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Raynaud’s Disease

Raynaud’s phenomenon is characterized by paroxysmal reversible episodes of vasospam , usually involving peripheral small vessela of the fingers or toes and resulting in a triple-colour chage starting with pallor and followed by cyanosis and erythema 
( Linnemann B , Erbe M 2015 )

How common –Described in 1862 by Maurice Raynaud , he thought this was ischaemic in nature Highly prevalent in the general population , 3-21 % depending upon climate BMJ paper in 2012 cites ‘ non-population based studies of prevalence show that 3-12.5 % of men and 6-20 % of women report symptoms of Raynaud’s phenomenon Climate is known to influence the prevalence of Raynaud’s phenomenon Several studies have shown that it is more common in women.

Fingers turn white
( ischaemia )-Then they turn blue 
( Cyanosis )-Then red
( reperfusion )

What happens ? Exact pathogenesis is not understood yet completely and is complex .
 Herrick AL in 2012 has listed the possible pathogenesis under the following factors

Vascular – Balance between vasodilatation and vasoconstriction is disturbed
 impaired vasodilatation increased vasconstriction stuctural abnormalities
( 2ary RP )

Neural –Patients describe worsening of RP symptoms with stress.
Role of CNS is being studied and it is likely that the autonomic nervous system has a significant role via both central and peripheral mechanisms. Variety of neurotransmitters and their receptors are possibly involved

Intravascular –Several circulating factors have been implicated particularly in patients with systemic sclerosis and include platelet activation , impaired fibrinolysis , white blood cell activation , redcued RB deformability ad oxidative stress

Primary Raynauds’s phenomena –Called primary when no identifiable cause can be found More than 90 % of cases of RP are primary This is a benign condition It does not lead to irreversible tissue injury It tends to begin at a younger age that secondary RP No trophic changes are seen in PRP PRP is more common in women ( 9 : 1 ) PRP overall prevalence is about 10 % PRP typically develops at a younger age Typical PRP patient is a young female in her teens and twenties with a familial predisposition Majority of patients seen in primary care would have PRP

Secondary Raynaud’s phenomena-Much rarer compared to PRP When the condition is secondary to an underlying illness for e.g connective tissue disease ( CTD ) it is termed secondary A wide range of conditions can be responsible for 2ary RP which includes CTD , autoimmune diseases , occupational causes ( vibration 
white fingers ) , drugs , endocrine , neoplasm 
( hypothyroidism ) and miscellaneous causes as Beurger’s disease About 65 % causes of 2ary RP are thought to be due to scleroderma & over 90 % of people with SSc have RP Unlike PRP patients with secondary RP can suffer with digital ulceration , scarring and gangrene

Presentation-ask when did the symptoms start how frequent suspect if patients describes well demarcated episodic blanching of one or more fingers ( full triphasic color change may not always be seen but blanching must happen for a diagnosis of RP ) if the attacks are associated with
- numbness
- paraesthesia on rewarming or pain what precipitates the attack ( triggers )
- most common trigger is considered to be exposure to cold
- due to stress/ emotion
- even minor changes in temperature can precipitate an attack ( for e.g moving from a warm environment to an air-conditioned room or holding a cold milk bottle
- emotional stress
- injury due to vibrations or forcible trauma
- prolonged use of digits for e.g typing What parts are affected ?
- in primary RP the attacks are usually symmetrical
- thumb is generally not affected and if involved this raises the suspicion of an underlying CTD ( Connective tissue disease )
- typically the colour change start at the tip of the finger and spreads downwards or to more digits
- although uncommon other body parts may also be affected as the tip of the nose , ear lobes , tongue and nipples The symptoms can last for minutes to up to several hours Ask about family history ( up to 25 % may have a 1st degree relative with RP ) Smoking Full medication history ( such as beta blocker ) Occupational history

Evaluation –check fingers and toes particularly looking for
-digital ulceration
- pitting scars
- sclerodactyly ( tight hardened fingers Sclero.org )
- synovitis


