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RAYNAUD'S: MAIN MENU
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What is Raynaud's?

This page was written by Shelley Ensz, and has not yet been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer.
What is Raynaud's?
Photos of Raynaud's Attacks
Raynaud's Disease vs. Phenomenon
Raynaud's and Scleroderma
Raynaud's and Other Diseases
Raynaud's of the Nipple
Raynaud's in the Lungs
Doubles Risk of Heart Problems
Importance of Controlling Raynaud's
Other Ways SD Can Affect Hands
Raynaud's in Children
What is Raynaud's?

Raynaud'sRaynaud's is a vascular disease commonly found in sclerodema. It is an extreme spasm of blood vessels in response to cold or stress. The fingers and/or toes become white and/or blue, and may become red on re-warming.

Photo: Severe attack of Raynaud's in a systemic scleroderma patient with both blue and white fingers (and also calcinosis on one finger).

How do you pronounce "Raynaud's"? Raynard, Raynod, Raynoze, RAYnadds, Raynaws, Raynowds, RAYnoz, Rayners, RP, or Raywhatsit? ISN's Sclero Forums discussion.
Raynaud Phenomenon. eMedicine Journal.
Blunted increase of digital skin vasomotion following acetylcholine and sodium nitroprusside iontophoresis in systemic sclerosis (SSc) patients. This study showed a selective abnormality of the endothelial, sympathetic and myogenic-dependent finger skin vasomotion in SSc patients, consistent with a parallel endothelial, sympathetic and myogenic macrovascular dysfunction. M. Rossi. Rheumatology. April 22, 2008. (Vasomotion is cyclic variation in vascular diameter) (Also see: Digital Ulcers)
Raynaud's phenomenon. Raynaud's phenomenon (RP) is a vasospastic disorder characterized by episodic color changes of blanching, cyanosis, and hyperemia in response to cold and/or emotional stress. Secondary RP is characterized by an age of onset of more than 30 years, painful and asymmetric attacks, ischemic skin lesions, positive autoantibodies, capillaroscopic abnormalities and/or clinical features suggestive of connective tissue diseases (CTDs). Among the CTDs, systemic sclerosis has the highest frequency of RP. PubMed. Reumatismo. 2004 Apr-Jun;56(2):77-81.
Photos of Raynaud's Attacks
Most Raynaud's attacks are not this visible. Usually attacks cause the skin to go dead white. However, atttacks that incur blue fingers are much more dramatic and photogenic. In between attacks, the hands look normal.
Raynaud's Attack, Blue Fingers, by ISN Photo RepositoryISN Photo Repository: Raynaud's (Alexandra M.) ISN.
ISN Photo Repository: Raynauds (Joyce) ISN.
ISN Photo Repository: Raynauds (Judith). ISN.
Raynaud's in Fingertips by ISN Photo RepositoryISN Photo Repository: Raynaud's (Shelly S). ISN.
Gangrene and Amputations. ISN.
ISN Photo Repository: Finger Ulcers. ISN.
ISN Photo Repository (Main Page). ISN.
Raynaud's Disease vs. Raynaud's Phenomenon (or, Secondary Raynaud's)
Raynaud's occurs in 3 to 5% of the general population; however, more than 80% of patients with Raynaud's do not have a connective tissue disease or other serious illness. Scleroderma: Symptoms and Complications. University of Maryland Medical Center.
When Raynaud's appears by itself, without any other medical condition, it is called Raynaud's Disease. When it appears along with autoimmune diseases such as Scleroderma, Systemic Lupus Erythematosus, and Rheumatoid Arthritis, it is called Raynaud's Phenomenon (or, Secondary Raynaud's.)
In primary Raynaud's, the blood vessels return to normal afterwards. However, in Raynaud's with Scleroderma there may be blood vessel scarring which can make medication ineffective.Digital (Finger) Ulcers are caused by the lack of oxygen to skin cells.
