II. Definitions

  1. Complex Regional Pain Syndrome (CRPS, previously Reflex Sympathetic Dystrophy)
    1. Severe, Chronic Pain and Disability of a limb (part or whole)

III. Pathophysiology

  1. Unclear mechanism
  2. Post-Traumatic inflammatory changes are present in CRPS
  3. Genetic component is likely
    1. Another family member with CRPS is not uncommon

IV. Epidemiology

  1. Incidence: 26 per 100,000 patients
  2. Female predominance
  3. Peak Incidence age 61-70 years old

V. Types

  1. Type I Complex Regional Pain Syndrome (CRPS1)
    1. Not attributable to a specific nerve lesion
  2. Type II Complex Regional Pain Syndrome (CRPS2) or Causalgia
    1. Attributable to a specific nerve lesion
    2. However, resulting Allodynia and hyperalgesia does not follow a single nerve

VI. Risk Factors

  1. Local Trauma
  2. Myocardial Infarction
  3. Routine surgery
  4. Diabetes Mellitus
  5. Malignancy with paraneoplastic syndrome

VII. Causes: Precipitating events

  1. Precipitating injury (most common)
    1. Limb Fracture
      1. Colles Fracture (28% develop CRPS)
      2. Distal Radius Fracture (3-5% develop CRPS)
    2. Stab or Puncture Wound
    3. Animal Bite
  2. Shoulder-Hand Syndrome
    1. Myocardial Infarction
    2. Cerebrovascular Accident

VIII. Symptoms

  1. Limb pain
    1. Upper limb is affected in 60% of cases
  2. Onset 4-6 weeks after a mild to moderate limb injury
    1. See precipitating injuries above

IX. Signs

  1. Pain
    1. Burning ache in a non-Dermatome pattern (early)
    2. Hyperalgesia (disproportionate pain to inciting event) develops
    3. Pain does not follow a single nerve innervation pattern (even when the initial cause was nerve related)
  2. Trophic Changes
    1. Early Changes
      1. Local edema
    2. Progressive Changes
      1. Skin thins
      2. Hair coarsens
      3. Nail thickens
    3. Late Changes
      1. Muscles shorten and atrophy
      2. Joints ankylose moving distal to proximal
  3. Autonomic instability (Late)
    1. Limb cool and sweaty
    2. Sympathetic hyperactivity
  4. Sensory Abnormalities
    1. Allodynia (exquisite sensitivity to slight touch)
    2. Hyperpathia (severe pain from gentle pressure)
  5. Bony changes
    1. Patchy Osteopenia

X. Stages

  1. Mild (Days to weeks)
    1. Burning pain worse with movement
    2. Edema
    3. Muscle spasm
    4. Patchy Osteopenia may occur
    5. Usually self limited
  2. Moderate: weeks to months
    1. Pain of local hyperesthesia
    2. Muscle wasting
      1. May radiate up extremity
      2. May affect contralateral extremity
    3. Skin cold and pale
    4. Abnormal bone scan
    5. Requires aggressive treatment
  3. Severe: weeks to months
    1. Pain from cold and from touch of additional clothing
    2. Loss of mobility of several joints
    3. Psychiatric changes
    4. Aggressive management may be too late

XI. Imaging

  1. XRay (Changes in 85% of cases)
    1. Diffuse Osteopenia in 69% of cases (non-specific)
  2. Abnormal bone scan in 50-85% of cases

