Download FM-ELAR2015

Prof.Mohamed Elwy
Professor of Rheumatology and
physical medicine
Ain Shams University
Prof.Mohamed Elwy AinShams U. 2015
Soft Tissue
(Non-articular)
Rheumatism
Prof.Mohamed Elwy AinShams U., 2015
Soft-tissue rheumatic disorders causing pain
outside joints may be classified as follows:
1. Generalized rheumatic pain due to systemic disorders.
2. Tendinitis and tenosynovitis.
3. Enthesopathies.
4. Bursitis.
5. Capsulitis.
6. Myositis.
7. Muscle cramps.
8. Fasciitis.
9. Fibromyalgia.
10. Panniculitis.
11. Neuritis.
12. Soft-tissue injury.
The Normal Pain Processing Pathway
3. A signal is sent via
the ascending tract
to the brain, and
perceived as pain
Pain
Perceived
4. The descending tract carries
modulating impulses back to
the dorsal horn
2. Impulses from afferents
depolarize dorsal horn
neurons, then, extracellular
Ca2+ diffuse into neurons
causing the release of Pain
Associated
Neurotransmitters –
Glutamate and Substance P
1. Stimulus sensed by
the peripheral
nerve (ie, skin)
1. Staud R and Rodriguez ME. Nat Clin Pract Rheumatol. 2006;2:90-98.
2. Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1984.
Glutamate
Substance P
4
Central Sensitization: A Theory for Pain
Amplification in FM
 Central sensitization is believed to be an underlying cause of the
amplified pain perception that results from dysfunction in the CNS1
 May explain hallmark features of generalized heightened pain sensitivity2


Hyperalgesia – Amplified response to painful stimuli
Allodynia - Pain resulting from normal stimuli
 Theory of central sensitization is supported by:
 Increased levels of pain neurotransmitters3,4


Glutamate
Substance P
 fMRI data demonstrates low intensity stimuli in patients with FM
comparable to high intensity stimuli in controls5
fMRI = functional magnetic resonance imaging
1. Staud R and Rodriguez ME. Nat Clin Pract Rheumatol. 2006;2:9098.
2. Williams DA and Clauw DJ. J Pain. 2009;10(8):777-791.
3. Sarchielli P, et al. J Pain. 2007;8:737-745.
4. Vaerøy H, et al. Pain. 1988;32:21-26.
5. Gracely RH, et al. Arthritis Rheum. 2002;46:1333-1343.
5
Central Sensitization Produces Abnormal
Pain Signaling
Perceived pain
Ascending
input
Perceived pain
(hyperalgesia/allodynia)
Pain
amplificatio
n
After nerve injury, increased input to the
dorsal horn can induce central sensitization
Nerve dysfunction
Descending
modulation
Nociceptive afferent
Induction
fiber of central
sensitization
Increased release of pain
neurotransmitters
glutamate and substance P
Minima
l
stimuli
Increased pain perception
1. Adapted from Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1984.
2. Woolf CJ. Ann Intern Med. 2004;140:441-451.
6
FM: An Amplified Pain Response
Subjective pain intensity
10
8
Pain in
FM
Hyperalgesia
6
(when a pinprick causes an
intense stabbing sensation)
4
Allodynia
Pain
amplification
response
Normal
pain
response
(hugs that feel painful)
2
0
Stimulus intensity
Adapted from Gottschalk A and Smith DS. Am Fam Physician. 2001;63:1979-1986.
7
2.5%
Why FM is important:
Very Common:
*2-8% of the general population(Clauw and Daniel, 2014).
*It is the 2nd most common Rhic. Dis. after OA.
Clauw, Daniel J.
(16 April 2014).
"Fibromyalgia".
JAMA 311 (15):
1547.
General population
25%
+ve ANA
Misdiag.:
*25% of patients referred to Rheuma.Clinics
with +ve ANA had FMS.
