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Overview: What is Raynaud's?

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
What is Raynaud's?
Photos of Attacks
Disease vs. Phenomenon
With Scleroderma
With Other Diseases
Raynaud's of the Nipples
Raynaud's of the Lungs
Doubles Risk of Heart Problems
Importance of Controlling
Other Ways Scleroderma Can Affect Hands
Raynaud's in Children

Overview: What is Raynaud's?


Overview of Raynaud's
in Systemic Scleroderma
Presented by Amanda Thorpe

Raynaud's is a vascular disorder 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. (Also see: What is Scleroderma?, Types of Scleroderma and Systemic Symptoms)

Raynaud'sPhoto: 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.

Photos of Raynaud's Attacks

Most Raynaud's attacks are not this visible. Usually attacks cause the skin to go dead white. However, attacks 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 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.
Systemic sclerosis without antinuclear antibodies (ANA) or Raynaud's phenomenon (RP): a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. Prospective studies are needed to elucidate the clinical presentation, evolution and outcome of such patients. PubMed, Rheumatology. 2013 Mar;52(3):560-7. (Also see: What is Scleroderma? and Antibodies in Systemic Scleroderma)
Thumb Involvement in Raynaud's Phenomenon as an Indicator of Underlying Connective Tissue Disease. 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. JRheum. March 1, 2010.
Vascular Disease and Systemic Scleroderma. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN.
Overview
Vascular in Scleroderma
Causes
Types in Scleroderma
In Other CTDs
Biomarkers
Angiogenesis
Hypoxia
VEGF
Personal Stories
Related Information

Raynaud's and Other Diseases

The association of antiphospholipid antibodies (APLA) with cardiopulmonary manifestations of systemic sclerosis. APLA is associated with pulmonary arterial hypertension (PH), interstitial lung disease, Raynaud's phenomenon and digital ulcers, so endothelial abnormalities and small vessel thrombosis may be important in the pathogenesis of these disease features. PubMed, Clin Exp Rheumatol, 2014 Feb 24. (Also see: Antiphospholipid Syndrome, Causes of Pulmonary Hypertension , and Digital Ulcers)

Raynaud's Phenomenon of the Nipples

Raynaud's can affect the nipples of breastfeeding mothers, causing severe pain.
Raynaud's Phenomenon of the Nipple Associated with Labetalol Use. Precipitants for Raynaud's phenomenon of the nipple are known to be cold temperatures, caffeine, and emotional stress. J Hum Lact, February 2013 vol. 29 no. 1 17-19.

Raynaud's in the Lungs

Raynaud's may also affect the tip of the nose, earlobes, cheeks, chin, or lungs. Some people with scleroderma and Raynaud's experience coughing attacks when breathing cold air or drinking/eating cold liquids and foods. The coughing may or may not be caused by pulmonary vasospasms caused by exposure to cold.

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

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.

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)
 
Go to Raynaud's: Diagnosis
 

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