Raynaud syndrome
Raynaud syndrome | |
---|---|
Other names | Raynaud's, Raynaud's disease, Raynaud's phenomenon, Raynaud's syndrome |
Frequency | 4% of people[3] |
Named after | Maurice Raynaud |
Raynaud syndrome, also known as Raynaud's phenomenon, is a
Episodes are typically triggered by cold or emotional stress.
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
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 (
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]
Causes
Primary
Raynaud's disease, or primary Raynaud's, is diagnosed if the symptoms are
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]
- Connective tissue disorders:
- Scleroderma[12]
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Sjögren's syndrome
- Dermatomyositis
- Polymyositis
- Mixed connective tissue disease
- Cold agglutinin disease
- Ehlers-Danlos syndrome
- Eating disorders:
- Obstructive disorders:
- Atherosclerosis
- Buerger's disease
- Takayasu's arteritis
- Subclavian aneurysms
- Thoracic outlet syndrome
- Drugs:
- Beta-blockers
- Cytotoxic drugs – particularly chemotherapeutics and most especially bleomycin
- Cyclosporin
- Bromocriptine
- Ergotamine
- Sulfasalazine
- Anthrax vaccineswhose primary ingredient is the Anthrax Protective Antigen
- OTC pseudoephedrine medications (Chlor-Trimeton, Sudafed, others)[14]
- Occupation:
- Jobs involving vibration, particularly drilling and prolonged use of a string trimmer (weed whacker), experience vibration white finger
- Exposure to vinyl chloride, mercury
- Exposure to the cold (e.g., by working as a frozen food packer)
- Others:
- Physical trauma to the extremities
- Lyme disease
- Hypothyroidism
- Cryoglobulinemia
- Cancer
- Chronic fatigue syndrome
- Reflex sympathetic dystrophy
- Carpal tunnel syndrome
- Magnesium deficiency
- Multiple sclerosis
- Erythromelalgia (clinically presenting as the opposite of Raynaud's, with hot and warm extremities, often co-exists in patients with Raynaud's[15])
- Chilblains (also clinically presenting as the opposite of Raynaud's, with hot and itchy extremities; however, it affects smaller areas than erythromelalgia, for instance the tip of a toe rather than the whole foot)
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]
Diagnosis
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
A careful medical history will seek to identify or exclude possible secondary causes.
- Digital artery pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHgis diagnostic (positive).
- Doppler ultrasoundto assess blood flow
- anaemia of chronic disease or kidney failure.
- Blood test for urea and electrolytes may reveal kidney impairment.
- Thyroid function tests may reveal hypothyroidism.
- Tests for rheumatoid factor, erythrocyte sedimentation rate, C-reactive protein, and autoantibody screening may reveal specific causative illnesses or an inflammatory process. Anti-centromere antibodies are common in limited systemic sclerosis (CREST syndrome).
- Nail fold vasculature (capillaroscopy) can be examined under a microscope.
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.
- dihydropyridines nifedipine or amlodipine, preferably slow-release preparations – are often first-line treatment.[25] They have the common side effects of headache, flushing, and ankle edema, but these are not typically of sufficient severity to require cessation of treatment.[26] The limited evidence available shows that calcium-channel blockers are only slightly effective in reducing how often the attacks happen.[27] Although, other studies also reveal that CCBs may be effective at decreasing severity of attacks, pain and disability associated with Raynaud's phenomenon.[28] People whose disease is secondary to erythromelalgiaoften cannot use vasodilators for therapy, as they trigger 'flares' causing the extremities to become burning red due to too much blood supply.
- People with severe disease prone to ulceration or large artery thrombotic events may be prescribed aspirin.[25]
- The prostaglandin iloprost is used to manage critical ischemia and pulmonary hypertension in Raynaud's phenomenon, and the endothelin receptor antagonist bosentan is used to manage severe pulmonary hypertension and prevent finger ulcers in scleroderma.[25]
- SSRIs such as fluoxetine may help symptoms, but the data is weak.[25]
- PDE5 inhibitors, such as sildenafil and tadalafil, are used off-label to treat severe ischemia and ulcers in fingers and toes for people with secondary Raynaud's phenomenon; as of 2016, their role more generally in Raynaud's was not clear.[33]
Surgery
- In severe cases, an Microvascular surgeryof 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
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
- ^ 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.
- ^ 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.
- ^ PMID 27509103.
- ^ 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.
- ISBN 9780521629607. Archivedfrom the original on 24 April 2017.
- S2CID 209409136. Retrieved 4 October 2023.
- ^ "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.
- S2CID 71701101.
- PMID 16609626. Archived from the originalon 28 March 2020. Retrieved 6 February 2010.
- PMID 27509103.
- S2CID 51720201.
- PMID 17218139.
- PMID 18240233.
- ^ "Raynaud's disease Treatments and drugs". mayoclinic.org. Mayo Clinic. Archived from the original on 12 December 2015. Retrieved 13 December 2015.
- PMID 14988692.
- PMID 9814067.
- PMID 29763008, retrieved 15 December 2022
- PMID 28895508.
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- ^ PMID 24491823.
- ^ ISBN 978-1-84076-173-3.
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Phosphodiesterase inhibitors (e.g., sildenafil) can also improve [Raynaud's phenomenon] symptoms and ulcer healing
- PMID 17098681.
- S2CID 26698472.
- S2CID 8696332.
- ^ Hansen-Dispenza H (4 August 2022). "Raynaud Phenomenon: Practice Essentials, Pathophysiology, Etiology". Medscape.com. Retrieved 4 October 2023.
External links
- What Is Raynaud's Disease at National Heart, Lung, and Blood Institute
- Questions and Answers about Raynaud's Phenomenon at National Institutes of Health
- 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. PMID 18656283.