25%
Other speciality:
In FM, anxiety/depression are present in ~25% of patients.
FM
('Fibromyalgia Syndrome')
(Fibrositis)
ACR Rheumatol
Patient presentation:
Soft-tissue pain and tenderness
+
Sometimes with little palpable lumps
Particularly in the neck and back regions
(These are areas of pain but without redness, swelling nor hotness)
Clinical Feature:
Exaggerated tenderness of normal tender points'.
Tender point Exam:
In normal subjects,
 uncomfortable to firm pressure,
But in fibromyalgia patients,
 produce wince or withdrawal.
The degree of pressure is important. “Dolorimeter” =
“Standard pressure spring device” is ideal, but reasonable
palpation (at 4kg) is enough for clinical exam.
‫ مؤلمين ويمكن‬18‫ نقطة أو أكثر من ال‬11 ‫ولتشخيص المرض يكون‬
.‫استعمال الضغط اليدوى حتى يحدث ابيضاض لجلد األظافر‬
Physical Exam Requirement
Systematic palpation of
the 18 tender point sites
Palpation force is 4 kg or
equal to the force
needed to just blanch
your thumbnail
Clue to diagnose
&
Associated diagnostic findings
Clue to diagnose:
Fibromyalgia affects patients at 40s or 50s.
Female preponderance (7/1 to 9/1)
Prof.Mohamed Elwy AinShams U., 2015
(Hawkins, Sept.2013).
Associations of pain-related conditions among patients diagnosed with FM in
the DMBA database between 1997 and 2002
Patients With FM are More Likely to Have
Concomitant Chronic Pain Conditions
Female
7
Risk ratio ‡
6
Male
FM Patients
Female
n=906
Male n=1689
5
Baseline†
4
3
2
1
0
SLE
RA
IBS
Headache*
• 20% of patients with SLE, RA and OA have concomitant FM2
• Because patients with FM are often diagnosed with other pain-related conditions, FM may go undetected
DMBA = Deseret Mutual Benefits Administration
SLE = Systemic lupus erythematosus; RA = Rheumatoid Arthritis; IBS = Irritable Bowel Syndrome
*Headache = headache, tension headache, migraine
†Baseline from 52,698 females and 52,232 males without FM
‡Risk ratio = The probability of each condition occurring as compared to a normal, healthy control group
1.(baseline=1)
Weir PT, et al. J Clin Rheumatology. 2006;12(3):124-128.
15
2. Wolfe F and Rasker JJ. Fibromyalgia. In: Firestein, ed. Kelly’s Textbook of Rheumatology, 8th Edition. St. Louis, MO: WB Saunders Co; 2008.
Associated diagnostic findings
Pain
Day&
Night
Other Systems
Psychol-
Associated diagnostic findings
1. Local tender points at several sites
(NB: normal sleep-deprived patients exhibit tender points).
Pain
#. Negative control sites
(non-tender), such as
forehead, distal forearm
and lateral fibular head.
Associated diagnostic findings
1. Local tender points at several sites
(NB: normal sleep-deprived patients exhibit tender points).
Pain
#. Negative control sites
(non-tender), such as
forehead, distal forearm
and lateral fibular head.
Day&
Night
Associated diagnostic findings
1. Local tender points at several sites
(NB: normal sleep-deprived patients exhibit tender points).
Pain
@. Negative control sites
(non-tender), such as
forehead, distal forearm
and lateral fibular head.
8. Headache: Occipital
and bifrontal. & migrain
9. Irritable and weepy. &
Chronic Depression
10. Poor concentration
11- Can not cope with a
and forgetfulness.
job or household activities
Psychol-
Day&
Night
Associated diagnostic findings
1. Local tender points at several sites
(NB: normal sleep-deprived patients exhibit tender points).
Pain
Day&
Night
#. Negative control sites
(non-tender), such as
forehead, distal forearm
and lateral fibular head.
