Overview of Raynaud's
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)
Photo: Severe attack of Raynaud's in a systemic scleroderma patient with both blue and white fingers (and also calcinosis on one finger).
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 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.
The Vascular Microenvironment and Systemic Sclerosis. The role of the vascular microenvironment in the pathogenesis Systemic Sclerosis (SSc) is appreciated clinically as Raynaud's syndrome with capillary nail bed change. This manifestation of vasculopathy is used diagnostically in both limited and diffuse cutaneous subsets of SSc, and is thought to precede fibrosis. Tracy Frech. Int J Rheumatol. 2010. (Also see: Vascular Involvement)
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.
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.
Raynaud's Phenomenon of the Nipples
Raynaud's can affect the nipples of breastfeeding mothers, causing severe pain.
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.
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)
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