Raynaud's phenomenon
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Introduction
Reversible vasospasm of the digital arteries which may result in ischemia of the digits.
Etiology
- primary (idiopathic) Raynaud's disease (70%)
- Raynaud's phenomenon is secondary to other conditions
- connective tissue disease
- systemic sclerosis (90%)
- mixed connective tissue disease (85%)
- systemic lupus erythematosus (10-44%)
- Sjogren's syndrome (20%)
- rheumatoid arthritis (5%)
- dermatomyositis/polymyositis (20%)
- pharmacologic agents
- trauma/occupational
- 90% of loggers - vibration white finger
- carpal tunnel syndrome
- polyvinyl chloride
- jackhammer use
- electric shock
- cold injury
- piano playing
- arterial occlusive disease
- hyperviscosity diseases
- polycythemia
- cryoglobulinemia
- paraproteinemia, especially Waldenstrom's
- cryofibrinogenemia
- cold agglutinins
- thrombocytosis
- arterial disease
- atherosclerosis
- thromboangiitis obliterans (associated with smoking)
- tobaccoism (aggravated by cigarette smoking)
- neurologic disorders
- migraine syndrome (10%)
- primary pulmonary hypertension
- connective tissue disease
Epidemiology
- primary
- common
- most patients 15-20 years
- predominance of females
- affects 3-5% of male population, 4-9% of female population
- < 50% of affected individuals seek medical attention
- secondary
- most individuals > 35 years
- no sex preference
- suspect if patient is male, >35 or <15 years of age
- 8% of men, 11% of women (mean age 54 years)[8]
Pathology
- vasospasm[5]
- increased sympathetic tone or increased density or sensitivity of alpha 2-adrenergic receptors
- endothelial damage
- reduced release of nitric oxide & venodilation secondary to endothelial damage
- intimal proliferation
- fixed vascular obstruction
- inflammation & release of vasospastic mediators
- three phases
- pallor: vasospasm halts blood flow
- cyanosis: blood moving slowly through capillaries becomes deoxygenated
- redness: previously constricted arterioles dilate during hyperemic phase
Clinical manifestations
- primary Raynaud's phenomenon
- temperature-dependent episodic finger pain[5]
- painful digits exacerbated by exposure to cold temperature or emotional stress
- pallor often followed by cyanosis, then redness
- affects fingers more often than toes, may spare thumb
- may also affect earlobes, lips or tip of nose
- symptoms last 15-30 minutes after rewarming
- paresthesias may occur during rewarming
- secondary Raynaud's phenomenon
- symptoms similar to primary Raynaud's phenomenon, but may be more severe with rapid progression
- symptoms may be unilateral
- manifestations of precipitating etiology may be present
- digital pitting scar or gangrene may occur
- digital pitting & ulceration of the fingertips typically reflect ischemic injury[5]
- occurs rarely in primary (idiopathic) Raynaud disease but commonly in patients with secondary Raynaud phenomenon[5]
- most cases without underlying connective tissue disease are mild[8]
Criteria for diagnosis of primary Raynaud's phenomenon
- episodic attacks with acral pallor or cyanosis
- strong & symmetric peripheral pulses
- no pitting edema, gangrene or ulcerations
- normal finger & toenail capillaries
- antinuclear antibody (ANA) titer < 1:100 (i.e. 1:10)
- ESR < 20 mm/hr
Laboratory
- serology for connective tissue disease not routinely indicated for patients with primary Raynaud's phenomenon[5]
- yield is low & not cost effective, unless
- severe & prolonged vasospasms
- asymmetric involvement of digits
- digital pitting
- yield is low & not cost effective, unless
- antinuclear antibody (ANA) titer for secondary Raynaud's phenomenon[5]
Diagnostic procedures
- capillaroscopy for differentiation of primary from secondary Raynaud's phenomenon[5][13][18]
- abnormal nailfold capillaries are a sign of secondary Raynaud's phenomenon[5]
- decreased capillary density, tortuous capillaries with dilated loops, & flame hemorrhages may be seen
- abnormal nailfold capillaries are a sign of secondary Raynaud's phenomenon[5]
Complications
< 10% of patients with isolated Raynaud's phenomenon will develop systemic connective tissue disease within 10 years
Differential diagnosis
Management
- general measures
- avoid exposure to the cold
- protect hands & feet from cold & trauma
- cessation of smoking (smoking exacerbates Raynaud's phenomenon)
- avoid over the counter sympathomimetics
- pharmacologic agents
- dihydropyridine calcium channel blockers prior to precipitating event[11]
- anti-platelet agent
- topical nitroglycerin applied to fingers or toes
- prazosin
- reserpine