XII. Differential Diagnosis

XIII. Management

  1. Active range of motion ("Move it OR lose it")
    1. Aggressive physical therapy with pain control
    2. Avoid immobilization (e.g. splints, slings)
  2. Physical therapy
    1. Isotonic stength
    2. Passive range of motion
    3. Aerobic Exercises
    4. Pool Exercises
    5. Ergonomic adjustments
  3. Analgesics
    1. NSAIDS (e.g. Ibuprofen or Naprosyn )
    2. Acetaminophen
  4. Antiinflammatory
    1. Oral Corticosteroids
  5. Neuropathy agents
    1. Gabapentin (Neurontin)
    2. Pregabalin (Lyrica)
    3. Tricyclic Antidepressant (e.g. Amitriptyline, Nortriptyline)
  6. Topical Medications
    1. Topical Lidocaine Patch (Lidoderm 5% or the OTC Lidocare 4%)
    2. Topical Dimethyl sulfoxide cream (50%) applied for 2 months
      1. May act as free radical scavenger
  7. Novel medications (consult pain management specialists or rheumatology)
    1. Bisphosphonates (e.g. Pamidronate 60 mg IV for 1 dose)
    2. Intravenous Immunoglobulin
      1. Goebel (2010) Ann Intern Med 152:152-8 [PubMed]
      2. Goebel (2013) Rheumatology 52 (11): 2091-3 [PubMed]
    3. Lidocaine infusion
      1. Initial: 1.5 mg/kg (1-3 mg/kg) IV over 20-30 min
      2. Later: 0.2 to 2 mg/kg per hour
      3. Wallace (2000) Anesthesiology 92(1):75-83 [PubMed]
      4. Ferrini (2004) J Support Oncol 2(1): 90-4 [PubMed]
  8. Invasive measures (pain management procedures)
    1. Spinal cord stimulation
    2. Intrathecal Baclofen Pump
    3. Sympathetic Nerve block (Stellate Ganglion, Brachial Plexus, lumbar)
      1. Temporary relief
  9. Other measures
    1. TENS Unit (questionable efficacy)
    2. Mental health counseling for patient and family

XIV. Prognosis

  1. Longterm Disability is common
    1. Only 20% can participate in prior activities (pain is the limiting factor in >70%)
    2. Schwartzman (2009) Clin J Pain 25(4): 273-80 [PubMed]

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Related Studies

Ontology: Causalgia (C0007462)

Definition (MSHCZE) Tupá či intenzivní stálá bolest, obv. spojená s pocením a poruchou prokrvení v dané oblasti. Vzniká při poškození perif. nervu, jak přímém (trauma), tak nepřímém (porucha krevního zásobení). Srov. Sudeckův syndrom, neuralgie. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) A complex regional pain syndrome characterized by burning pain and marked sensitivity to touch (HYPERESTHESIA) in the distribution of an injured peripheral nerve. Autonomic dysfunction in the form of sudomotor (i.e., sympathetic innervation to sweat glands), vasomotor, and trophic skin changes may also occur. (Adams et al., Principles of Neurology, 6th ed, p1359)
Concepts Disease or Syndrome (T047)
MSH D002422
ICD10 G56.4
SnomedCT 66056001, 247397004, 155074001, 267705008, 408751001
English Causalgia, Complex region pain syndrom II, CRPS II, CRPS-Complex regn pain synd II, causalgia (diagnosis), causalgia, Causalgia Syndrome, Causalgia Syndromes, Syndrome, Causalgia, Syndromes, Causalgia, Complex Regional Pain Syndrome Type II, Type II Complex Regional Pain Syndrome, CRPS Type II, Deafferentation Pain, Pain, Deafferentation, Causalgia [Disease/Finding], causalgia syndrome, crps type ii, Causalgia (disorder), Causalgia (finding), causalgia; syndrome, syndrome; causalgia, Complex regional pain syndrome type II, Deafferentation pain, CRPS - Complex regional pain syndrome type II, Complex regional pain syndrome, type II (disorder), Complex regional pain syndrome, type II, Complex regional pain syndrome Type II, Causalgia NOS
Dutch causalgie, complex regionaal pijn syndroom type II, causalgie; syndroom, syndroom; causalgie, Causalgie, Complex regionaal pijnsyndroom type II
French Syndrome de douleur régionale complexe de type II, Causalgie, SDRC de type 2, SDRC de type II, Syndrome de douleur régionale complexe de type 2, Syndrome douloureux régional complexe de type 2
German komplexes regionales Schmerzsyndrom Typ II, Kausalgie, Komplexes regionales Schmerzsyndrom Typ II
Italian Sindrome da dolore regionale complesso tipo II, CRPS tipo II, Dolore da deafferentazione, Sindrome dolorosa regionale complessa tipo II, Causalgia
Portuguese Síndrome de dor regional complexa Tipo II, Causalgia, Síndrome da Dor Regional Complexa Tipo II
Spanish Síndrome de dolor regional complejo tipo II, causalgia, causalgia (trastorno), síndrome de dolor regional complejo, tipo II (trastorno), síndrome de dolor regional complejo, tipo II, Causalgia, Síndrome de Dolor Regional Complejo Tipo II
Japanese 複合性局所疼痛症候群2型, カウザルギー, フクゴウセイキョクショトウツウショウコウグン2ガタ, カウザルギー, 求心路遮断痛, 灼熱痛, 複合性局所疼痛症候群タイプ2
Swedish Kausalgi
Czech kauzalgie, Kauzalgie, Syndrom komplexní regionální bolesti typu II, CRPS II
Finnish Kausalgia
Korean 복합 부위 통증증후군 2형
Polish Kauzalgia
Hungarian Complex regionális fájdalom-syndroma II. típus, Causalgia
Norwegian Komplekst regionalt smertesyndrom type 2, Kausalgi, Komplekst regionalt smertesyndrom type II

