Raynaud syndrome

{{short description|Medical condition in which spasm of arteries causes episodes of reduced blood flow}} {{cs1 config|name-list-style=vanc}} {{Use dmy dates|date=July 2017}} {{Infobox medical condition | name = Raynaud syndrome | image = Raynaud_syndrome_on_female_airman's_hand.jpg | caption = The hand of a person with Raynaud syndrome during an attack. | field = Rheumatology | synonyms = Raynaud's, Raynaud's disease, Raynaud's phenomenon, Raynaud's syndrome<ref name=NIH2014What/> | pronounce = {{IPAc-en|r|eɪ|ˈ|n|oʊ}} {{respell|ray|NOH}} | symptoms = An affected part turning white, then blue, then red, burning<ref name=NIH2014Sym/> | complications = skin sores, gangrene<ref name=NIH2014Sym/> | onset = 15–30 years old, typically females<ref name=NEJM2016/><ref name=NIH2014Epi/> | duration = Up to several hours per episode<ref name=NIH2014Sym/> | causes = | risks = Cold, emotional stress, sympathomimetic drugs, dysautonomia, sometime underlying rheumatological findings<ref name=NIH2014Sym/> | diagnosis = Based on the symptoms<ref name=NEJM2016/> | differential = Causalgia, erythromelalgia<ref>{{cite book|last1=Barker|first1=Roger A.|title=The A-Z of Neurological Practice: A Guide to Clinical Neurology|date=2005|publisher=Cambridge University Press |isbn= 978-0-521-62960-7|page=728|url=https://books.google.com/books?id=LRRiuFfr9UkC&pg=PA728|language=en|url-status=live|archive-url=https://web.archive.org/web/20170424174703/https://books.google.ca/books?id=LRRiuFfr9UkC&pg=PA728|archive-date=24 April 2017}}</ref> | prevention = | treatment = Avoiding cold, calcium channel blockers, iloprost<ref name=NEJM2016/> | medication = | prognosis = | frequency = 4% of people<ref name=NEJM2016/> | deaths = | named after = Maurice Raynaud }} <!-- Definition and symptoms -->

'''Raynaud syndrome''', also known as '''Raynaud's phenomenon''', is a medical condition in which the spasm of small arteries causes episodes of reduced blood flow to end arterioles.<ref name=NIH2014What/> Typically the disease affects the fingers, and, less commonly, the toes, though it rarely also affects the nose, ears, nipples, or lips.<ref name=NIH2014What>{{cite web |title=What Is Raynaud's?|url= http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud |website= nhlbi.nih.gov | publisher= National Heart, Lung, and Blood Institute, National Institutes of Health | place= US |access-date=1 October 2016|date=21 March 2014|archive-url=https://web.archive.org/web/20161004225518/http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud|archive-date=4 October 2016}}</ref> The episodes classically result in the affected part turning white and then blue.<ref name=NIH2014Sym/> Often, numbness or pain occurs.<ref name=NIH2014Sym/> As blood flow returns, the area turns red and burns.<ref name=NIH2014Sym/> The episodes typically last minutes but can last several hours.<ref name=NIH2014Sym/> The condition is named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862.<ref name= mystery>{{cite journal | title= Maurice Raynaud (1834-1881) and the Mystery of 'Raynaud's Phanomenon' | journal= Dtsch Med Wochenschr | date= December 2019 | volume= 144 | number= 25 | pages= 1778–1783 | language= de | first1= Ulrich | last1= Koehler | first2= Irene | last2= Portig | first3= Olaf | last3= Hildebrandt | first4= Niklas Alexander | last4= Koehler | pmid= 31847013 | doi= 10.1055/a-0869-9899 | s2cid= 209409136 }}</ref>

