IN CONCLUSION: CRPS is a chronic debilitating painful condition There has been significant advances in our understanding of its Pathophysiology Early diagnosis and management – is essential to help patients and reduce suffering The Budapest Criteria should help while excluding others A Multidisciplinary Approach to Management has been shown to be beneficial With particular emphasis on Patient Education and Support

1. Complex Regional Pain Syndrome
(CRPS)
Ramani Vijayan
University Malaya Medical Centre
Kuala Lumpur
Malaysia

2. CRPS
 Is a debilitating painful condition in a limb
 Associated with sensory, motor, autonomic, skin and
bone abnormalities
 PAIN is the leading symptom
 Often associated with limb dysfunction
 Psychological Distress

3.  It commonly arises after injury to a limb (sometimes
trivial)
CRPS
Type 2Type 1
Absence of a major nerve
lesion
Presence of a major nerve
lesion
(Reflex sympathetic dystrophy
Sudeck’s Atrophy )
(Causalgia)

4. Historical Background
 First described by Weir Mitchell after the American Civil
War in 1872
 When he encountered soldiers who were injured by gun-shot
wounds exhibiting “bizarre” symptoms and coined the term
“Causalgia”
 Early 20th century Peter Sudeck
 Described features of pain, swelling, atrophy etc. following
minor injury to limbs – hence this phenomenon came to be
called “Sudeck’s atrophy”
 A few years on – its association with the sympathetic
nervous system was recognized and hence the term
“reflex sympathetic dystrophy” was increasingly used

5. IASP Consensus group - in 1994
 The presence of an initiating
noxious event, or a cause for
immobilization
 Continuing pain and allodynia
which is disproportionate to any
inciting event
 Evidence at some time of
oedema, changes in skin blood
flow, abnormal sudomotor activity
in the region of pain
 The diagnosis is excluded by the
existence of other conditions that
can account for the degree of
pain and dysfunction
 Criteria 2-4 must be satisfied
 Continuing pain, allodynia, or
hyperalgesia after nerve injury,
not necessarily limited to the
distribution of the injured nerve
 Evidence at some time of
oedema, changes in skin blood
flow, abnormal sudomotor activity
in the region of pain
 The diagnosis is excluded by the
existence of other conditions that
can account for the degree of
pain and dysfunction
 All 3 Criteria must be satisfied
Complex Regional Pain Syndrome
TYPE I TYPE II

6. 1994 IASP Criteria
 Proved to be extremely
sensitive
 Insufficiently specific
 Over diagnoses of the
syndrome
 Difficult to validate
 In 2003, a workshop was
held in Budapest
 Published in 2007
 Modified diagnostic
criteria
Better discrimination
between CRPS and Non-
CRPS neuropathic pain

7. IASP revised criteria – “Budapest criteria”
 Continuing pain that is disproportionate to any inciting
event
 At least one symptom reported in at least 3 of the
following categories
 Sensory: Hyperesthesia or allodynia
 Vasomotor: Temperature asymmetry, skin colour changes,
skin colour asymmetry
 Sudomotor/ Oedema, sweating changes or sweating
oedema asymmetry
 Motor / Trophic Decreased range of motion, motor
dysfunction (E.g. weakness, tremor, dystonia),
or trophic changes (e.g. hair, skin, nails)

8. Criteria continued ……………
 At least one sign at time of evaluation in at least two of
the following categories
 Sensory: Evidence of hyperalgesia (to pinprick), allodynia
(to light touch), temperature sensation, deep
somatic pressure or joint movement
 Vasomotor: Evidence of temperature asymmetry (>1°C), skin
colour changes or symmetry
 Sudomotor/ Evidence or oedema, sweating changes, or
Oedema sweating asymmetry
 Motor/Trophic Evidence of deceased range of movement,
motor dysfunction (E.g. weakness, tremor,
dystonia), trophic changes (E.g. nail, skin, hair)
 No other diagnosis explaining the signs and symptoms

9. More stringent criteria in a research setting: to
increase specificity
 At least one SYMPTOM in Three ‘SYMPTOM’ categories
 At least one SIGN in Three ‘SIGN’ categories
 For diagnosis of CRPS in a Research Setting

10. Epidemiology
 A population study from the Netherlands showed
 Incidence of 26 in 100,000;
 Female : Male ratio of 3.5:1
 Peak incidence in the age group of 55-70 years
 Upper limb more common than the lower
 Fracture was the most common precipitating factor
 CRPS I more common than CRPS II
(de Mos M et al. Pain 2007; 129:12-20)
 This was 4 times that of a previous population study in
the US – 5.5 / 100,000

11. Pathophysiology
 Not well understood for many years
 Renewed research interest
 Revised diagnostic criteria
 Availability of animal models of CRPS
Pain 2015; 156: S94-S103

12. Inciting event –
Trivial injury to the limb or injury to a peripheral nerve
 Inflammation is exaggerated for yet unclear reasons
 inflammatory mediators leading to swelling, changes in colour,
increased skin temperature
 Pain and hyperalgesia
 Hyperhidrosis / hypohidrosis due to mediators such as
neuropeptides (Substance P, CGRP, bradykinin)
 Trophic changes due to cytokines
 Motor function is also impaired by peripheral inflammation
 Increased levels of TNF-α, with a decrease in anti-
inflammatory cytokines

