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Disease Profile
Complex regional pain syndrome
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
All ages
ICD-10
G90.5 G90.6
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
CRPS; Reflex sympathetic dystrophy
Categories
Nervous System Diseases
Summary
Complex regional pain
Symptoms
- sensitivity to touch or cold
- swelling of the painful area
- changes in skin temperature, color, and/or texture
- joint stiffness and swelling
- muscle weakness and/or muscle spasms
Symptoms may change over time and vary from person to person. In some people, signs and symptoms of go away on their own. In others, symptoms can persist for months to years.[4]
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
Limb pain | 0009763 | |
30%-79% of people have these symptoms | ||
Abnormality of hair growth | 0040170 | |
Allodynia | 0012533 | |
Dry skin | 0000958 | |
Edema of the upper limbs |
Fluid accumulation in upper limbs
|
0010742 |
Erythema | 0010783 | |
Hyperalgesia | 0031005 | |
Involuntary movements |
Involuntary muscle contractions
|
0004305 |
Pedal edema |
Fluid accumulation in lower limbs
Lower leg swelling
[ more ] |
0010741 |
Slow-growing nails | 0008383 | |
Trophic changes related to pain | 0010834 |
Cause
Treatment
A combination of therapies is usually necessary including medications, physical and occupational therapy, interventional procedures, and psychosocial/behavioral management.
Medications may include:
- Oral and topical pain relievers
- Antidepressants or anticonvulsants (which are sometimes used to treat pain)
Corticosteroids - Bone-loss medications
- Sympathetic nerve-blocking medications
- Intravenous anesthetics (Ketamine)
- Intravenous immunoglobulin
Interventional procedures may include:
- Trigger/tender point injections
- Regional sympathetic nerve block
- Spinal cord stimulation
- Epidural clonidine
- Chemical or mechanical sympathectomy
Other therapies may include applying heat or cold; electrical nerve stimulation; and biofeedback.[2][3]
Psychosocial and behavioral aspects of CRPS should be addressed, and it has been suggested that people with chronic CRPS should have a thorough psychological evaluation. This may be followed by cognitive-behavioral pain management, including relaxation training with biofeedback.[5]
Unfortunately, published research studies validating the efficacy of these treatment options are limited and no single drug or therapy (or combination) has shown consistent, long-lasting improvement.[2][3]
For more information on treatment options for CRPS, view information from the Reflex Sympathetic Dystrophy Association of America (RSDSA).
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
American Chronic Pain Association (ACPA)
P.O. Box 850
Rocklin, CA 95677-0850
Toll-free: 800-533-3231
Telephone: 916-632-0922
Fax: 916-652-8190
E-mail: ACPA@theacpa.org
Website: https://theacpa.org/ -
CRPS Forum
3940 Laurel Canyon Blvd., #634
Studio City, CA 91604
E-mail: contact@crpsforum.org
Website: https://crpsforum.com/ -
Pain Relief Foundation
Clinical Sciences Centre
University Hospital Aintree, Lower Lane
Liverpool L9 7AL
United Kingdom
Telephone: 0151 529 5820
Fax: 0151 529 5821
Website: https://painrelieffoundation.org.uk/ -
Reflex Sympathetic Dystrophy Syndrome Association of America (RSDA)
99 Cherry St
PO Box 502
Milford, CT 06460
Toll-free: 1-877.662.7737 (Helpline)
Telephone: +1-203-877-3790
Fax: +1-203.882.8362
E-mail: info@rsds.org
Website: https://rsds.org/
Social Networking Websites
- The Burning Nights CRPS/RSD Forum is an online community forum for people with CRPS/RSD as well as family, friends, loved ones, and caretakers.
- Visit the following Facebook groups related to Complex regional pain syndrome:
The Burning Limb Foundation
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- MayoClinic.com has an information page on Complex regional pain syndrome.
- MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
- The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- Reflex Sympathetic Dystrophy Syndrome Association of America (RSDSA) offers information and support for Complex regional pain syndrome
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Complex regional pain syndrome. Click on the link to view a sample search on this topic.
References
- Telltale Signs and Symptoms of CRPS/RSD. Reflex Sympathetic Dystrophy Syndrome Association of America (RSDSA). 2015; https://rsds.org/telltale-signs-and-symptoms-of-crpsrsd/.
- NINDS Complex Regional Pain Syndrome Information Page. NINDS. November 3, 2015; https://www.ninds.nih.gov/disorders/reflex_sympathetic_dystrophy/reflex_sympathetic_dystrophy.htm.
- Salahadin Abdi. Complex regional pain syndrome in adults: Prevention and management. UpToDate. Jan 06, 2016; https://www.uptodate.com/contents/complex-regional-pain-syndrome-in-adults-prevention-and-management.
- Complex regional pain syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/complex-regional-pain-syndrome/basics/definition/con-20022844. Accessed 11/11/2014.
- Bruehl, S and Chung OY. Psychological and Behavioral Aspects of Complex Regional Pain Syndrome Management. Clinical Journal of Pain. June, 2006; 22(5):430-437. https://journals.lww.com/clinicalpain/Abstract/2006/06000/Psychological_and_Behavioral_Aspects_of_Complex.5.aspx.
- David D. Sherry. Complex regional pain syndrome in children. UpToDate. Waltham, MA: UpToDate; November, 2014; Accessed 11/11/2014.
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