Raynaud's Syndrome and Raynaud's Phenomenon. The primary form (Syndrome) occurs by itself, is not related to other diseases or conditions, and has no known cause. In contrast, the secondary form (Phenomenon) has the potential for serious complications and may be caused by another disease, such as rheumatoid arthritis and scleroderma. Dr. Rob Danoff. MSN Health and Fitness.
Raynaud Phenomenon. Raynaud phenomenon manifests as recurrent vasospasm of the fingers and toes and usually occurs in response to stress or cold exposure. Secondary Raynaud phenomenon should be distinguished from primary Raynaud phenomenon (Raynaud disease). A Anita Narayanan, MD. emedicine from WebMD. 06/03/09.
Primary Raynaud phenomenon and small-fiber neuropathy (SFN): is there a connection? A pilot neurophysiologic study. The presence of SFN raises the possibility that a subset of patients with primary RP have an underlying, subclinical small-fiber dysfunction. (SpringerLink) N. Manek. Rheumatology International. December 24 2009.
Health-Related Quality of Life (HRQL) In Primary Raynaud Phenomenon (RP). This study shows a reduction of the HRQL in patients with primary RP, compared with control subjects. The psychologic wellbeing represents the most involved area, whereas physical functioning and symptoms are significantly influenced by age and comorbidities. Moreover, these patients are more anxious/depressed. De Angelis, Rossella. Journal of Clinical Rheumatology. August 2008.
Color Doppler Ultrasonography of Hand and Finger Arteries to Differentiate Primary from Secondary Forms of Raynaud's Phenomenon (RP). Digital artery ultrasound depicts the same anatomical structures as angiography, but it is a cheaper, faster, and noninvasive method of differentiating primary versus secondary RP. Wolfgan A. Schmidt. J Rheumatol July 15 2008.
Transition from primary Raynaud's (RP) phenomenon to secondary Raynaud's phenomenon identified by diagnosis of an associated disease. Patients diagnosed initially as having primary RP may actually comprise 1 of 3 groups: those with idiopathic RP, those with a rather benign disease course, and those with a more severe course of the disease. Arthritis & Rheumatism Journal. Vol 54, Issue 6:1974-1981 June 2006.
Raynaud's and Scleroderma
Just 5 to 20 percent of people with Raynaud's phenomenon subsequently develop scleroderma, but about 90 percent of scleroderma patients experience this extreme cold sensitivity with red/blue flashes in their hands and feet as a primary manifestation of their disease. What Causes Raynaud's? National Heart Lung and Blood Institute. NIH.
In fact, Raynaud's is often the first symptom of scleroderma, and may precede the onset of Scleroderma and other scleroderma symptoms by years or by months. Raynaud's is a type of vascular disease.
Thumb Involvement in Raynaud’s Phenomenon as an Indicator of Underlying Connective Tissue Disease. In a retrospective study to assess whether the degree of thumb involvement differs between primary Raynaud’s phenomenon (PRP) and secondary Raynaud’s phenomenon (SRP), it was found that although the median temperature gradient along the thumb was not significantly different between SRP and PRP, the thumb is more likely to be involved in SRP than in PRP. Thumb involvement is one of a number of clinical indicators that should alert the clinician to the possibility of an underlying connective tissue disease/disorder. Batsi Chikura. JRheum. March 1, 2010.
Vascular Disease and Systemic Scleroderma. The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN.
Overview of Vascular Disease
Vascular Involvement in Scleroderma
Causes of Vascular Involvement
Types of Vascular Disease in Scleroderma
Vascular Disease in Other Connective Tissue Disease
Biomarkers in Vascular Disease

