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Raynaud's phenomenon

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Raynaud's phenomenon

Raynaud's phenomenon
Hands with Raynaud's phenomenon
Classification and external resources
Specialty Rheumatology
ICD-10 I73.0
ICD-9-CM 443.0
OMIM 179600
DiseasesDB 25933
MedlinePlus 000412
eMedicine med/1993
MeSH D011928

In medicine, Raynaud's Disease or Raynaud phenomenon is excessively reduced blood flow in response to cold or emotional stress, causing discolouration of the fingers, toes, and occasionally other areas. This condition may also cause nails to become brittle with longitudinal ridges. Named after French physician Maurice Raynaud (1834–1881), the phenomenon is believed to be the result of vasospasms that decrease blood supply to the respective regions.

Raynaud's phenomenon by itself is just a sign (hypoperfusion) accompanied by a symptom (discomfort). When linked to pathogenesis, it can be part of Raynaud's disease (also known as primary Raynaud's phenomenon), where the cause is unknown,[1] or part of Raynaud's syndrome (secondary Raynaud's phenomenon), which is a syndrome caused by a known primary disease, most commonly connective tissue disorders such as systemic lupus erythematosus. Measurement of hand-temperature gradients is one tool used to distinguish between the primary and secondary forms.[2]

It is a hyperactivation of the sympathetic nervous system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Chronic, recurrent cases of Raynaud phenomenon can result in atrophy of the skin, subcutaneous tissues, and muscle. In rare cases it can cause ulceration and ischemic gangrene.[3]

Contents

  • Signs and symptoms 1
  • Cause 2
    • Primary 2.1
    • Secondary 2.2
  • Diagnosis 3
  • Management 4
    • Surgery 4.1
  • See also 5
  • References 6
  • Further reading 7
  • External links 8

Signs and symptoms

An image taken by a thermographic camera. The top hand belongs to a person who suffers from Raynaud's, the bottom hand does not. Red indicates a heat signature whilst green indicates little/no heat.

The condition can cause pain within the affected extremities, discoloration (paleness), and sensations of cold and/or numbness. This can often be distressing to those who are not diagnosed, and sometimes it can be obstructive. If someone with Raynaud's is placed into a cold climate, it could potentially become dangerous.

Example of Raynaud's phenomenon, viewed from the front of the hand
Occurrence of Raynaud's phenomenon in only one finger in the hand.

  1. When exposed to cold temperatures, the blood supply to the fingers or toes, and in some cases the nose or earlobes, is markedly reduced; the skin turns pale or white (called pallor) and becomes cold and numb.
  2. When the oxygen supply is depleted, the skin color turns blue (called cyanosis).
  3. These events are episodic, and when the episode subsides or the area is warmed, the blood flow returns, and the skin color first turns red (rubor), and then back to normal, often accompanied by swelling, tingling, and a painful "pins and needles" sensation.

All three color changes are observed in classic Raynaud's. However, not all patients see all of the aforementioned color changes in all episodes, especially in milder cases of the condition. Symptoms are thought to be due to reactive hyperemias of the areas deprived of blood flow.

In pregnancy, this sign normally disappears owing to increased surface blood flow. Raynaud's has also occurred in breastfeeding mothers, causing nipples to turn white and become extremely painful.[4] Nifedipine, a calcium channel blocker and vasodilator, was recommended to increase blood flow to the extremities and noticeably relieved pain in the breast in an extremely small study group.[5]

Cause

Primary

Raynaud's disease, or "Primary Raynaud's", is diagnosed if the symptoms are idiopathic, that is, if they occur by themselves and not in association with other diseases. Some refer to Primary Raynaud's disease as "being allergic to coldness". It often develops in young women in their teens and early adulthood. Primary Raynaud's is thought to be at least partly hereditary, although specific genes have not yet been identified.[6]

Smoking increases frequency and intensity of attacks, and there is a hormonal component. Caffeine also worsens the attacks. Sufferers are more likely to have migraines and angina.

Secondary

Raynaud's syndrome, or "Secondary Raynaud's", occurs secondary to a wide variety of other conditions. Secondary Raynaud's has a number of associations:

Raynaud's can herald these diseases by periods of more than twenty years in some cases, making it effectively their first presenting symptom. This may be the case in the CREST syndrome, of which Raynaud's is a part.

Patients with Secondary Raynaud's can also have symptoms related to their underlying diseases. Raynaud's phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.

When Raynaud's phenomenon is limited to one hand or one foot, it is referred to as Unilateral Raynaud's. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses.[10]

Diagnosis

It is important to distinguish Raynaud's disease (primary Raynaud's) from syndrome (secondary Raynaud's). Looking for signs of arthritis or vasculitis as well as a number of laboratory tests may separate them.

A careful medical history will often reveal whether the condition is primary or secondary. Once this has been established, an examination is largely to identify or exclude possible secondary causes.

To aid in the diagnosis of Raynaud's phenomenon, multiple sets of diagnostic criteria have been proposed.[11][12][13][14] Table 1 below provides a summary of these various diagnostic criteria.[15]

Recently, International Consensus Criteria were developed for the diagnosis of primary Raynaud's phenomenon by a panel of multiple experts in the fields of rheumatology and dermatology.[15]

Consensus Diagnostic Criteria Raynaud's Phenomenon

Management

Raynaud's syndrome (secondary Raynaud's) is managed primarily by treating the underlying cause and as with Raynaud's disease (primary Raynaud's), avoiding triggers, such as cold, keeping a warm core body temperature, and avoiding smoking (including passive smoking) and sympathomimetic drugs.[16]

Drugs can be helpful for moderate or severe RP.

