - certain diseases 3. - Rheumatoid arthritis and - SLE (Lupus)

Question 33

FMS 1. Other conditions such as what have been associated with FMS? 2 2.What is associated with day-time fatigue and FMS?

Answer 33

1. - Lyme disease and - obstructive sleep apnea (OSA) 2. Sleep deprivation with disruption of delta-wave sleep (non-REM stage IV)

Question 34

FMS Most common population? Higher incidence in which dzs? 3

Answer 34

1. Most common in middle-aged women - Men and children may also develop the disorder 2. Patients with - RA, - SLE and - Ankylosing spondylitis have a higher incidence

Question 35

Fibromyalgia Syndrome Diagnosis | 4

Answer 35

1. Widespread pain (axial + upper and lower + L and R sides) 2. 11 of 18 reproducible tender points 3. At least 4 of: generalized fatigue, headache, sleep disturbance, neuropsych complaints, numbness/tingling, IBS 4. Explained by no other condition

Question 36

FMS Dx 1. Generalized chronic pain where? 3 2. At least 4 of the following symptoms? 6

Answer 36

Generalized, chronic pain (≥ 3 months) affecting the - axial, - plus upper and lower segments, - plus left and right sides of the body At least 4 of the following symptoms 1. Generalized fatigue 2. Headaches 3. Sleep disturbance 4. Neuropsychiatric complaints 5. Numbness, tingling sensations 6. Irritable bowel symptoms

Question 37

Shared Features of FM and Depression: Clues to Pathophysiology 8

Answer 37

1. Both have strong genetic predisposition and similar co-morbidity 2. Similar sleep disturbances 3. Similar cognitive disturbances 4. Orthostatic features, ANS dysfunction 5. Childhood abuse, stress 6. Can be debilitating 7. Imaging studies - unremarkable 8. Neuroendocrine studies - unremarkable

Question 38

Labs or Dx for FMS?

Answer 38

X-rays, blood tests, specialized scans such as nuclear medicine and CT, muscle biopsies are all normal Objective “markers of inflammation” such as ESR, CPR are normal Must be distinguished from other common diffuse pain conditions such as RA, SLE, Hypothyroidism and Polymyalgia Rheumatica (PMR)

Question 39

Which two drugs classes are often prescribed for FMS?

Answer 39

Medication trial - antidepressants, - anticonvulsants

Question 40

Current studies suggest that the best pharmacologic treatment for treating pain and improving sleep disturbance includes: 3

Answer 40

1. Tricyclic compounds such as: 2. Anticonvulsants 3. Dual reuptake inhibitors (SNRIs) such as:

Question 41

Name the two drugs in each of the following categories given: 1. Tricyclic compounds such as: 2 2. Anticonvulsants: such as? 2 3. Dual reuptake inhibitors (SNRIs) such as: 2

Answer 41

1. - Amitriptyline (Elavil), 25-50 mg at bedtime - Cyclobenzaprine (Flexeril), 10-30 mgs at bedtime 2. - Pregabalin(Lyrica), 300-450 mg/day - Gabepentin (Neurontin), 1600-2400 mg/day 3. - Duloxetine (Effexor), 60-120 mg/day - Milnacipran (Savella), 100-200mg/day

Question 42

FMS Effectiveness of: 1. NSAIDS? 2. Narcotics? 3. Benzos? 2

Answer 42

1. NSAIDs (non-steroidal anti-inflammatory drugs) such as ibuprofen and naproxen are generally ineffective 2. Long acting opioids (narcotics) generally are not of great benefit either 3. Benzodiazepines such as diazepam (Valium) and clonazepam (Klonipin) may be useful for patients with - restless leg syndrome or - very severe sleep disturbance who have not responded to other therapies

Question 43

As a first-line approach for patients with moderate to severe pain, trial with evidence-based medications for example: 4

Answer 43

Trial with 1. low-dose tricyclic antidepressants, 2. SSRI, 3. SNRI, 4. antiseizure medication

Question 44

Complementary and alternative therapies have been used although not well studied in FMS 3

Answer 44

- Therapeutic massage - Myofascial release therapy - Acupuncture