Angiogenesis and Vascular Disease
Hypoxia and Vascular Disease
Vascular Endothelial Growth Factor (VEGF)
Personal Stories

Related Information
Raynaud’s Phenomenon and Plasma Endothelin (ET-1): Correlations with Capillaroscopic Patterns in Systemic Sclerosis (SSc). Highest ET-1 plasma levels were detected in the more advanced stage of the SSc microangiopathy, namely the late nailfold videocapillaroscopy pattern, characterized by capillary loss and increased tissue fibrosis; this might support the involvement of ET-1 in the progression of the microvascular/fibrotic SSc damage. Alberto Sulli. Journal of Rheumatology. June 2009, 36 (6). (Also see: Causes of Scleroderma)
Systemic Sclerosis (SSc) Risk 60-Fold Higher in Raynaud's with SSc Antibodies, Nailfold Capillary Microscopy Changes. Positive antinuclear antibodies (ANAs), positive SSc autoantibodies, and characteristic nailfold capillary microscopy patterns can help identify patients with Raynaud's phenomenon who will progress to definite SSc. J. Kelly. Musculoskeletal Report. 12/08/09. (Also see: Antibodies)
Inflammatory morphea in the context of Raynaud phenomenon. Even in the absence of initial symptoms to support systemic disease, patients presenting with morphea in the setting of Raynaud phenomenon or anti-centromere antibodies deserve close surveillance for the possibility of CREST syndrome and systemic sclerosis. Naheed Abbasi MD MPH. Dermatology Online Journal. October 2008. (Also see: Morphea)
Prince: Prognostic Index For Nailfold Capillaroscopic Examination For Identifying Raynaud's Phenomenon (RP) At High Risk Of Developing Into A Scleroderma Spectrum Disorder. Our prognostic capillaroscopic index identifies RP patients at high risk of developing scleroderma spectrum disorders. Our results suggest that this index could be used in clinical practice, and its further inclusion in prospective studies will undoubtedly help to explore its potential in predicting treatment response. F. Ingegnoli. SAT0421.EULAR 2008. (Also see: Capillaroscopy)
Discrepancy Between Simultaneous Digital Skin Microvascular and Brachial Artery Macrovascular Post-Occlusive Hyperemia in Systemic Sclerosis. Microvascular function is impaired in SSc, whereas brachial artery endothelial function is normal. M. Roustit. Journal of Rheumatology. Vol 35 No 6 June 2008.
Relationship between capillaroscopic alterations and bone ultrasound parameters in patients with Raynaud phenomenon (RP). Our study suggest that RP, primarily in patients with scleroderma, modify phalangeal bone mass, bone density, and bone quality with a good correlation between nailfold patterns and quantitative ultrasound, independently from confounding variables. Alfredomaria Lurati, MD. The Internet Journal of Rheumatology. 2008. Vol 4 No 2. (Also see: Osteoporosis)
Serum nitric oxide (NO) metabolites and disease activity in patients with systemic sclerosis. Serum NO(x) in SSc patients were elevated compared to healthy controls. Serum NO(x) level was determined by multiple factors including age, prednisolone use, and elevated pulmonary arterial pressure. (PubMed) Clin Rheumatol. 2008 Mar;27(3):315-22. Epub 2007 Aug 15. (Also see: Nitric Oxide)
Hand vascular involvement assessed by magnetic resonance angiography in systemic sclerosis. Impairment of the microcirculation is a cardinal feature of systemic sclerosis (SSc). Magnetic resonance angiography (MRA) has improved the assessment of vascular lesions of the hand. The aim of this study was to evaluate vascular abnormalities in the hands of patients with SSc, using MRA. (Wiley InterScience) Arthritis & Rheumatism.Volume 56, Issue 8, Pages 2747 - 2754. July 30 2007. (Also see: Digital Ulcers)
Therapeutic management of acral manifestations of systemic sclerosis. Acral manifestations of systemic sclerosis include Raynaud's phenomenon, calcinosis cutis, and sclerodactyly. In the later stages of the disease, contractures of the skin and joints as well as obliterative vasculopathy leading to digital ulcers and necrotic lesions may occur. PubMed. Med Klin (Munich). 2007 Mar 15;102(3):209-18. (Also see: Calcinosis, Sclerodactyly, Digital Ulcers, and Skeletal Involvement: Joint Contractures)
Long Term Follow Up of a Cohort of Patients With Positive Antinuclear Antibodies (ANA) and Raynaud’s Phenomenon (RP) in the Absence of Connective Tissue Disease at Initial Evaluation. Initial evaluation indicates that there is a subset of patients who remain with only positive ANA and RP and do not develop a defined CTD. Marie S. O'Brien. 1881/553 ACR 2006. (Also see: Antibodies)
Smokers With Systemic Sclerosis Risk Severe Digital Vascular Complications. Patients with systemic sclerosis (SSc) who smoke are three to four times more likely than are never-smokers to incur digital vascular complications, researchers in England have found. Doctor's Guide. 1/24/03 Arthritis & Rheumatism 2002;46:12:3312-3316. (Also see: Digital Ulcers)