<!-- Cause and diagnosis --> Episodes are typically triggered by cold or emotional stress.<ref name=NIH2014Sym>{{cite web|title=What Are the Signs and Symptoms of Raynaud's?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/signs |website= nhlbi.nih.gov | publisher= National Heart, Lung, and Blood Institute, National Institutes of Health| place= US |access-date=1 October 2016|date=21 March 2014|archive-url= https://web.archive.org/web/20161005190513/http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/signs |archive-date=5 October 2016}}</ref> Primary Raynaud's is idiopathic (spontaneous and of unknown cause) and not correlated with another disease. Secondary Raynaud's is diagnosed given the presence of an underlying condition and is associated with an older age of onset.<ref name="NEJM2016">{{cite journal |last1=Wigley |first1=FM |last2=Flavahan |first2=NA |date=11 August 2016 |title=Raynaud's Phenomenon |journal=The New England Journal of Medicine |volume=375 |issue=6 |pages=556–65 |doi=10.1056/nejmra1507638 |pmid=27509103}}</ref> In comparison to primary Raynaud's, episodes are more likely to be painful and asymmetric and progress to digital ulcerations.<ref>{{Cite journal |last=Temprano |first=Katherine K |date=March 2016 |title=A Review of Raynaud's Disease |journal=Missouri Medicine |publisher=Missouri State Medical Association |volume=113 |issue=2 |page=124 |issn=0026-6620 |oclc=790281069 |pmc=6139949 |pmid=27311222 }}</ref> Secondary Raynaud's can be due to a connective-tissue disorder such as scleroderma or lupus, injuries to the hands, prolonged vibration, smoking, thyroid problems, and certain medications, such as birth control pills and stimulants.<ref>{{cite web|title=What Causes Raynaud's?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/causes|website= nhlbi.nih.gov | publisher= National Heart, Lung, and Blood Institute, National Institutes of Health| place= US |access-date=1 October 2016|date=21 March 2014|archive-url=https://web.archive.org/web/20161004225530/http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/causes|archive-date=4 October 2016}}</ref> Diagnosis is typically based on the symptoms.<ref name=NEJM2016/>

<!-- Treatment and prognosis --> The primary treatment is avoiding the cold.<ref name=NEJM2016/> Other measures include the discontinuation of nicotine or other stimulant use.<ref name=NEJM2016/> Medications for treatment of cases that do not improve include calcium channel blockers and iloprost.<ref name=NEJM2016/> There is little evidence that alternative medicine reduce symptoms.<ref name=NEJM2016/> Severe Raynaud's may in rare cases lead to complications, specifically skin sores or gangrene.<ref name=NIH2014Sym/>

<!-- Epidemiology and history --> About 4% of people have the condition.<ref name=NEJM2016/> Onset of the primary form is typically between ages 15 and 30. The secondary form usually affects older people.<ref name="NIH2014Epi">{{cite web |date=21 March 2014 |title=Who Is at Risk for Raynaud's? |url=http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/atrisk |archive-url=https://web.archive.org/web/20161005190503/http://www.nhlbi.nih.gov/health/health-topics/topics/raynaud/atrisk |archive-date=5 October 2016 |access-date=1 October 2016 |website=nhlbi.nih.gov |publisher=National Heart, Lung, and Blood Institute, National Institutes of Health |place=US }}</ref> Both forms are more common in cold climates.<ref name=NIH2014Epi/>