Other Systems
8. Headache: Occipital
and bifrontal. & migrain
9. Irritable and weepy. &
Chronic Depression
10. Poor concentration
11- Can not cope with a
and forgetfulness.
job or household activities
Psychol-
12. Urinary & GIT:
-Urinary: Nocturnal
freq..& urgency.
-Abdominal diffuse pain
& irritable bowel $.
- Dysmenorrhoea,
Qualitative Studies in FMS
Physical Domain
Pain
Fatigue
Disturbed sleep
Emotional/Cognitive Domains
Depression, anxiety
Cognitive impairment (decreased concentration, disorganization)
Memory problems
Social Domain
Disrupted family relationships
Social isolation
Disrupted relationships with friends
Work/Activity Domains
Reduced activities of daily living
Reduced leisure activities/avoidance of physical activity
Loss of career/inability to advance in career or education
Arnold, L., Crofford, L., Mease, P., Burgess, S., Palmer, S., Abetz, L., Martin, S. (2008). Patient perspectives on the impact of
fibromyalgia. Patient Education and Counseling 73: 114-120.
Qualitative Studies in FMS
100
80
60
FMS Patients
Non-FMS Patients
40
20
0
Memory Decline
Mental Confusion
Speech Difficulty
Katz, R., Heard, A., Mills, M., Leavitt, F. (2004). The prevalence and clinical impact of reported cognitive difficulties (fibrofog) in
patients with rheumatic disease with and without fibromyalgia. Journal of Clinic Rheumatology 10(2): 53-58.
Interaction between Symptoms
and Function in FM
Psychological
and Behavioral
Consequences
“STRESS”
GENES
Symptoms
• Decreased activity
• Poor sleep
ENVIRONMENT
• Increased distress
• Maladaptive illness
behaviors
Overlap between Fibromyalgia and Other
“Systemic” Syndromes:
Chronic Multi-symptom Illnesses
FIBROMYALGIA
2 - 4% of population;
defined by widespread
pain and tenderness
EXPOSURE
SYNDROMES e.g.
Gulf War Illnesses, silicone
breast implants, sick building
syndrome
MULTIPLE CHEMICAL
SENSITIVITY - symptoms in
multiple organ systems in
response to multiple
substances
CHRONIC FATIGUE
SYNDROME 1% of
population; fatigue and
4/8 “minor criteria”
SOMATOFORM
DISORDERS 4% of
population; multiple
unexplained
symptoms - no
organic findings
Inclusion criteria
 To be eligible veterans had have been deployed to the Gulf War between August
1990 and August 1991, and to endorse > 2 of the following symptoms:
fatigue limiting usual activity
 pain in > 2 body regions
 neurocognitive symptoms

 These symptoms had to begin after August 1990, last for more than six months,
and be present at the time of screening.
Differential
Diagnosis
Prof.Mohamed Elwy AinShams U., 2015
You Should Exclude
Other Similar/s
Their C/P
1- Referred pain
Usually from cervical, thoracic or lumbar spine. (FM is a
primary soft-tissue disease).
2- Acute infection &
Influenza:
Diffuse muscle pain (which is sometimes termed
'fibrositis') is a common event during acute infections.
3- Tension State:
EMG shows continuous muscle spasm.
4- Hypothyroidism:
gives also sleep disturbance.
5- SLE
6- Chronic Fatigue $, 7- PMR,
8- DM
9- Osteomalacia & Hyperparathyroidism
10- Fabrication/
Psychogenic
More likely if a patient claims to be tender all over
Also; is it
OR
1. Primary FM:
(fibromyalgia syndrome).
2. Secondary FM:
(in 20% of RA & SLE; hypothyroidism. etc.).
Hypothesis of
FM pathogenesis
Prof.Mohamed Elwy AinShams U., 2015
Many Theories:
1- Deficiency of serotonin: (or its precursor, tryptophan).
2- High substance P: increase production of substance P in the afferent neurons
 increase pain awareness and sensitivity.