13.  Neuropeptides – released by sensitized peptidergic
nociceptors ( neurogenic inflammation)
 Endothelin-1 – potent vasoconstrictor (hyperalgesia)
 Autoantibodies on surface structures of β2-adregeric
receptors and m2-cholinergic receptors (which provides
a link to the sympathetic nervous system)
 In a significant subset of patients, CRPS gradually
“centralizes” – (time frame varies with individuals)
 Mechanical hyperalgesia
 Non-dermatomal sensory deficits
 Body perception disturbances
 Movements disorders

14. Conceptual model of CRPS -
• Enhanced anti-dromic secretion of
Neuropeptides
• Enhanced release of Immune
mediators
• Surface binding auto-antibodies
• Contributes to changes in sensory
nerve function and axonal
degeneration
• Viscous cycle is set in motion

15. Electrical Stimulation induces Plasma Protein Extravasation
in CRPS

16. Pathophysiology which includes central components

17. Clinical Features are a continuum
 Stage One (acute stage – 6-8 weeks after injury)
 Warmth, coolness, burning pain, oedema, increased sensitivity to
touch, increased pain with hyperalgesia, accelerated hair / nail
growth, tenderness or stiffness of joints, spasm, bone changes on
X-ray
 Decreased sympathetic activity

18. Clinical Features of CRPS
 Stage Two: Dystrophic phase ( can last several months)
 Pain is constant – throbbing, burning, aching, exaggerated by
stimuli
 Affected limb may still have oedema, cool, mottled
appearance
 Nails – brittle and ridged
 Pain and stiffness of joints persist
 Muscles – tremors, signs of wasting
 Psychological distress sets in (mainly from lack
of pain relief)
 Changes in body perception (limbs)
 Increased sympathetic activity

19. Stage 3 – Atrophic phase (unlimited amount of time)
 Typically the patient has had CRPS for 3+ years
 Pain is still constant (varies in degree depending on the
patient)
 Skin is cool, thin and shiny
 Atrophy of limb – with contractures of joints
 Muscle wasting
 Increase in osteoporosis
 Unlikely for sympathetic blocks to be effective
 Central changes
 CRPS features can extend beyond the original region

20. Diagnosis
 Clinical – There are no specific tests
 Differential diagnosis needs to be considered and
excluded
• 53 year old male patient
• Clinical features of pain and allodynia
• Limited to the fourth and fifth fingers
• Bluish and significantly cooler
• Thermography showed only the
innervation of the ulnar nerve was colder
• Traumatic ulnar paresis
• Diagnosis : Posttraumatic neuralgia

21. Having a Check-List
will help with the
Diagnosis

22. Item 4. No other diagnosis explaining the signs and symptoms

23. Management

24. Early Recognition is the key to Management
• Patients may be first seen
by a host of different
specialists
• It is important to create
awareness about CRPS in
these groups
• Early recognition and
referral for appropriate
therapy can improve
chances for better
management

25. Budapest diagnostic criteria (A–D must apply).
Andreas Goebel Rheumatology 2011;rheumatology.ker202
© The Author 2011. Published by Oxford University Press on behalf of the British Society for
Rheumatology. All rights reserved. For Permissions, please email:
journals.permissions@oup.com
CHECK LIST

26. The 4 Pillars of Management

27. Pain Relief
 Corticosteroids
 Calcium-regulating drugs
 Calcitonin, Alendronate
 Opioids
 Anti Neuropathic drugs
 Ketamine
 Sodium channel blockers
 Lignocaine
 Clonidine
Very little consistent information is
available for these pharmacological
agents
 In early CRPS – may be useful
 Has been tried
 When others fail to provide pain relief
 TCA, gabapentin, pregabalin
 Ketamine ( Low dose infusions have
been tried with mixed results)
 Pain relief – so that patients are able
to comply with physical therapy,
which is the KEY to management
Medications

28. Procedures
 Intravenous Regional
Techniques
 Local anaesthetics
 Guanethidine
 Selective Sympathetic
ganglion blocks
 Useful is some cases of
sympathetically maintained pain
 Spinal Cord Stimulation and
Neuromodulation

29. Physical and Vocational therapy
 Patient education
 Stretching
 General exercise and
Strengthening
 Desensitization
 Gait re-education
 TENS
 Postural control
 Oedema control
strategies
 Sleep hygiene
 Graded Motor Imagery
Mirror Visual Feedback

30. Patient viewing non‐reflective surface with painful limb
hidden.
C. S. McCabe et al. Rheumatology 2003;42:97-101
© British Society for Rheumatology

31. Patient viewing non‐painful limb in mirror with painful limb hidden.
C. S. McCabe et al. Rheumatology 2003;42:97-101
© British Society for Rheumatology

32. Psychological Therapy
 Patient education and support
 Goal setting
 Relaxation techniques
 Pacing techniques
 Coping skills
 Facilitating self-management of condition

33. Watch for Yellow Flags

34. In Conclusion
 CRPS is a chronic debilitating painful condition
 There has been significant advances in our
understanding of its Pathophysiology
 Early diagnosis and management – is essential to help
patients and reduce suffering
 The Budapest Criteria should help while excluding others
 A Multidisciplinary Approach to Management has been
shown to be beneficial
 With particular emphasis on Patient Education and Support

35. https://www.rcplondon.ac.uk/sites/default/files/documents/complex-regional-pain-full-guideline.pdf