Complex Regional Pain Syndrome

 

Am Fam Physician. 2021 Jul ;104(1):49-55.

  Patient information: A handout on this topic is available at https://familydoctor.org/condition/complex-regional-pain-syndrome/.

Author disclosure: No relevant financial affiliations.

Complex regional pain syndrome (CRPS) is a rare, chronic pain disorder. It is challenging for patients and physicians because it leads to significant morbidity due to chronic pain that may last for years. CRPS typically develops four to six weeks after direct trauma, such as an injury or surgery, and causes pain that is out of proportion to the inciting injury. It is associated with multiple physiologic mechanisms, affecting skin texture or localized sensory, motor, sudomotor, and vasomotor pathways. The diagnosis is made clinically using standardized diagnostic criteria. Ancillary testing is not necessary for the diagnosis of CRPS but can be useful to rule out alternative diagnoses. There are two types of CRPS, type 1 and type 2, although treatment is the same for both. There is no single proven treatment modality for CRPS, and there have been no large randomized controlled studies of CRPS treatments. Most treatments are based on studies of their use for other types of neuropathic pain. The mainstay of treatment is to improve function of the affected body part and to decrease pain; therefore, treatment requires multiple modalities, including medications, behavioral health interventions, and referral to a pain specialist.

Complex regional pain syndrome (CRPS) is a rare, chronic pain disorder. CRPS is challenging for patients and physicians because it leads to significant morbidity due to chronic pain that can last years. CRPS typically develops four to six weeks after direct trauma, such as an injury (e.g., fracture) or surgery.1 Many treatment recommendations for CRPS are based on smaller studies or consensus guidelines and on practice. Although most cases of CRPS resolve spontaneously without treatment,2 the pain, associated symptoms, psychological impact, and disability require a well-informed, patient-centered approach.

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComments

CRPS is diagnosed clinically using the Budapest criteria. Adjunct testing should be used to evaluate for alternative diagnoses when clinically indicated.3,6,7,16

C

Consistent disease-oriented findings from validation studies of diagnostic criteria for CRPS

Functional improvement is a main goal of CRPS treatment. The disease is most likely to respond to a comprehensive, integrated, multidisciplinary treatment approach that involves medical, psychological, and physical and occupational therapy components.7

C

Expert guideline that includes small retrospective studies, open-label trials, and pilot studies

Early aggressive treatment of CRPS may lead to prevention of chronic, disabling pain.713,

C

Consensus guidelines, inconsistent findings with disease-oriented outcomes

Evidence suggests that bisphosphonates and a short course of oral corticosteroids significantly improve pain in patients with CRPS.7,19

B

Expert guidelines and systematic review of small clinical trials

Patients with persistent symptoms that affect their quality of life despite treatment with medications, counseling, and physical and occupational therapy should be referred to a pain management specialist to discuss additional treatment options (e.g., ketamine [Ketalar] infusions, intrathecal drug pumps, regional nerve blocks, peripheral nerve stimulators, sympathetic ganglion blocks, surgical sympathectomy).7,8,19,2427

C

Consensus guidelines, inconsistent findings with disease-oriented outcomes


CRPS = complex regional pain syndrome.

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

The Authors

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EMILLIA C. O. LLOYD, MD, FAAFP, is program director of the Southern Colorado Family Medicine Residency at St. Mary-Corwin Hospital, Pueblo....

BRADLEY DEMPSEY, MD, is associate program director of the Southern Colorado Family Medicine Residency at St. Mary-Corwin Hospital.

LINDSEY ROMERO, MD, is a resident physician at the Southern Colorado Family Medicine Residency at St. Mary-Corwin Hospital.

Address correspondence to Emillia C. O. Lloyd, MD, FAAFP, 902 Lakeview Dr., Pueblo, CO 81004 (email: emillialloyd@centura.org). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

References

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