3- HPA dysfunction: Hypothalamic-pituitary adrenal axis (HPA) dysfunction, and
other abnormalities of the “NeuroEndocrinal Axis”.
4- Muscle mitochondrial dysfunction & oxidative stress.
5- Viral etiology: Was reported in patients with chronic fatigue $, but in FM is
lacking.
6- Life style and chronic stress.
7- Sympathetic hyperactivity.
8- Dopamine dysfunction: Some fibromyalgia patients responded in controlled
trials to pramipexole, a dopamine agonist that selectively stimulates dopamine
D2/D3 receptors and is used to treat both Parkinson's disease and restless leg
syndrome.
Many Theories (Cont.):
10- Sleep disturbance: This explain both the sleep disorder and pain associated
with fibromyalgia.
(due to poor relaxation at night) involving alpha-wave intrusion into Stage IV (non-REM) deep sleep.
Local nodule
Exam
Prof.Mohamed Elwy AinShams U., 2015
Local Gross Exam.  “Fibrositic” nodules:
- Are small, tender lumps which may appear at or near sites
of pain.
- These ('trigger areas') often correspond to ‘acupuncture
points‘.
- Sometimes, but not always, lies on the 'classical Chinese
meridians'.
*******************
Local Histological Exam. 
 (1) Herniation of oedematous fat through fascia.
 (2) Small round-cell infiltration.
 (3) Type II Muscle atrophy+ fibre necrosis+ lipid
accumulations.
Diagnostic
Criteria
(a) ACR (1990)
(b) ACR (May,2010)
(a) ACR (1990)
(a) ACR (1990) Diagnostic Criteria for FM:
1) Widespread pain
- Rt side.
- +Lt side.
- +Above waist.
- +Below waist.
For >3months
2) Tenderness in >11 point of 18.
Prof.Mohamed Elwy AinShams U., 2015
+
Axial skeletal pain
(C-spine,
ant.chest,
thoracic spine
and LBP).
Common tender points in fibrositic syndrome (n=9, total score = 18),
if >11 (simultaneously, or sequentially), it is diagnostic
--------------------------
X
2
1- Occipital.
2- Mid-trapezius = Supraspinatus
(above spine of scapula).
3- 2nd costochondral junctions.
4- Medial border of scapula.
5- Lateral epicondyles (maximum tenderness
1-2cm distal to epicondyles).
6- Gluteus (upper outer quadrants of buttocks).
7- Greater trochanter.
8- Medial fat pads of knees
9- Low cervical (C4-6 interspinous lig.).
& Lower lumber spine (L4-S1 interspinous lig.).
--------------------------------------------
ACR-Recommended Manual Tender Point
Survey* for the Diagnosis of FM
LOW CERVICAL –
Anterior aspects of C5,
C7 intertransverse
spaces
TRAPEZIUS –
Upper border of trapezius,
midportion
OCCIPUT –
At nuchal muscle
insertion
FOREHEAD
SUPRASPINATUS –
SECOND RIB SPACE –
At attachment to medial
border of scapula
about 3 cm lateral to
sternal border
RIGHT
FOREARM
ELBOW –
Muscle attachments to
Lateral Epicondyle
GLUTEAL –
Upper outer quadrant of
gluteal muscles
KNEE –
Medial fat pad of
knee proximal to
joint line
LEFT
THUMB
GREATER
TROCHANTER –
Muscle attachments
just posterior to GT
Manual Tender Points Survey:
• Presence of 11 tender points on palpation to a maximum of 4 kg
of pressure (just enough to blanch examiners thumbnail)
*Based on 1990 ACR FM Criteria
1. Adapted from Chakrabarty S and Zoorob R. Am Fam Physician.
2007;76(2);247-254.