Ontology: Reflex Sympathetic Dystrophy (C0034931)

Definition (MSHCZE) Sudeckův syndrom, algodystrofický syndrom, syndrom rameno-ruka – komplikace některých úrazů horní končetiny, které se léčí znehybněním. V některých případech se příčina neprokáže. Projevuje se bolestmi a zánětlivým postižením ramena (periartritidou) a ruky. V počátcích jsou silné klidové bolesti, omezená pohyblivost, otok, zarudnutí, zvýšené pocení i růst nehtů, později dochází k tuhnutí kloubů a k vzniku kontraktur, atrofií, zkrácení šlach, bolesti postupně mizí, zůstává však výrazné funkční omezení a strukturální změny. Na kostech vzniká osteoporóza. Léčba by měla začít co nejrychleji, spočívá v rehabilitaci, fyzikální terapii, podávají se protizánětlivé léky, kalcitonin, důležitá je prevence. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A rare neurologic syndrome characterized by burning pain, tenderness, swelling, and changes in the skin color and temperature of a body part or extremity. It is usually caused by injury or surgery.
Definition (MSH) A syndrome characterized by severe burning pain in an extremity accompanied by sudomotor, vasomotor, and trophic changes in bone without an associated specific nerve injury. This condition is most often precipitated by trauma to soft tissue or nerve complexes. The skin over the affected region is usually erythematous and demonstrates hypersensitivity to tactile stimuli and erythema. (Adams et al., Principles of Neurology, 6th ed, p1360; Pain 1995 Oct;63(1):127-33)
Concepts Disease or Syndrome (T047)
MSH D012019
ICD9 337.20, 337.21, 337.2
ICD10 M89.0 , G90.5
SnomedCT 2103002, 50642008, 156849009, 192921009, 203492008, 403604006, 128079007, 393605009, 203490000, 15743005
English Dystrophies, Reflex Sympathetic, Dystrophy, Reflex Sympathetic, Reflex Sympathetic Dystrophies, Reflex Sympathetic Dystrophy, Shoulder Hand Syndrome, Shoulder-Hand Syndrome, Sudeck's Atrophy, Sympathetic Dystrophies, Reflex, Sympathetic Dystrophy, Reflex, Syndrome, Shoulder-Hand, Shoulder-Hand Syndromes, Syndromes, Shoulder-Hand, Complex regionl pain syndrom I, RSD - Reflex sympath dystroph, REFLEX SYMPATHETIC DYSTROPHY, CRPS TYPE I, CRPS-Complex regn pain synd I, Reflex sympathetic dystrophy of upper extremity, Sudeck's atrophy (diagnosis), reflex sympathetic dystrophy (diagnosis), reflex sympathetic dystrophy, reflex sympathetic dystrophy of upper limb (diagnosis), shoulder-hand syndrome (diagnosis), shoulder-hand syndrome, reflex sympathetic dystrophy of upper limb, Atrophy, Sudek, Atrophy, Sudek's, Sudek Atrophy, Sudek's Atrophy, Sudeks Atrophy, Complex Regional Pain Syndrome, Type I, Pain Syndrome Type I, Complex Regional, Pain Syndrome Type I, Regional, Complex, Type I Complex Regional Pain Syndrome, CPRS Type I, I, CPRS Type, Type I, CPRS, Reflex Dystrophia, Sympathetic, Sympathetic Reflex Dystrophia, Sympathetic Reflex Dystrophias, RSD (Reflex Sympathetic Dystrophy), Reflex Sympathetic