== Signs and symptoms == thumb|Raynaud's affecting all five fingers thumb|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 (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 yet not all patients see all of the aforementioned color changes in all episodes, especially in milder cases of the condition. The red flush is due to reactive 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.<ref>{{cite journal |vauthors=Holmen OL, Backe B |journal=BMJ |year=2009 |volume=339 |article-number=b2553 |doi=10.1136/bmj.b2553 |title=An underdiagnosed cause of nipple pain presented on a camera phone|s2cid=71701101 }}</ref> [[File:Nipple-blanching.jpg|thumb|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.<ref name="pmid16609626">{{cite journal |vauthors=Pistorius MA, Planchon B, Schott JJ, Lemarec H |title=[Heredity and genetic aspects of Raynaud's disease] |language=fr |journal=Journal des Maladies Vasculaires |volume=31 |issue=1 |pages=10–5 |date=February 2006 |pmid=16609626 |url=http://www.masson.fr/masson/MDOI-JMV-01-2006-31-1-0398-0499-101019-200517601 |doi=10.1016/S0398-0499(06)76512-X |access-date=6 February 2010 |archive-date=28 March 2020 |archive-url=https://web.archive.org/web/20200328154841/http://www.masson.fr/masson/MDOI-JMV-01-2006-31-1-0398-0499-101019-200517601 |url-access=subscription }}</ref> In a large genetic study two genes were identified that predispose for the condition: ADRA2A (alpha-2A-adrenergic receptor for adrenaline) and the transcription factor IRX1.<ref>{{Cite web |last=Max-Delbrück-Centrum |first=Berliner Institut für Gesundheitsforschung-Charité und |date=2023-10-12 |title=First Genetic Causes of Raynaud's Phenomenon Discovered - News - BIH at Charité |url=https://www.bihealth.org/en/notices/first-genetic-causes-of-raynauds-phenomenon-discovered |access-date=2025-01-04 |website=Berliner Institut für Gesundheitsforschung - Charité und Max-Delbrück-Centrum |language=en}}</ref>

Smoking increases the 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.<ref>{{cite journal|last1=Wigley|first1=Fredrick M.|last2=Flavahan|first2=Nicholas A.|date=2016-08-10|title=Raynaud's Phenomenon|journal=New England Journal of Medicine|language=EN|volume=375|issue=6|pages=556–565|doi=10.1056/nejmra1507638|pmid=27509103}}</ref>

=== 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:<ref>{{cite journal |last1=Stringer |first1=Thomas |last2=Femia |first2=Alisa N. |date=2018-07-01 |title=Raynaud's phenomenon: Current concepts |url=https://www.sciencedirect.com/science/article/pii/S0738081X1830066X |journal=Clinics in Dermatology |series=Rheumatologic Dermatology |language=en |volume=36 |issue=4 |pages=498–507 |doi=10.1016/j.clindermatol.2018.04.007 |pmid=30047433 |s2cid=51720201 |issn=0738-081X |access-date=26 November 2022 |archive-date=2 May 2024 |archive-url=https://web.archive.org/web/20240502094925/https://www.sciencedirect.com/science/article/abs/pii/S0738081X1830066X |url-status=live |url-access=subscription }}</ref> * '''Connective tissue disorders''': ** Scleroderma<ref name="pmid17218139">{{cite journal |author=Gayraud M |title=Raynaud's phenomenon |journal=Joint, Bone, Spine |volume=74 |issue=1 |pages=e1–8 |date=January 2007 |pmid=17218139 |doi=10.1016/j.jbspin.2006.07.002}}</ref> ** Systemic lupus erythematosus ** Rheumatoid arthritis ** Sjögren's disease ** Dermatomyositis ** Polymyositis ** Mixed connective tissue disease ** Cold agglutinin disease ** Ehlers-Danlos syndrome * '''Eating disorders''': ** Anorexia nervosa * '''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 vaccines whose primary ingredient is the Anthrax Protective Antigen ** Stimulant medications, such as those used to treat ADHD (amphetamine and methylphenidate)<ref>{{cite journal | vauthors = Goldman W, Seltzer R, Reuman P | year = 2008 | title = Association between treatment with central nervous system stimulants and Raynaud's syndrome in children: A retrospective case–control study of rheumatology patients | journal = Arthritis & Rheumatism | volume = 58 | issue = 2| pages = 563–566 | doi=10.1002/art.23301| pmid = 18240233 | doi-access = }}</ref> ** OTC pseudoephedrine medications (Chlor-Trimeton, Sudafed, others)<ref name=":0">{{cite web|title = Raynaud's disease Treatments and drugs |publisher= Mayo Clinic |url = http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/treatment/con-20022916|website = mayoclinic.org|access-date = 13 December 2015|url-status = live|archive-url = https://web.archive.org/web/20151212054619/http://www.mayoclinic.org/diseases-conditions/raynauds-disease/basics/treatment/con-20022916|archive-date = 12 December 2015}}</ref> * '''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 ** Empty nose syndrome ** Multiple sclerosis ** Erythromelalgia (clinically presenting as the opposite of Raynaud's, with hot and warm extremities, often co-exists in patients with Raynaud's<ref>{{cite journal |vauthors=Berlin AL, Pehr K |title=Coexistence of erythromelalgia and Raynaud's phenomenon |journal=Journal of the American Academy of Dermatology |volume=50 |issue=3 |pages=456–60 |date=March 2004 |pmid=14988692 |doi=10.1016/S0190-9622(03)02121-2}}</ref>) ** 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 syndrome 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|date=June 2022}}