Control Points
Tender Points
37
Fibromyalgia Syndrome (FMS)
 “ACR” Diagnostic Criteria
 Widespread pain lasting ≥ 3 months
 11 positive tender points out of possible 18 using 4 kg of palpation
 Occiput
 Low cervical
 Trapezius
 Supraspinatus
 Second rib
 Lateral epicondyle
 Gluteal
 Greater trochanter
 Knee
Wolfe F, Smythe HA, Yunus MB, Bennett RM, Bombardier C, Goldenberg DL, et al. (1990) The American College of Rheumatology 1990
criteria for the classification of fibromyalgia: report of the multicenter criteria committee. Arthritis Rheum 33:160–72.
(b) ACR (May, 2010) NewCriteria
(b) New Criteria:
(1) WPI (Widespread Pain Index) Scale:
•Lists 19 areas of the body and you say where you've had
pain in the last week.
•You get 1 point for each area, so the score is 0-19.
(2) SS Scale:
Symptom Severity Scale
Zero
1
(1) Fatigue
a
(2) Waking Un-refreshed
(3) Cognitive symptoms
b
(4)Somatic (Physical) Symptoms
(Headache, weakness, bowel problems, nausea,
dizziness, numbness/tingling, hair loss).
The numbers assigned to each are added up, for a total of 0-12.
2
3
For a diagnosis you need I+II+III
(I)
WPI of at least 7
And
SS score of at least 5,
OR
WPI of 3-6
And
SS score of at least 9
(II)
Symptoms have persisted at this level for the
past 3 months.
(III)
Patient does not have any other disorder or
cause to explain the pain.
Determining What’s Wrong
How is FM diagnosed?
 Symptoms of FM
are typically very
non-specific,
common to many
other conditions.
Many Symptoms cannot
be objectively
evaluated.
Value of the New Criteria:
1- Eliminate the use of tender point exam.
2- It allows people with fewer painful areas but more
severe symptoms to be diagnosed.
3- It includes cognitive symptoms.
4- It recognizes the difference between "fatigue" and
"waking un-refreshed”.
5- Some flexibility is built in, which recognizes the
fact that fibromyalgia impacts patients differently, and
that symptoms can fluctuate.
6- Better sensitivity and accuracy than the 1990
criteria.
Prof.Mohamed Elwy AinShams U., 2015
Investigations
FM is a diagnosis of
Exclusion
CBC
ESR
Blood
Sugar
CPK
ALT &
AST
RF;
ANA
HCV
Done : Either done to: Prove 1ry FMS ( being all normal ),
OR: To diagnose 2ry FM.
Treatment
Who treat Fibromyalgia?
Since the 1990s, fibromyalgia primarily has been treated by rheumatologists.
Even so, it can be difficult to find a doctor who's willing and able to effectively
diagnose and treat fibromyalgia.
Even so, it can be difficult to find a doctor who's willing and able to effectively
diagnose and treat fibromyalgia.
Controversies ?
It’s not a real illness, it’s in the
“patient’s head”
A real condition with severe physical effects in some,
although psychologic factors including depression
may be the major determinant of pain in others
Controversies ?
The prognosis is “hopeless”
Early, aggressive treatment can prevent physical
deconditioning and loss of function
Only 3 Meds are FDA Approved
for FM
 Duloxetine (Cymbatex)
 Pregabalin (Pregavalex)
 Milnacipran (Savella)
Treatment
1- Reassurance: It is not d.t. organic lesion.
2- Family interview: Include family member to enquire about life events, family
problems.
3- Physiotherapy: Various physiotherapy modalities are prescribed for FM.
a) TENS (50-100Hz) .
b) Heat and Cold.
c) Biofeedback.
d) Graded exercise program: Titrated up to 30min most days of the week. To
increase aerobic fitness. It may initially exacerbate symptoms.
e) Stretching Ex.: By Spray and stretch technique. Use topical anesthetic or ice then
stretch the involved muscle passively.
f) Relaxation training Ex.: Diaphragmatic breathing Ex., self hypnotherapy.
g) Massage by lidocaine: To tender point.
h) Acupuncture+ Local anesthetic injection: Can be combined to give better result.