Dystrophy Syndrome, Syndrome, Reflex Sympathetic Dystrophy, Unsp rflx sympth dystrph, Rflx sym dystrph up limb, Reflex Sympathetic Dystrophy [Disease/Finding], reflex sympathetic dystrophy syndrome (RSDS), sudek's atrophy, Dystrophy;reflex sympathetic, shoulder hand syndrome, hand shoulder syndrome, sudeck's syndrome, steinbrocker's syndrome, sudeck's atrophy, Atrophies, Sudek's, CPRS Type Is, Is, CPRS Type, RSDs (Reflex Sympathetic Dystrophy), Sudek's Atrophies, Type Is, CPRS, reflex sympathetic dystrophy upper limb, Algodystrophy, Complex regional pain syndrome, type I, Sudeck's atrophy (disorder), Reflex sympathetic dystrophy (disorder), Reflex sympathetic dystrophy (& Sudek's atrophy) (disorder), Sudek's atrophy, Reflex sympathetic dystrophy (& Sudek's atrophy), Sudek's atrophy (disorder), Shoulder-hand syndrome (disorder), Algoneurodystrophy, Reflex sympathetic dystrophy, Shoulder-hand syndrome, Steinbrocker's syndrome, Sudeck's atrophy, Complex regional pain syndrome type I, RSD - Reflex sympathetic dystrophy, CRPS - Complex regional pain syndrome type I, Algodystrophy (disorder), Reflex sympathetic dystrophy of upper extremity (disorder), atrophy; Sudeck, Sudeck; atrophy, Sudeck, hand-shoulder; syndrome, shoulder-hand; syndrome, syndrome; hand-shoulder, syndrome; shoulder-hand, Reflex sympathetic dystrophy [Ambiguous], Shoulder hand syndrome, Reflex sympathetic dystrophy of the upper limb, Reflex sympathetic dystrophy, unspecified, Complex regional pain syndrome Type I
Spanish distrofia refleja simpática de la extremidad superior, Distrofia simpática refleja, Distrofia simpática refleja del miembro superior, Distrofia simpática refleja no especificada, Síndrome de hombro y mano, Síndrome de dolor regional complejo tipo I, distrofia refleja simpática, distrofia refleja simpática (trastorno), distrofia simpática refleja, atrofia de Sudek (trastorno), atrofia de Sudek, síndrome de dolor regional complejo, tipo I, algodistrofia (trastorno), algodistrofia, atrofia de Sudeck, distrofia refleja simpática de la extremidad superior (trastorno), síndrome de Steinbrocker, síndrome hombro-mano, Atrofia de Sudeck, Distrofia Simpática Refleja, Síndrome de Dolor Regional Complejo Tipo I, Síndrome de Dolor Tipo I Regional Complejo, Síndrome de Hombro-Mano
Dutch reflex autonome dystrofie, Sudeck-atrofie, schouder- en handsyndroom, reflex autonome dystrofie van bovenste ledemaat, reflex autonome dystrofie, niet-gespecificeerd, complex regionaal pijn syndroom type I, Sudeck; atrofie, atrofie; Sudeck, hand-schouder; syndroom, schouder-hand; syndroom, syndroom; hand-schouder, syndroom; schouder-hand, Complex regionaal pijnsyndroom type I, Dystrofie, sympathische reflex-, Pijnsyndroom type I, regionaal complex, Reflexdystrofie, sympathische, Schouder-handsynroom, Sudeck-dystrofie, Sympathische reflexdystrofie