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|date=June 2022}}

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.<ref>{{cite journal |author=Priollet P |title=[Raynaud's phenomena: diagnostic and treatment study] |language=fr |journal=La Revue du Praticien |volume=48 |issue=15 |pages=1659–64 |date=October 1998 |pmid=9814067}}</ref>

==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 a cold atmosphere. In all cases, the primary cause is an underlying hyperactivation of the sympathetic nervous system.<ref>{{cite journal |last1=Cooke |first1=John P |last2=Marshall |first2=Janice M|author-link2= Janice Marshall |title=Mechanisms of Raynaud's disease |journal=Vascular Medicine |date=November 2005 |volume=10 |issue=4 |pages=293–307 |doi=10.1191/1358863x05vm639ra|pmid=16444858 }}</ref> Although, with different types, the exact pathophysiology differs. In the primary type, there is an increase in sensitivity due to the reasons mentioned above resulting in vasoconstriction. In the secondary type, normal activity of blood vessels is disrupted due to the same reasons mentioned above causing vasoconstriction which leads to ischemia and tissue death.<ref>{{Citation |last1=Musa |first1=Rina |title=Raynaud Disease |date=2022 |url=https://www.ncbi.nlm.nih.gov/books/NBK499833/ |work=StatPearls |place=Treasure Island (FL) |publisher=StatPearls Publishing |pmid=29763008 |access-date=2022-12-15 |last2=Qurie |first2=Ahmad |archive-date=29 November 2022 |archive-url=https://web.archive.org/web/20221129124648/https://www.ncbi.nlm.nih.gov/books/NBK499833/ |url-status=live }}</ref>thumb|right|Underlying mechanism

== Diagnosis == [[File:A thermal image demonstrating the loss of heat in a Reynaud's sufferer.jpg|thumb|Thermogram depicting a Raynaud's hand (top) and a healthy hand (bottom): Red indicates warm areas whilst green indicates cool areas.]] thumb|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 several 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.<ref name=guide>{{cite journal |last1=Belch |first1=Jill |last2=Carlizza |first2=Anita |last3=Carpentier |first3=Patrick H. |last4=Constans |first4=Joel |last5=Khan |first5=Faisel |last6=Wautrecht |first6=Jean-Claude |last7=Visona |first7=Adriana |last8=Heiss |first8=Christian |last9=Brodeman |first9=Marianne |last10=Pécsvárady |first10=Zsolt |last11=Roztocil |first11=Karel |date=2017-09-12 |title=ESVM guidelines – the diagnosis and management of Raynaud's phenomenon |journal=VASA. Zeitschrift für Gefässkrankheiten |volume=46 |issue=6 |pages=413–423 |doi=10.1024/0301-1526/a000661 |pmid=28895508 |issn=0301-1526|doi-access=free }}</ref>

If suspected to be secondary to systemic sclerosis, one tool which may help aid in the prediction of systemic sclerosis is thermography.<ref name="pmid17018538">{{cite journal |vauthors=Anderson ME, Moore TL, Lunt M, Herrick AL |title=The 'distal-dorsal difference': a thermographic parameter by which to differentiate between primary and secondary Raynaud's phenomenon |journal=Rheumatology |volume=46 |issue=3 |pages=533–8 |date=March 2007 |pmid=17018538 |doi=10.1093/rheumatology/kel330|doi-access=free }}</ref>