4- Psychological Support: Group psychotherapy. Education.
Treatment
5- Medications:
a) Analgesics (Acetaminophen) and NSAIDs: Are ineffective and may even worsen
symptoms. Gastropathy, liver and renal side effects limit their continuous use.
****************************
b) Hypnotics: Including Benzodiazepines has no role in ttt. Only relief anxiety.
****************************
c) Opioid: Should be avoided
They usually give good results, but addiction and tolerance limit their use.
* Morphine sulphate (15-30mg, /4hours)
* Codeine (30-60mg/6hours).
* Tramadol (50-100mg/6hours).
Action: They bind to pre and post synaptic terminals of peripheral sensory
fibers and inhibit release of substance p and other mediators (In addition Tramadol inhibits
reuptake of NE and Serotonin).
Side Effect: They all may cause: Nausea, vomiting constipation, miosis myoclonus, urine
retention & respiratory depression.
Treatment
5- Medications (Cont.):
d) Tricyclic Antidepressants (such as amitriptyline 25-75mg) have beneficial effect.
And
e) Cyclobenzapine (a muscle relaxant with no antidepressant effect) is also effective.
**********************
f) Serotonin-norepinephrine reuptake inhibitors (SNRIs) :
Duloxetine (R/ Cymbatex 30 then 60mg\day after lunch) and
Milnacipram (R/Effexor) are FDA approved for use in FM.
Side effect: Nausea, expensive, long term use (3-6months to stabilize,
often there are relapses.
g) Pregabalin (R/ Pregavalex; 75-300mg/day). Effective, but expensive. It binds to Ca
channels on nerves and modify the release of neurotransmittors. Stopped if the
patient C/O of weight gain, drowsiness.
h) Gabapentin: Also effective
i) Selective serotonin reuptake inhibitors (SSRI): Are equivocal. Citalopram
(R/Cipram).
Treatment
5- Medications (Cont.):
J) Steroids: May help in regional pain $, but its chronic use limit their use.
k) B12 IM + Mg-Sulphate: Useful against pain, insomnia and low energy.
If these drugs are ineffective after a trial of 4-6weeks, further drug ttt should be
avoided.
Treatment
6- Coping strategies: Such as meditational yoga.
7- Cognitive behavioral therapy.
8- Vocational therapy:
Motivation to return to his work, reduce functional impairment. Improve coping
strategies
9- Diet and Nutrition:
High fiber diet;
4-5 small meals/day;
avoid processed food.
Avoid caffeine and alcohol.
Drink fresh juice, herbal tea and plenty liquids.
Calcium and magnesium supplement to improve muscle/nerve function.
10- Avoid further unnecessary investig. and drug ttt.
What about Diet?
 No “magic” diet
 No controlled studies, but …
 May suggest avoidance of foods associated with fatigue :
 High fat
 Refined sugar
 White flour
 Fried foods
“Junk” food
Caffeine
Salt
Alcohol
Prognosis
Prof.Mohamed Elwy AinShams U., 2015
Explaining the Typical
Outcome






FM does not herald a systemic disease
No progressive, structural or organ damage
Most patients in specialty practice have chronic, persistent symptoms
Primary care patients more commonly report complete remission of symptoms
Most patients continue to work, but 10-15% are disabled
Most patients’ quality of life improves with medical management
FM and Prognosis
 Patients treated in primary care settings and those with
recent onset of symptoms generally have a
better prognosis
 Longer-term studies needed
to define prognostic factors
Prognosis
 With resolution of sleep disturbance, may resolve totally
 Aggressive physical therapy is critical in those who do not respond
 Approximately 5% do not respond to any form of therapeutic
intervention.
Hypnosis may be attempted in that group.
Thank You
Prof.Mohamed Elwy AinShams U., 2015