Raynaud's and Other Diseases
Blue fingers linked with breathlessness. The coincidence of recent-onset Raynaud's disease with the pulmonary findings suggested the possibility of a connective tissue disorder. Lawrence J. Kagen, MD. Clinical Advisor. 01/21/10.
Vasospastic individuals demonstrate significant similarity to glaucoma patients as revealed by gene expression profiling in circulating leukocytes. This result indicates a potential predisposition of vasospastic individuals to glaucomatous optic nerve atrophy. The targeted expression profiles might be further considered for early/predictive glaucoma diagnosis. Molecular Vision, Nov 2009. (Also see: Normal Tension Glaucoma)
The Temporal Relationship of Raynaud's Phenomenon (RP) and Features of Connective Tissue Disease in Rheumatoid Arthritis (RA). Idiopathic RP may have a different clinical effect on RA than secondary RP; the latter is correlated with more severe RA. Sclerodactyly is associated with erosive arthritis and RP in RA. Janet E Pope. J Rheumatol First Release. Oct 1 2008. (Also see: Rheumatoid Arthritis and Sclerodactyly)
Raynaud's Phenomenon of the Nipple
Raynaud's Phenomenon of the Nipple: A Treatable Cause of Painful Breastfeeding. Raynaud's phenomenon has been reported to affect the nipples of breastfeeding mothers and is recognized by many lactation experts as a treatable cause of painful breastfeeding. Because the breast pain associated with Raynaud's phenomenon is so severe and throbbing, it is often mistaken for Candida albicans infection. PubMed. Pediatrics. 2004 Apr;113(4):E360-4.
Raynaud's in the Lungs
Raynaud's may also affect the tip of the nose, earlobes, cheeks, chin, or lungs. Breathing cold air can cause a coughing attack if there is Raynaud's in the lungs. The lungs are the internal organ most frequently affected by Raynaud's.
Raynaud's phenomenon (RP) is correlated with elevated systolic pulmonary arterial pressure (PASP) in patients with systemic lupus erythematosus (SLE). In patients with SLE, the presence of RP was associated with elevation in PASP. Further investigation is needed to clarify the significance of this relation. Lupus (2007) 16, 505—508. (Also see: Lupus)
Doubles Risk of Coronary Artery Disease and Stroke
A 1998 study indicated that people who have Raynaud's may have double the risk of coronary artery disease and stroke. "Hopkins: Is Raynaud's a Predictor of Heart Disease?" by Fredrick M. Wigley, M.D., Professor of Medicine and Director of Molecular and Clinical Rheumatology, Johns Hopkins School of Medicine, August 19, 1998, Aetna InteliHealth.
Coronary Artery Disease HeartPoint Gallery
Discrepancy Between Simultaneous Digital Skin Microvascular and Brachial Artery Macrovascular Post-Occlusive Hyperemia in Systemic Sclerosis (SSc) . Microvascular function is impaired in SSc, whereas brachial artery endothelial function is normal. M. Roustit. J Rheumatol 2008; 35:1576–83. (Also see: Cardiac Involvement)
Importance of Controlling Raynaud's
Scleroderma and Raynaud's can in some instances become so severe as to require amputation of fingers and/or toes, so keeping "aggressively warm" plus promptly treating all Raynaud's attacks is essential for all Scleroderma patients. As Raynaud's  progresses, slight drops in temperature may trigger it with longer duration of the attacks.
Additionally, recent research indicates that Raynaud's might be part of how the disease process advances in Scleroderma, due to the oxygen deprivation caused by Raynaud's spurring an abnormal chemical reaction which produces oxygen radicals. Therefore, taking great precautions to prevent Raynaud's attacks could be of paramount importance for Scleroderma patients.
Management of cutaneous vascular complications in systemic scleroderma: experience from the German network. Management of Raynaud's Phenomenon and ulcerations in SSc were heterogeneous at initiation of the network, reflecting a sometimes insufficient use of the diagnostic and therapeutic possibilities. (PubMed) Rheumatol Int. 2008 Mar 5. (Also see: Digital Ulcers)
Other Ways Scleroderma Can Affect Hands
Raynaud's isn't the only way in which Scleroderma can affect the hands. Hand involvement may include skin tightening and hardening, finger contractures, Telangiectasia, Calcinosis, and arthritis. See Skin Involvement.
Raynaud's in Children
Raynaud's phenomenon in children: a retrospective review of 123 patients. RP in children, as in adults, principally affects girls and is frequently free of association with connective tissue disease. Antinuclear antibody positivity and abnormal nailfold capillaries correlate with secondary disease. Antiphospholipid antibodies are surprisingly common, a new finding of uncertain implications. PubMed. Pediatrics 2003 Apr;111(4 Pt 1):715-21. (Also see: Juvenile Scleroderma)
 
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