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 mmHg is diagnostic (positive). * Doppler ultrasound to assess blood flow * Full blood count may reveal a normocytic anaemia suggesting the 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.<ref>{{cite journal |vauthors=Brennan P, Silman A, Black C |title=Validity and reliability of three methods used in the diagnosis of Raynaud's phenomenon. The UK Scleroderma Study Group|journal=British Journal of Rheumatology|volume=32|issue=5|pages=357–361|date=May 1993|pmid=8495253|doi=10.1093/rheumatology/32.5.357}}</ref><ref>{{cite journal |author=Wigley FM|title=Clinical Practice.Raynaud's phenomenon|journal=New England Journal of Medicine|volume=347|issue=13|pages=1001–1008|date=September 2002|pmid=12324557|doi=10.1056/nejmcp013013}}</ref><ref>{{cite journal |vauthors=LeRoy EC, Medsger TA |title=Raynaud's phenomenon: a proposal for classification|journal=Clinical and Experimental Rheumatology|volume=10|issue=5|pages=485–488|date=September–October 1992|pmid=1458701}}</ref><ref>{{cite journal |vauthors=Maricq HR, Weinrich MC |title= Diagnosis of Raynaud's phenomenon assisted by color charts|journal=Journal of Rheumatology|volume=15|issue= 3|pages=454–459|date=March 1998|pmid=3379622}}</ref> Table 1 below provides a summary of these various diagnostic criteria.<ref name="Maverakis E, Patel F, Kronenberg D 60–65">{{cite journal |vauthors=Maverakis E, Patel F, Kronenberg D |title=International consensus criteria for the diagnosis of Raynaud's phenomenon |journal=Journal of Autoimmunity|volume= 48–49|pages= 60–5|date= 2014|pmid=24491823 |pmc=4018202 |doi=10.1016/j.jaut.2014.01.020}}</ref>

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.<ref name="Maverakis E, Patel F, Kronenberg D 60–65" />

