Raynaud syndrome

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Raynaud syndrome
Other namesRaynaud's, Raynaud's disease, Raynaud's phenomenon, Raynaud's syndrome
calcium channel blockers, iloprost[3]
Frequency4% of people[3]
Named afterMaurice Raynaud

Raynaud syndrome, also known as Raynaud's phenomenon, is a

numbness or pain occurs.[2] As blood flow returns, the area turns red and burns.[2] The episodes typically last minutes but can last several hours.[2] The condition is named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862.[6]

Episodes are typically triggered by cold or emotional stress.

stimulants.[7] Diagnosis is typically based on the symptoms.[3]

The primary treatment is avoiding the cold.

About 4% of people have the condition.[3] Onset of the primary form is typically between ages 15 and 30 and occurs more frequently in females.[3][4] The secondary form usually affects older people.[4] Both forms are more common in cold climates.[4]

Signs and symptoms

Raynaud's affecting all five fingers
Bluish coloration

The condition can cause localized pain, discoloration (paleness), and sensations of cold and/or numbness.

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. 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 (

hyperemia
of the areas deprived of blood flow.

In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud's has occurred in breastfeeding mothers, causing nipples to turn white and painful.[8]

Nipple blanching, or vasospasm of the nipple, can cause pain and difficulty breastfeeding.

Causes

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.[9]

Smoking increases frequency and intensity of attacks, and a hormonal component exists. Caffeine, estrogen, and nonselective beta-blockers are often listed as aggravating factors, but evidence that they should be avoided is not solid.[10]

Secondary

Raynaud's phenomenon, or secondary Raynaud's, occurs secondary to a wide variety of other conditions.

Secondary Raynaud's has a number of associations:[11]

Raynaud's can precede these other diseases by many years, making it the first presenting symptom. This may be the case in the CREST syndrome, of which Raynaud's is a part.[citation needed]

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.[citation needed]

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.[16]

Mechanism

Three main changes are seen in the mechanism of Raynaud's phenomenon which are reduced blood flow, blood vessel constriction and neurogenic, inflammatory, and immune responses. It is induced by mental stress and cold atmosphere. In all cases, the primary cause is an underlying hyperactivation of the sympathetic nervous system. Although, with different types, the exact pathophysiology differs. In primary type there is increase in sensitivity due to reasons mentioned above resulting in vasoconstriction. In secondary type, normal activity of blood vessel is disrupted due to the same reasons mentioned above causing vasoconstriction which leads to ischemia and tissue death.[17]

Underlying mechanism

Diagnosis

Thermogram depicting a Raynaud's hand (top) and a healthy hand (bottom): Red indicates warm areas whilst green indicates cool areas.
Consensus diagnostic criteria

Distinguishing Raynaud's disease (primary Raynaud's) from Raynaud's phenomenon (secondary Raynaud's) is important. Looking for signs of arthritis or vasculitis, as well as a number of laboratory tests, may separate them. Nail fold capillary examination or "capillaroscopy" is one of the most sensitive methods to diagnose RS with connective tissue disorders, i.e. distinguish a secondary from a primary form objectively.[18]

If suspected to be secondary to

systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography.[19]

A careful medical history will seek to identify or exclude possible secondary causes.

To aid in the diagnosis of Raynaud's phenomenon, multiple sets of diagnostic criteria have been proposed.[20][21][22][23] Table 1 below provides a summary of these various diagnostic criteria.[24]

Recently, International Consensus Criteria were developed for the diagnosis of primary Raynaud's phenomenon by a panel of experts in the fields of rheumatology and dermatology.[24]

Management

Secondary Raynaud's is managed primarily by treating the underlying cause, and as primary Raynaud's, avoiding triggers, such as cold, emotional and environmental stress, vibrations and repetitive motions, and avoiding smoking (including passive smoking) and sympathomimetic drugs.[25]

Medications

Medications can be helpful for moderate or severe disease.

Surgery

  • In severe cases, an
    Microvascular surgery
    of the affected areas is another possible therapy, but this procedure should be considered as a last resort.
  • A more recent treatment for severe Raynaud's is the use of botulinum toxin. The 2009 article[35] 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, three 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[36] describes similar improvement in a series of 11 patients. All patients had significant relief of pain.

Alternative medicine

Evidence does not support the use of alternative medicine, including

laser therapy.[3]

Prognosis

The prognosis of primary Raynaud syndrome is often very favorable, with no mortality and little morbidity. However, a minority develops gangrene. The prognosis of secondary Raynaud is dependent on the underlying disease, and how effective blood flow-restoring maneuvers are.[37]

References

  1. ^ a b c d "What Is Raynaud's?". nhlbi.nih.gov. US: National Heart, Lung, and Blood Institute, National Institutes of Health. 21 March 2014. Archived from the original on 4 October 2016. Retrieved 1 October 2016.
  2. ^ a b c d e f g h i j "What Are the Signs and Symptoms of Raynaud's?". nhlbi.nih.gov. US: National Heart, Lung, and Blood Institute, National Institutes of Health. 21 March 2014. Archived from the original on 5 October 2016. Retrieved 1 October 2016.
  3. ^
    PMID 27509103
    .
  4. ^ a b c d "Who Is at Risk for Raynaud's?". nhlbi.nih.gov. US: National Heart, Lung, and Blood Institute, National Institutes of Health. 21 March 2014. Archived from the original on 5 October 2016. Retrieved 1 October 2016.
  5. from the original on 24 April 2017.
  6. . Retrieved 4 October 2023.
  7. ^ "What Causes Raynaud's?". nhlbi.nih.gov. US: National Heart, Lung, and Blood Institute, National Institutes of Health. 21 March 2014. Archived from the original on 4 October 2016. Retrieved 1 October 2016.
  8. S2CID 71701101
    .
  9. PMID 16609626. Archived from the original
    on 28 March 2020. Retrieved 6 February 2010.
  10. .
  11. .
  12. .
  13. .
  14. ^ "Raynaud's disease Treatments and drugs". mayoclinic.org. Mayo Clinic. Archived from the original on 12 December 2015. Retrieved 13 December 2015.
  15. PMID 14988692
    .
  16. .
  17. , retrieved 15 December 2022
  18. .
  19. .
  20. .
  21. .
  22. .
  23. .
  24. ^ .
  25. ^ .
  26. .
  27. .
  28. .
  29. .
  30. .
  31. .
  32. .
  33. . Phosphodiesterase inhibitors (e.g., sildenafil) can also improve [Raynaud's phenomenon] symptoms and ulcer healing
  34. .
  35. .
  36. .
  37. ^ Hansen-Dispenza H (4 August 2022). "Raynaud Phenomenon: Practice Essentials, Pathophysiology, Etiology". Medscape.com. Retrieved 4 October 2023.

External links