French Dystrophie réflexe sympathique, Algoneurodystrophie, non précisée, Atrophie de Sudeck, Syndrome de douleur régionale complexe de type I, Algoneurodystrophie d'un membre supérieur, Dystrophie sympathique réflexe, Atrophie osseuse de Südeck-Leriche, DSR (Dystrophie Sympathique Réflexe), Dystrophie sympathique réflexe post-traumatique, Ostéoporose algique de Südeck-Leriche, Ostéoporose algique post-traumatique, Ostéoporose douloureuse post-traumatique, Rhumatisme neuro-trophique, Rhumatisme neurotrophique, SDRC de type 1, SDRC de type I, Syndrome de Steinbroker, Syndrome de Südeck-Leriche, Syndrome de Südeck, Syndrome de douleur régionale complexe de type 1, Syndrome douloureux régional complexe de type 1, Syndrome épaule-main
German komplexes regionales Schmerzsyndrom Typ I, sympathische Reflexdystrophie, Sudeck-Atrophie, sympathische Reflexdystrophie einer oberen Gliedmasse, sympathische Reflexdystrophie, unspezifisch, Schulter-Hand-Syndrom, Sympathische Reflexdystrophie, Komplexes regionales Schmerzsyndrom, Typ I, Schmerzsyndrom Typ I, regionales komplexes, Sudeckatrophie
Italian Distrofia simpatica riflessa, Atrofia di Sudeck, Sindrome da dolore regionale complesso tipo I, Distrofia simpatica riflessa dell'arto superiore, Distrofia simpatica riflessa non specificata, Sindrome spalla-mano, Atrofia di Sudek, Distrofia simpatica riflessa (DSR), Sindrome della distrofia simpatetica riflessa, CPRS tipo I, Sindrome complessa del dolore regionale tipo I, Distrofia simpatetica riflessa
Portuguese Distrofia simpática reflexa, Distrofia simpática reflexa do membro superior, Síndrome ombro mão, Distrofia simpática reflexa NE, Síndrome de dor regional complexa Tipo I, Atrofia de Sudeck, Distrofia Simpática Reflexa, Síndrome da Dor Regional Complexa Tipo I, Síndrome de Ombro-Mão
Japanese ズーデック萎縮, 複合性局所疼痛症候群1型, 上肢の反射性交感神経性ジストロフィー, 反射性交感神経性ジストロフィー、詳細不明, 肩手症候群, ハンシャセイコウカンシンケイセイジストロフィー, ハンシャセイコウカンシンケイセイジストロフィーショウサイフメイ, フクゴウセイキョクショトウツウショウコウグン1ガタ, ズーデックイシュク, ケンシュショウコウグン, ジョウシノハンシャセイコウカンシンケイセイジストロフィー, 反射性交感神経性異栄養症, 交感神経反射性ジストロフィー, 交感神経性異栄養症-反射性, 反射性交感神経性ジストロフィー, 反射性交感神経性ジストロフィ, 疼痛性ジストロフィー症候群, 反射性交感神経性萎縮症, 交感神経反射性異栄養症, Sudeck萎縮, 交感神経性ジストロフィー-反射性
Swedish Reflektorisk sympatisk dystrofi
Finnish Sympaattinen refleksidystrofia
Czech Reflexní sympatická dystrofie, blíže neurčená, Syndrom rameno-ruka, Syndrom komplexní regionální bolesti typu I, Sympatická reflexní dystrofie, Reflexní sympatická dystrofie horní končetiny, Sudeckova atrofie, Sudeckova dystrofie, reflexní neurovaskulární dystrofie, syndrom rameno-ruka, reflexní sympatická dystrofie, Sudeckův syndrom, syndrom reflexní algodystrofie
Polish Zespół algodystroficzny, Choroba Sudecka, Zespół ramię-ręka, Algodystrofia, Zespół Sudecka
Hungarian Felső végtag reflex dystrophiája, Complex regionális fájdalom- syndroma, I. típus, Sudeck-atrophia, Reflex dystrophia, Reflex dystrophia, nem meghatározott, Kéz-váll syndroma
Norwegian Sudecks atrofi, Komplekst regionalt smertesyndrom type 1, Komplekst regionalt smertesyndrom type I, Sympatisk refleksdystrofi