== 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.<ref name="Mikuls" />

===Medications=== Medications can be helpful for moderate or severe disease. * Vasodilators – calcium channel blockers, such as the dihydropyridines nifedipine or amlodipine, preferably slow-release preparations – are often first-line treatment.<ref name="Mikuls">{{cite book |last1= Mikuls|first1= Ted R |last2= Canella|first2= Amy C|last3=Moore|first3=Gerald F|last4=Erickson|first4=Alan R|last5=Thiele|first5=Geoffery M|last6=O'Dell|first6=James R |title= Rheumatology|year= 2013|publisher= Manson Publishing|location= London|isbn= 978-1-84076-173-3|page= 117|chapter= Connective Tissue Diseases}}</ref> 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.<ref name="pmid3881908">{{cite journal |vauthors=Smith CR, Rodeheffer RJ |title=Raynaud's phenomenon: pathophysiologic features and treatment with calcium-channel blockers |journal=The American Journal of Cardiology |volume=55 |issue=3 |pages=154B–157B |date=January 1985 |pmid=3881908 |doi=10.1016/0002-9149(85)90625-3}}</ref> The limited evidence available shows that calcium-channel blockers are only slightly effective in reducing how often the attacks happen.<ref>{{cite journal|last1=Ennis|first1=Holly|last2=Hughes|first2=Michael|last3=Anderson|first3=Marina E.|last4=Wilkinson|first4=Jack|last5=Herrick|first5=Ariane L. |date=25 February 2016|title=Calcium channel blockers for primary Raynaud's phenomenon|journal=The Cochrane Database of Systematic Reviews|volume=2|issue=2 |article-number=CD002069|doi=10.1002/14651858.CD002069.pub5|issn=1469-493X |pmid= 26914257 |pmc=7065590}}</ref> Although, other studies also reveal that CCBs may be effective at decreasing the severity of attacks, pain, and disability associated with Raynaud's phenomenon.<ref>{{cite journal| last1=Rirash |first1= Fadumo |last2= Tingey|first2=Paul C|last3=Harding|first3=Sarah E|last4=Maxwell|first4=Lara J|last5=Tanjong Ghogomu|first5=Elizabeth|last6=Wells|first6=George A|last7=Tugwell|first7=Peter|last8=Pope| first8=Janet| date=2017-12-13|title=Calcium channel blockers for primary and secondary Raynaud's phenomenon|journal=Cochrane Database of Systematic Reviews|volume=2017|issue=12 |article-number=CD000467|doi=10.1002/14651858.cd000467.pub2|pmid=29237099|pmc=6486273|issn=1465-1858}}</ref> People whose disease is secondary to erythromelalgia often 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.<ref name="Mikuls" /> * Sympatholytic agents, such as the alpha-adrenergic blocker prazosin, may provide temporary relief to secondary Raynaud's phenomenon.<ref name="Mikuls" /><ref>{{cite journal|last1=Harding|first1=Sarah E| last2= Tingey |first2= Paul C|last3=Pope|first3=Janet|last4=Fenlon|first4=D|last5=Furst|first5=Dan|last6=Shea|first6=Beverley|last7=Silman|first7=Alan|last8=Thompson|first8=A|last9=Wells|first9=George A | date=1998-04-27|title=Prazosin for Raynaud's phenomenon in progressive systemic sclerosis|journal=Cochrane Database of Systematic Reviews|volume=1998 |issue=2|article-number=CD000956|doi=10.1002/14651858.cd000956|pmid=10796398|issn=1465-1858|pmc=7032637}}</ref> * Angiotensin receptor blockers, such as Losartan, or ACE inhibitors may aid blood flow to the fingers,<ref name="Mikuls" /> and some evidence shows that angiotensin receptor blockers (often losartan) reduce frequency and severity of attacks,<ref name="pmid9408065">{{cite journal |vauthors=Pancera P, Sansone S, Secchi S, Covi G, Lechi A |title=The effects of thromboxane A2 inhibition (picotamide) and angiotensin II receptor blockade (losartan) in primary Raynaud's phenomenon |journal=Journal of Internal Medicine |volume=242 |issue=5 |pages=373–6 |date=November 1997 |pmid=9408065 |doi=10.1046/j.1365-2796.1997.00219.x|s2cid=2052430 }}</ref> and possibly better than nifedipine.<ref name="pmid10616013">{{cite journal |author=Dziadzio M |title=Losartan therapy for Raynaud's phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial |journal=Arthritis and Rheumatism |volume=42 |issue=12 |pages=2646–55 |date=December 1999 |pmid=10616013 |doi=10.1002/1529-0131(199912)42:12<2646::AID-ANR21>3.0.CO;2-T|author2=Denton CP |author3= Smith R |last4=Howell |first4=Kevin |last5=Blann |first5=Andrew |last6=Bowers |first6=Emma |last7=Black |first7= Carol M. |publisher=Elsevier Saunergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider |doi-access=free }}</ref><ref name="pmid762741">{{cite journal |author=Waldo R |title=Prazosin relieves Raynaud's vasospasm |journal=JAMA |volume=241 |issue=10 |page=1037 |date=March 1979 |pmid=762741 |doi=10.1001/jama.241.10.1037}}</ref> * 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.<ref name="Mikuls" /> * Statins have a protective effect on blood vessels, and SSRIs such as fluoxetine may help symptoms, but the data is weak.<ref name="Mikuls" /> * 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.<ref name="Raynaud's treatments">{{cite journal | vauthors = Linnemann B, Erbe M | title = Raynaud's phenomenon and digital ischaemia – pharmacologic approach and alternative treatment options | journal = VASA. Zeitschrift für Gefässkrankheiten | volume = 45 | issue = 3 | pages = 201–12 | date = 2016 | pmid = 27129065 | doi = 10.1024/0301-1526/a000526 | quote = Phosphodiesterase inhibitors (e.g., sildenafil) can also improve [Raynaud's phenomenon] symptoms and ulcer healing }}</ref>