Digital ulceration is indicative of a secondary RP
 check skin ( e.g malar rash ) and nail ( for any nail fold or trophic changes )
 Check peripheral pulses Blood pressure in both arms Nail fold capilloroscopy- examines microcirculation Ask the patient for any photos , video that they may have taken during an episode ( well demarcated color changes are considered by some to a diagnostic hallmark of RP and a photographic evidence would support the diagnosis )

Tests –FBC ESR ANA screen , Us & Es and urinalysis Other tests based on underlying suspicion

Differentials –acrocyanosis erythromelalgia perniosis and Chill-blain Lupus livedo-reticularis carpal tunnel syndrome sub-clavian steal syndome embolism ( for e.g from thoracic outlet syndrome ) malignancies cryo diseases ( cryoglobulinaemia , cryofibrogenaemia and cold agglutinin disease ) hyperviscocity syndromes hepatitis associated vasculitis

International consensus criteria for the diagnosis of Raynaud’s phenomenon has proposed a 3 step approach

Screening questionnaire –Are your fingers usually sensitive to cold ?
Assess colour change –Occurrence of biphasic color change during the vasospastic episodes ( white and blue )
Assess severity- episodes triggered by things other than cold involve both hands , even if the involvement is asynchronous and or / asymmetric accompanied by numbness &/or paraesthesia well demarcated border between affected and non-affected skin photo supports RP diagnosis they can also occasionally involve other body parts as nose , ears , feet and areola triphasic color change during vasospastic episodes

Primary Raynauds phenomenon diagnostic criteria Meets the 3 step criteria Capillorscopy ( CKS suggests the nail folds could be seen using a dermatoscope ) No physical finding suggestive of a secondary cause as ulceration , tissue necrosis , gangrene , sclerodactyly , calcinosis or skin fibrosis No h/o existing CTD Negative or low titter ANA ( Adapted from ‘ International consensus criteria for the diagnosis of Raynaud’s phenomenon E Maverakis et al Journal of Autoimmunity 48049 ( 2014 ) 60-65 )

Primary Care Management-Advice information , 
lifestyle interventions and support- Most patients with PRP can be managed in primary care If RP is drug related consider stopping if possible avoiding cold exposure keeping warm – hand warmers , gloves patient education smoking cessation regular exercise address stress / emotional issues

Nifedipine- Although a large number of agents are available CKS recommends a trial of Vasodilator in the form of Nifedipine as a prophylaxis for adults > 18 Dose may range from
5 mg tds up to 20 mg tds for immediate release
10 mg for MR up to 60 mg OD Amlodipine could be used as next line if Nifedipine is not tolerated .

Referral to secondary care –A suggestion that the RP may be of secondary origin as
- digital ulcers , gangrene , ischaemia of one or more fingers
- late onset of symptoms – for e.g after 30 yrs
- asymmetric , intense painful symptoms
- any other feature which may support a diagnosis of CTD for e.g + ve ANA or a positive test for ant–SCL -70 antibodies children < 12 ( CKS NHS and BMJ ) treatment fails and symptoms getting worse despite Occupational cause of RP diagnostic uncertainty. Severe digital ischaemia- arrange urgent admission

PATIENT INFORMATION

Scleroderma & Raynaud’s UK – a large UK charity https://www.sruk.co.uk/raynauds/what-raynauds/

American College of Rheumatology printable 2 page leaflet https://www.rheumatology.org/Portals/0/Files/Raynauds-Phenomenon-Fact-Sheet.pdf

National Heart Lung & Blood Institute – a detailed page on Raynaud’s https://www.nhlbi.nih.gov/health-topics/raynauds

Versus arthritis 20 page leaflet on Raynaud’s phenomenon https://www.versusarthritis.org/media/1263/raynauds-phenomenon-information-booklet.pdf

