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Complex Regional Pain Syndrome (CRPS): State of the Art review Angela Mailis Gagnon MD, MSc, FRCPC(PhysMed)

Director, Comprehensive Pain Program/UHN and

Senior Investigator Krembil Neuroscience Centre,

Professor, Dept. of Medicine, UofT

Faculty/Presenter Disclosure

Faculty: Angela Mailis-Gagnon

Relationships with commercial interests:

Grants/Research Support: Purdue; Merck; Valeant; Sanofi; Ortho Janssen

Speakers Bureau/Honoraria: None

Consulting Fees: None for the last 5 yrs.

Other: Advisory Board Lyrica and Cymbalta (terminated 2013)

Disclosure of Commercial Support

This program has not received any financial support.

Mitigating Potential Bias

Unrestricted educational grants (slide 2) have no relationship whatsoever with presented topic

Objectives

By the end of the presentation the audience will be able to:

A. Define what is CRPS;

B. Describe the Budapest criteria;

C. Understand the differential diagnosis between CRPS and other conditions.

A. What is CRPS?

CRPS is a chronic disorder primarily following local trauma;

The pathophysiology of CRPS (neuropathic, nociceptive or mixed) is debated.

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Two types of CRPS are recognized: type I (formerly called RSD) without a definable nerve injury and type II (formerly called causalgia), where a definable nerve injury is present.

What is CRPS?

The diagnosis of CRPS is clinical, based on signs and symptoms;

There is no difference between the clinical manifestations of CRPS I and II;

Often nerve injury can not be shown because a) injury to small nerves or sensory nerves may not be detected, and b) EMG/NCT may not be tolerated by the patients

Examples of CRPS I and II

CRPS I after ankle sprain

CRPS I: Notice

severe swelling and

brown discoloration

of the skin

CRPS I: Severe dystonic posturing with

permanent contractures within 6

months post onset of symptoms

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Digital CRPS (thumb and

index)

Terminal CRPS I: Cold,

contracted hand

CRPS-like appearance

after sympathectomy

Looks like CRPS but it is NOT

Brachial plexus injury

Infected knee prosthesis

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B. CRPS IASP definition (1994)

Presence of an inciting noxious event or a cause for immobilization;

Continuing pain, allodynia or hyperalgesia, with disproportionate pain to any inciting event;

Evidence at some time of edema, skin blood flow changes or abnormal sweating in the region of pain;

Diagnosis excluded by conditions accounting for degree of pain and dysfunction.

Current State of knowledge regarding the 1994 IASP criteria

RESEARCH over the past 10 years has shown that:

The criteria are NOT internally valid, i.e., they do not adequate reflect natural groupings between various symptoms and signs;

The criteria are NOT externally valid, i.e., they can not distinguish between CRPS patients and patients with other types of neuropathic pain;

CRPS was overdiagnosed in 37-60% of patients in a number of studies.

Budapest Clinical Diagnostic Criteria

Continuing pain disproportionate to inciting event, AND Patient must report at least 1 symptom in 3 out of the 4 categories:

Sensory: Hyperesthesia and/or allodynia;

Vasomotor: Temperature asymmetry and/or skin color changes and/or colour asymmetry;

Budapest Clinical Diagnostic Criteria Sudomotor/edema: Edema and/ or sweating changes;

Motor/trophic: ROM and /or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nails, skin)

Must display at least one sign in 2 or more out of the 4 categories:

Sensory: Pinprick hyperalgesia and/or allodynia (to light touch and/or deep somatic pressure and/or joint movement);

Vasomotor: Temperature asymmetry and/or skin color changes and/or colour asymmetry;

New IASP clinical criteria cont’d New IASP clinical criteria

cont’d Sudomotor/edema: Edema and/or sweating changes;

Motor/trophic: ROM and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nails, skin);

PLUS No other diagnosis better explains the symptoms/ signs.

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This proposal has provided specific criteria for different clinical markers and distinction between signs and symptoms.

The criteria increase diagnostic specificity and reduce mis-diagnosis/ medical resource overutilization, with almost no reduction in diagnostic sensitivity.