Ontology: Complex Regional Pain Syndromes (C0458219)

Definition (MEDLINEPLUS)

Complex regional pain syndrome (CRPS) is a chronic pain condition. It causes intense pain, usually in the arms, hands, legs, or feet. It may happen after an injury, either to a nerve or to tissue in the affected area. Rest and time may only make it worse.

Symptoms in the affected area are

  • Dramatic changes in skin temperature, color, or texture
  • Intense burning pain
  • Extreme skin sensitivity
  • Swelling and stiffness in affected joints
  • Decreased ability to move the affected body part

The cause of CRPS is unknown. There is no specific diagnostic test. Your doctor will diagnose CRPS based on your signs and symptoms.

There is no cure. It can get worse over time, and may spread to other parts of the body. Occasionally it goes away, either temporarily or for good. Treatment focuses on relieving the pain, and can include medicines, physical therapy, and nerve blocks.

NIH: National Institute of Neurological Disorders and Stroke

Definition (MSH) Conditions characterized by pain involving an extremity or other body region, HYPERESTHESIA, and localized autonomic dysfunction following injury to soft tissue or nerve. The pain is usually associated with ERYTHEMA; SKIN TEMPERATURE changes, abnormal sudomotor activity (i.e., changes in sweating due to altered sympathetic innervation) or edema. The degree of pain and other manifestations is out of proportion to that expected from the inciting event. Two subtypes of this condition have been described: type I; (REFLEX SYMPATHETIC DYSTROPHY) and type II; (CAUSALGIA). (From Pain 1995 Oct;63(1):127-33)
Concepts Disease or Syndrome (T047)
MSH D020918
SnomedCT 128200000
Dutch complex regionaal pijn syndroom, Complex regionaal pijnsyndroom, Pijnsyndromen, regionale complexe, Pijnsyndroom, regionaal complex, Regionaal pijnsyndroom, complex, Regionale pijnsyndromen, complexe, Syndromen, regionale complexe pijn-, Syndroom, regionaal complex pijn-
French Syndrome de douleurs régionales complexes, SDRC, Syndrome de douleur régionale complexe, Syndrome douloureux régional complexe (SDRC ), Syndrome douloureux régional complexe
Italian Sindrome da dolore regionale complesso, CRPS (Sindromi da dolore regionale complesso), Sindromi da dolore regionale complesso
Portuguese Síndrome de dor regional complexa, Crps, Síndromes da Dor Regional Complexa
Spanish Síndrome de dolor regional complejo, Crps, síndrome de dolor regional complejo (trastorno), síndrome de dolor regional complejo, Síndromes de Dolor Regional Complejo, Síndromes Dolorosos Complejo Regional
Japanese 複合性局所疼痛症候群, フクゴウセイキョクショトウツウショウコウグン, Complex Regional Pain症候群, Regional Complex Pain症候群
Swedish Komplext regionalt smärtsyndrom
Czech komplexní regionální syndromy bolesti, Syndrom komplexní regionální bolesti, komplexní regionální bolestivý syndrom, bolestivý syndrom, komplexní regionální
Finnish Monimuotoiset alueelliset kipuoireyhtymät
English Complex regional pain syndrome NOS, CRPS (Complex Regional Pain Syndromes), Complex Regional Pain Syndromes, Pain Syndromes, Regional Complex, Complex Regional Pain Syndromes [Disease/Finding], complexed pain regional syndrome, complex regional pain syndrome, pain regional complex syndrome, complex pain regional syndrome, complex regional pain syndromes, regional complex pain syndrome, CRPS, Complex Regional Pain Syndrome, Complex regional pain syndrome (CRPS), Complex regional pain syndrome, Complex regional pain syndromes, Complex regional pain syndrome (disorder), Complex regional pain syndromes (disorder)
Polish Zespoły bólowe złożone lokalne, Lokalne zespoły bólowe, Zespoły bólowe miejscowe
Hungarian Komplex regionális fájdalom syndroma
Norwegian Smertesyndromer, komplekse, regionale, Komplekst regionalt smertesyndrom
German Komplexe umschriebene Schmerzsyndrome, Schmerzsyndrome, umschriebene komplexe, komplexes regionales Schmerzsyndrom