Scleroderma Org on RP https://www.scleroderma.org/site/DocServer/Raynaud.pdf?docID=322

Living with Raynaud’s Disease from Eurordis https://www.eurordis.org/news/living-with-scleroderma-and-raynauds-disease

Circulation Foundation on Raynaud’s and Systemic Sclerosis https://www.circulationfoundation.org.uk/help-advice/other-vascular-information/raynauds-systemic-sclerosis

Patient support group https://www.raynauds.org/

 

References

  1. The pathogenesis, diagnosis and treatment of Raynaud phenomenon Ariane L. Herrick Herrick, A. L. Nat. Rev. Rheumatol. 8, 469–479 (2012); published online 10 July 2012; doi:10.1038/nrrheum.2012.96 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4018202/
  2. Diagnosis and management of  Raynaud’sphenomenon Beth Goundry foundation year2doctor,Laura Bell general practice specialist traineeyear2, Matthew Langtree core medical traineeyear2, Arumugam Moorthy consultant rheumatologist BMJ2012;344:e289doi:10.1136/bmj.e289(Published7February2012) https://www.bmj.com/content/bmj/344/bmj.e289.full.pdf
  3. CKS NHS Raynaud’s phenomenon Last revised in February 2020 https://cks.nice.org.uk/raynauds-phenomenon#!diagnosisSub:2
  4. Review ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon Writing group Jill Belch1, Anita Carlizza2, Patrick H. Carpentier3, Joel Constans4, Faisel Khan1, and Jean-Claude Wautrecht5 Vasa (2017), 46 (6), 413–423 https://doi.org/10.1024/0301-1526/a000661 https://econtent.hogrefe.com/doi/pdf/10.1024/0301-1526/a000661
  5. The pathogenesis, diagnosis and treatment of Raynaud phenomenon Ariane L. Herrick Herrick, A. L. Nat. Rev. Rheumatol. 8, 469–479 (2012); published online 10 July 2012; doi:10.1038/nrrheum.2012.96 https://www.nature.com/articles/nrrheum.2012.96.pdf
  6. Raynaud’s phenomenon Cristiane KayserI; Marcelo José Uchôa CorrêaII; Luís Eduardo Coelho  AndradeRevista Brasileira de Reumatologia Print version ISSN 0482-5004On-line version ISSN 1809-4570
  7.  A. L. Herrick, Pathogenesis of Raynaud’s phenomenon, Rheumatology, Volume 44, Issue 5, May 2005, Pages 587–596, https://doi.org/10.1093/rheumatology/keh552
  8.  Current medical and surgical management of Raynaud’s syndrome Gregory J. Landry , MD, Portland Ore J Vasc Surg 2013 ;57 :1710-6. https://www.jvascsurg.org/article/S0741-5214(13)00598-3/pdf
  9. Pope, Janet. “Raynaud’s phenomenon (primary).” BMJ clinical evidence vol. 2013 1119. 10 Oct. 2013
  10. Raynaud’s phenomenon – assessment and differential diagnoses. Linnemann B1, Erbe M. 2015 May;44(3):166-77. doi: 10.1024/0301-1526/a000426. ( Abstract )
  11.  Differential diagnosis of Raynaud’s phenomenon based on modeling of finger thermoregulation Ismail E1, Orlando G, Corradini ML, Amerio P, Romani GL, Merla A. 2014 Apr;35(4):703-16. doi: 10.1088/0967-3334/35/4/703. Epub 2014 Mar 12.( Abstract )
  12. Differential diagnoses of Raynaud’s phenomenon Ahrazoglu M1, Moinzadeh P1, Hunzelmann N
    2014 May;139(20):1064-9. doi: 10.1055/s-0034-1370036. Epub 2014 May 6. ( Abstract )
  13. Raynaud’s phenomenon differential diagnosis  epocrates via https://online.epocrates.com/diseases/19335/Raynaud-phenomenon/Differential-Diagnosis

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