The “Budapest Clinical Diagnostic” criteria have been adopted by both the IASP Executive and the IASP Taxonomy Committee (January 2012)

Proportionate

Disproportionate

NRS now

NRS range

PAIN

Sensory Vasomotor Sudomotor

edema Motor/

trophic

SYMPTOMS Cloth sensitivity Blue, red or blotchy

limb

Swelling Reduced

ROM

Temperature Sweating weakness

dystonia

dystrophy

SIGN Hyperalgesia Temperature

Swelling ROM↓

LT sensitivity Discoloration Sweating weakness

Deep pain dystonia

Kinesthetic

allodynia

dystrophy

DIAGNOSIS CRPS –I CRPS-II Unclear Other

(specify)

Name ………. limb …… Date ………

………… Myths and misconceptions

CRPS/RSD occurs frequently after fractures or trivial injuries: FALSE. The only population based Olmsted County 2003 study shows incidence of 5.46/100,000 (2.16/100K for males/ 8.57/100K for females);

CRPS is an intractable progressive disease: FALSE. The same study shows that 74% of cases resolve, often spontaneously;

Myths and misconceptions

Females are not as frequently affected as males: TRUE. Females/ Males 4/1 with median age of onset 46 yrs.

+ve bone scan confirms the diagnosis and a -ve scan refutes it: FALSE. In a meta-analysis of 19 studies recently, half of all patients with clinical CRPS diagnosis had a normal bone scan;

Myths and misconceptions

If the patient responds to sympathetic blocks, the CRPS diagnosis is confirmed: FALSE. Scientific studies have shown a very high response to placebo (sham) blocks;

Chemical sympathectomy can cure CRPS: FALSE. It has only temporary effects, primarily on touch evoked pain;

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Myths and misconceptions

Surgical sympathectomy is a permanent solution for responders to sympathetic blocks:

FALSE. It has been shown to produce severe complications and should be avoided.

Watch out for imitators

The majority of post fracture patients meet many CRPS criteria early on;

CRPS signs/symptoms can be the mere product of immobilization;

CRPS signs/symptoms may be seen in other diseases (tumours, inflammation, infection, fracture);

CRPS signs/symptoms can be imitated in cases of factitious or self-induced disorders.

Self-induced Disorders: Watch out!

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SS:Oct.3, 2000

SS: Jan.30,2001

Ligature sign

Characteristics of Complex Regional Pain Syndrome in patients referred to a tertiary pain clinic by community physicians, assessed by the Budapest Clinical Diagnostic Criteria. Angela Mailis-Gagnon, S. Fatima Lakha, Matti D. Allen,

Amol Deshpande, R. Norman Harden, In Press, PAIN MEDICINE

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Out of 58 patients only 19 (32.7%) were deemed to have CRPS

Pearls for diagnosis and management

CRPS d/x is given indiscriminately to anyone with pain and/or limb discoloration or swelling;

While few serial sympathetic blocks have a place early on, numerous sympathetic blocks, IV infusions and trigger blocks have NO place in CRPS management;

For the CRPS diagnosis it is mandatory that not only the Budapest criteria are met but also OTHER disorders are excluded.

Management of CRPS “Functional restoration is the necessary and

often sufficient condition” to restore health. Other treatments serve primarily to facilitate this.

Based on the most recent concept that CRPS in general is “a multifaceted disorder,” its care must indeed be “multidimensional;”

Early mobilization is the cornerstone of treatment and should include ROM and stress-loading techniques.Simultaneously, pain control (oral and via sympathetic blocks) is necessary to allow mobilization.

Management of CRPS

The only evidence-based preventive strategy is the use of vitamin C (200 mg/d) in patients with wrist fractures;

In established CRPS, the literature suggests:

A short course of oral corticosteroids.

Gabapentin, Pregabalin and tricyclic antidepressants can also be of value in CRPS.

Bisphosphonates such as IV pamidronate (a single dose of 60 mg) and oral alendronate (40 mg OD x 8 weeks in early phases.

Topical lidocaine may be useful in the early phases of CRPS.

Management of CRPS

Opioid analgesics can be used to facilitate mobilization during physical therapy. Their long term use may be necessary in refractory cases.

Intranasal (200 to 400 UI/d x 8 weeks) or intramuscular (100 to 300 UI/d x 4 weeks) calcitonin has shown a slight benefit for CRPS, which is comparable with that obtained by other therapies (eg, physical therapy combined with analgesics).

Spinal Cord Stimulator has been shown to benefit CRPS resistant to medical and other therapies.

TAKE HOME MESSAGE

CRPS/RSD is a terrible diagnosis to make

and

a terrible diagnosis to miss

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Thank you for your

attention