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Rheumatology > Polymyalgia Rhuematica, Giant Cell Arteritis and Fibromyalgia > Flashcards

Polymyalgia Rhuematica, Giant Cell Arteritis and Fibromyalgia Flashcards

1
Q

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?
A
  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%
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2
Q

PMR
1. Incidence increases with age, peaks at _____yrs

  1. Gender? in all age groups (2:1)
  2. Higher incidence where?
A
  1. 70-80
  2. Females > Males
  3. at higher latitudes, Scandinavian countries
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3
Q

See Slide 50 for Dx criteria for PMR

A

50

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4
Q

Possible infectious triggers for PMR:

  1. Virus? 4
  2. Bacteria? 2
A
  1. Viruses:
    - adenovirus,
    - RSV,
    - parvovirus,
    - parainfluenza
  2. Bacteria:
    - Mycoplasma,
    - Chlamydia pneumoniae
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5
Q

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

  1. Possible etiology?
A
    • HLA-DRB1*04 and
    • DRB1*01
  2. Possible subclinical vasculitis
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6
Q

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

  1. Aching & morning stiffness in neck, shoulder and pelvic girdles lasting how long?
  2. Discomfort is
    - Unilateral or bilateral?
    - Worse with what?
    - Interferes with what?
A
  1. 1 month
  2. 30 min
    • bilateral,
    • worse with movement, and
    • usually interferes wth ADLs.
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7
Q

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
A
  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
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8
Q

PMR
1. Exam reveals little evidence of what?

  1. MRI studies have shown what are more prominent than actual joint synovitis?
A
  1. proximal joint swelling or tenderness

2. subdeltoid & subacromial bursitis

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9
Q

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

A
  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
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10
Q

LABs for PMR? 6

A
  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
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11
Q

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
A

SLE

  1. Look for
    - pleuritis or
    - pericarditis
    • 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
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12
Q

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

Other possible DDx? 2

A
  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
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13
Q

PMR Differential Diagnosis
1. Malignancy: What kinds? 2

  1. Infectious: 1 example?
  2. When would you consider these?
A
  1. Malignancy
    -Solid (kidney, ovary, stomach)
    -Hematologic (myeloma, primary amyloidosis)
    Infection
  2. Bacterial endocarditis
    • Lack of adequate response to prednisone and presence of atypical features should make one consider these
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14
Q

PMR Treatment & Course

  1. DOC?
  2. Trial of what if mild?
  3. If they dont get better in a few days?
  4. Reoccurrence?
A
  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.
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15
Q

PMR: Treatment & Course

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

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16
Q

GCA – Giant Cell Arteritis

  1. What is it?
  2. More common in what age?
  3. Gender?
A
  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
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17
Q

PP of GCA: 4 steps

Symptoms are due to?

A
  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

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18
Q

Possible etiology of GCA?

A

Cellular immune response involving T-cells, APCs, macrophages

19
Q

GCA Presentation

3

A
  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%)
20
Q

GCA symptoms in order of prevalence? 8

A
  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%
21
Q

GCA Clinical Signs in order of prevalence? 7

A
  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%
22
Q

GCA: Complications

3

A
  1. Blindness
  2. Aortic aneurysms
  3. Stroke
23
Q

Laboratory Findings
for GCA?
3

A
  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)
24
Q

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

  1. Sensitivity?
  2. NPV?
  3. What increases the sensitivity?
A
  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
25
Q

GCA Diagnosis: Imaging

  1. What could be used in large vessel GCA?
  2. What else may it help?
  3. Other possible imaging? 3
A
  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.
26
Q
  1. Treatment of GCA?
  2. Prolonged treatment often required for what?
  3. Vision loss present how do we treat?
  4. What is reccommened to reduce ischemic events?
A
  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
27
Q
  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?
A
  1. NOT be delayed for biopsy

2. several weeks

28
Q

What is Fibromyalgia Syndrome (FMS)?

A

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

29
Q

FMS

  1. Not associated with?
  2. Caused by?
  3. Usually extremely sensitive to what?
A
  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.
30
Q

FMS: Additional symptoms are common and include? 11

Symptoms including pain may wax and wane over time

A
  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

31
Q

FMS
1. Abnormally high levels of __________ in spinal fluid in some patients

  1. __________ important in transmission and amplification of pain signals to and from brain
  2. “Volume control” is turned up too high in what?
A
  1. Substance P
  2. Substance P
  3. brain’s pain centers
32
Q

FMS
1. Genetic?

  1. Can be triggered by physical, emotional or environmental stressors such as what? 3
  2. Patients with what are more likely to develop FMS? 2
A
  1. Familial tendency to develop FMS suggests genetic role
    • car accidents,
    • repetitive injuries and
    • certain diseases
    • Rheumatoid arthritis and
    • SLE (Lupus)
33
Q

FMS
1. Other conditions such as what have been associated with FMS? 2

2.What is associated with day-time fatigue and FMS?

A
    • Lyme disease and
    • obstructive sleep apnea (OSA)
  2. Sleep deprivation with disruption of delta-wave sleep (non-REM stage IV)
34
Q

FMS
Most common population?

Higher incidence in which dzs? 3

A
  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
35
Q

Fibromyalgia Syndrome Diagnosis

4

A
  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
36
Q

FMS Dx
1. Generalized chronic pain where? 3

  1. At least 4 of the following symptoms? 6
A

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
37
Q

Shared Features of FM and Depression: Clues to Pathophysiology

8

A
  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
38
Q

Labs or Dx for FMS?

A

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)

39
Q

Which two drugs classes are often prescribed for FMS?

A

Medication trial

  • antidepressants,
  • anticonvulsants
40
Q

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

A
  1. Tricyclic compounds such as:
  2. Anticonvulsants
  3. Dual reuptake inhibitors (SNRIs) such as:
41
Q

Name the two drugs in each of the following categories given:
1. Tricyclic compounds such as: 2

  1. Anticonvulsants: such as? 2
  2. Dual reuptake inhibitors (SNRIs) such as: 2
A
    • Amitriptyline (Elavil), 25-50 mg at bedtime
    • Cyclobenzaprine (Flexeril), 10-30 mgs at bedtime
    • Pregabalin(Lyrica), 300-450 mg/day
    • Gabepentin (Neurontin), 1600-2400 mg/day
    • Duloxetine (Effexor), 60-120 mg/day
    • Milnacipran (Savella), 100-200mg/day
42
Q

FMS Effectiveness of:

  1. NSAIDS?
  2. Narcotics?
  3. Benzos? 2
A
  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
43
Q

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

A

Trial with

  1. low-dose tricyclic antidepressants,
  2. SSRI,
  3. SNRI,
  4. antiseizure medication
44
Q

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

A
  • Therapeutic massage
  • Myofascial release therapy
  • Acupuncture

Rheumatology (11 decks)

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