- guanethidine
- bosentan may be effective in preventing recurrences of digital ulcers in severe Raynaud's disease[5]
- vasodilator
- prostacyclin analogs
- intravenous epoprostenol for acutely ischemic digit(s)[5]
- iloprost (not available in US)
- improved symptoms of 47% of patients who failed nefedipine
- ACE inhibitors (controversial)
- sildenafil (Viagra) 50 mg BID[9]; tadalafil[14]
- controversial
- individualized effect taken before or during an episode[16]
- prostacyclin analogs
- pentoxifylline (controversial)
- serotonin receptor antagonists
- ketanserin (not available in US)
- phenoxybenzamine
- affects vasoconstriction & platelet aggregation
- chronic pain or digital ulceration failing conservative management
- sympathetic ganglion blockade
- Botox 100 units injected into the hand effective (84%)[15]
- prognosis
- primary Raynaud's phenomenon carries low risk of progression[5]
- 36% of cases without underlying connective tissue disease persist for 7 years[8]
More general terms
Additional terms
References
- ↑ Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 525-26
- ↑ Guide to Physical Examination & History Taking, 4th edition, Bates B, JB Lippincott, Philadelphia, 1987
- ↑ Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 288-90
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 874-75
- ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 Medical Knowledge Self Assessment Program (MKSAP) 11,14,16,17. American College of Physicians, Philadelphia 1998,2006,2012,2014
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ Principles of Ambulatory Medicine, 4th edition, Barker et al (eds), Williams & Wilkins, Baltimore, 1995, pg 893
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1139
- ↑ 8.0 8.1 8.2 8.3 Journal Watch 25(11):91, 2005 Suter LG, Murabito JM, Felson DT, Fraenkel L. The incidence and natural history of Raynaud's phenomenon in the community. Arthritis Rheum. 2005 Apr;52(4):1259-63. PMID: https://pubmed.ncbi.nlm.nih.gov/15818710
- ↑ 9.0 9.1 Prescriber's Letter 13(1): 2006 Viagra and Raynaud's phenomenon Detail-Document#: http://prescribersletter.com/(5bhgn1a4ni4cyp2tvybwfh55)/pl/ArticleDD.aspx?li=1&st=1&cs=&s=PRL&pt=3&fpt=25&dd=220111&pb=PRL (subscription needed) http://www.prescribersletter.com
- ↑ Hirschl M et al, Transition from primary Raynaud's phenomenon to secondary Phenomenon identified by diagnosis of an associated disease: Results of ten years prospective surveillance. Arthritis Rheum 2006; 54:1974 PMID: https://pubmed.ncbi.nlm.nih.gov/16732585
- ↑ 11.0 11.1 Prescriber's Letter 15(12): 2008 Drugs that Aggravate or Improve Raynaud's Phenomenon Detail-Document#: http://prescribersletter.com/(5bhgn1a4ni4cyp2tvybwfh55)/pl/ArticleDD.aspx?li=1&st=1&cs=&s=PRL&pt=3&fpt=25&dd=241207&pb=PRL (subscription needed) http://www.prescribersletter.com
- ↑ Neumeister MW et al. Botox therapy for ischemic digits. Plast Reconstr Surg 2009 Jul; 124:191. PMID: https://pubmed.ncbi.nlm.nih.gov/19568080
- ↑ 13.0 13.1 Lambova SN, Muller-Ladner U. The role of capillaroscopy in differentiation of primary and secondary Raynaud's phenomenon in rheumatic diseases: a review of the literature and two case reports. Rheumatol Int. 2009 Sep;29(11):1263-71. PMID: https://pubmed.ncbi.nlm.nih.gov/19547979
- ↑ 14.0 14.1 Shenoy PD, Kumar S, Jha LK et al Efficacy of tadalafil in secondary Raynaud's phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology (Oxford). 2010 Dec;49(12):2420-8 PMID: https://pubmed.ncbi.nlm.nih.gov/20837499
- ↑ 15.0 15.1 Wigley FM, Flavahan NA Raynaud's Phenomenon N Engl J Med 2016; 375:556-565. August 11, 2016 <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/27509103 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMra1507638
- ↑ 16.0 16.1 Roustit M, Giai J, Gaget O et al On-Demand Sildenafil as a Treatment for Raynaud Phenomenon: A Series of n-of-1 Trials. Ann Intern Med. 2018. Oct 30. <PubMed> PMID: https://pubmed.ncbi.nlm.nih.gov/30383134 <Internet> http://annals.org/aim/article-abstract/2709822/demand-sildenafil-treatment-raynaud-phenomenon-series-n-1-trials
- ↑ Choi E, Henkin S. Raynaud's phenomenon and related vasospastic disorders. Vasc Med. 2021;26:56-70. PMID: https://pubmed.ncbi.nlm.nih.gov/33566754
- ↑ 18.0 18.1 Lambova SN. The value of nailfold capillaroscopy in the classification and differential diagnosis of Raynaud's phenomenon in rheumatology. Curr Rheumatol Rev. 2024;20:108-114. PMID: https://pubmed.ncbi.nlm.nih.gov/37691224
- ↑ National Institute of Arthritis and Muscluloskeletal and Skin Diseases (NIAMS) Raynaud's Phenomenon https://www.niams.nih.gov/health-topics/raynauds-phenomenon