Surgery

  • In severe cases, a sympathectomy[22] procedure can be performed. Here, the nerves that signal the blood vessels of the fingertips to constrict are surgically cut. Microvascular surgery of the affected areas is another possible therapy. Infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.
  • A more recent treatment for severe Raynaud's is the use of botulinum toxin. The 2009 article[23] studied 19 patients ranging in age from 15 to 72 years with severe Raynaud's phenomenon of which 16 patients (84%) reported pain reduction at rest. 13 patients reported immediate pain relief, 3 more had gradual pain reduction over 1–2 months. All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the patients required repeated injections. A 2007 article[24] describes similar improvement in a series of 11 patients. All patients had significant relief of pain.

See also

References

  1. ^ "Raynaud disease" at Dorland's Medical Dictionary
  2. ^ Anderson ME, Moore TL, Lunt M, Herrick AL (March 2007). "The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon". Rheumatology 46 (3): 533–8.  
  3. ^ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 542.  
  4. ^ Holmen OL, Backe B (2009). "An underdiagnosed cause of nipple pain presented on a camera phone". BMJ 339: b2553.  
  5. ^ Anderson JE, Held N, Wright K (April 2004). "Raynaud's phenomenon of the nipple: a treatable cause of painful breastfeeding". Pediatrics 113 (4): e360–4.  
  6. ^ Pistorius MA, Planchon B, Schott JJ, Lemarec H (February 2006). "[Heredity and genetic aspects of Raynaud's disease]". Journal Des Maladies Vasculaires (in French) 31 (1): 10–5.  
  7. ^ Gayraud M (January 2007). "Raynaud's phenomenon". Joint, Bone, Spine 74 (1): e1–8.  
  8. ^ Goldman, William, Ryan Seltzer, and Peter Reuman. "Association between treatment with central nervous system stimulants and Raynaud's syndrome in children: A retrospective case–control study of rheumatology patients." Arthritis & Rheumatism 58.2 (2008): 563-566.
  9. ^ Berlin AL, Pehr K (March 2004). "Coexistence of erythromelalgia and Raynaud's phenomenon". Journal of the American Academy of Dermatology 50 (3): 456–60.  
  10. ^ Priollet P (October 1998). "[Raynaud's phenomena: diagnostic and treatment study]". La Revue du praticien (in French) 48 (15): 1659–64.  
  11. ^ Brennan P, Silman A, Black C (May 1993). "Validity and reliability of three methods used in the diagnosis of Raynaud's phenomenon. The UK Scleroderma Study Group". British Journal of Rheumatology 32 (5): 357–361.  
  12. ^ Wigley FM (September 2002). "Clinical Practice.Raynaud's phenomenon". New England Journal of Medicine 347 (13): 1001–1008.  
  13. ^ LeRoy EC, Medsger TA (September–October 1992). "Raynaud's phenomenon: a proposal for classification". Clinical and Experimental Rheumatology 10 (5): 485–488.  
  14. ^ Maricq HR, Weinrich MC (March 1998). "Diagnosis of Raynaud's phenomenon assisted by color charts". Journal of Rheumatology 15 (3): 454–459.  
  15. ^ a b Maverakis E, Patel F, Kronenberg D (2014). "International consensus criteria for the diagnosis of Raynaud's phenomenon". Journal of Autoimmunity 48: 60–5.  
  16. ^ a b c d e f g h Mikuls, Ted R; Canella, Amy C; Moore, Gerald F; Erickson, Alan R; Thiele, Geoffery M; O'Dell, James R (2013). "Connective Tissue Diseases". Rheumatology. London: Manson Publishing. p. 117.  
  17. ^ Smith CR, Rodeheffer RJ (January 1985). "Raynaud's phenomenon: pathophysiologic features and treatment with calcium-channel blockers". The American Journal of Cardiology 55 (3): 154B–157B.  
  18. ^ Ennis H, Anderson ME, Wilkinson J, Herrick AL (2014). "Calcium channel blockers for primary Raynaud's phenomenon.". Cochrane Database Syst Rev 1: CD002069.  
  19. ^ Pancera P, Sansone S, Secchi S, Covi G, Lechi A (November 1997). "The effects of thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in primary Raynaud's phenomenon". Journal of Internal Medicine 242 (5): 373–6.  
  20. ^ Dziadzio M, Denton CP, Smith R, et al. (December 1999). "Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial". Arthritis and Rheumatism (Elsevier Saunergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider) 42 (12): 2646–55.  
  21. ^ Waldo R (March 1979). "Prazosin relieves Raynaud's vasospasm". JAMA 241 (10): 1037.  
  22. ^ Wang WH, Lai CS, Chang KP, et al. (October 2006). "Peripheral sympathectomy for Raynaud's phenomenon: a salvage procedure". The Kaohsiung Journal of Medical Sciences 22 (10): 491–9.  
  23. ^ Neumeister MW, Chambers CB, Herron MS, et al. (July 2009). "Botox therapy for ischemic digits". Plastic and Reconstructive Surgery 124 (1): 191–201.  
  24. ^ Van Beek AL, Lim PK, Gear AJ, Pritzker MR (January 2007). "Management of vasospastic disorders with botulinum toxin A". Plastic and Reconstructive Surgery 119 (1): 217–26.  

Further reading

  • Bakst R, Merola JF, Franks AG, Sanchez M (October 2008). "Raynaud's phenomenon: pathogenesis and management". Journal of the American Academy of Dermatology 59 (4): 633–53.  

External links

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