=== Surgery === * In severe cases, an endoscopic thoracic sympathectomy procedure can be performed.<ref name="pmid17098681">{{cite journal |author=Wang WH |title=Peripheral sympathectomy for Raynaud's phenomenon: a salvage procedure |journal=The Kaohsiung Journal of Medical Sciences |volume=22 |issue=10 |pages=491–9 |date=October 2006 |pmid=17098681 |doi=10.1016/S1607-551X(09)70343-2 |author2=Lai CS |author3=Chang KP |last4=Lee |first4=Su-Shin |last5=Yang |first5=Chih-Chiang |last6=Lin |first6=Sin-Daw |last7=Liu |first7=Chia-Ming|doi-access=free }}</ref> 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, 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<ref>{{cite journal |author=Neumeister MW |title=Botox therapy for ischemic digits |journal=Plastic and Reconstructive Surgery |volume=124 |issue=1 |pages=191–201 |date=July 2009 |pmid=19568080 |doi=10.1097/PRS.0b013e3181a80576 |author2=Chambers CB |author3=Herron MS |last4=Webb |first4=Kelli |last5=Wietfeldt |first5=Joel |last6=Gillespie |first6=Jessica N. |last7=Bueno |first7=Rueben A. |last8=Cooney |first8=Carisa M.|s2cid=26698472 }}</ref> 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<ref>{{cite journal |vauthors=Van Beek AL, Lim PK, Gear AJ, Pritzker MR |title=Management of vasospastic disorders with botulinum toxin A |journal=Plastic and Reconstructive Surgery |volume=119 |issue=1 |pages=217–26 |date=January 2007 |pmid=17255677 |doi=10.1097/01.prs.0000244860.00674.57|s2cid=8696332 }}</ref> 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 acupuncture and laser therapy.<ref name=NEJM2016/>

== Prognosis == The prognosis of primary Raynaud syndrome is often excellent, with no factor to mortality and little morbidity overall. In some very rare cases, gangrene has been known to develop. The prognosis of secondary Raynaud is related to the course of the underlying disease, and how effective blood flow-restoring maneuvers are.<ref>{{cite journal| title= Raynaud Phenomenon: Practice Essentials, Pathophysiology, Etiology| url= https://emedicine.medscape.com/article/331197-overview#a6| website= Medscape.com| date= August 4, 2022| first= Heather| last= Hansen-Dispenza| publisher= | access-date= October 4, 2023| archive-date= 11 October 2017| archive-url= https://web.archive.org/web/20171011073058/http://emedicine.medscape.com/article/331197-overview#a6| url-status= live}}</ref>

== References == {{Reflist}}

== External links == {{Commons category}} * [https://web.archive.org/web/20060709152907/http://www.nhlbi.nih.gov/health/dci/Diseases/raynaud/ray_what.html What Is Raynaud's Disease] at the National Heart, Lung, and Blood Institute * [https://www.niams.nih.gov/health-topics/raynauds-phenomenon Questions and Answers About Raynaud's Phenomenon] at the National Institutes of Health * {{cite journal |vauthors=Bakst R, Merola JF, Franks AG, Sanchez M |date=October 2008 |title=Raynaud's Phenomenon: Pathogenesis and Management |url=https://www.jaad.org/article/S0190-9622(08)00653-1/abstract |journal=Journal of the American Academy of Dermatology |volume=59 |issue=4 |pages=633–53 |doi=10.1016/j.jaad.2008.06.004 |pmid=18656283}}

{{Medical condition classification and resources | DiseasesDB = 25933 | ICD10 = {{ICD10|I|73|0|i|70}} | ICD9 = {{ICD9|443.0}} | OMIM = 179600 | MedlinePlus = 000412 | eMedicineSubj = med | eMedicineTopic = 1993 | MeshID = D011928 }} {{Vascular diseases}} {{Authority control}}

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