Raynaud's phenomenon is often the first sign of the scleroderma disease process. With this condition, small blood vessels narrow in the fingers, toes, ears, and even the nose.
Attacks of Raynaud's phenomenon can occur several times a day, and are often brought on or worsened by exposure to cold. Warmth relieves these attacks. In severe cases, attacks can develop regardless of the temperature. Severe cases may also cause open sores or damage to the skin and bones, if the circulation is cut off for too long. Stress also can trigger the syndrome.
Typically, the fingers go through three color changes:
Tingling and pain can occur in the affected regions.
Raynaud's is very common and occurs in 3 - 5% of the general population. It's important to note that more than 80% of patients with Raynaud's phenomenon do not have scleroderma, lupus, rheumatoid arthritis, or other serious illnesses. Raynaud's is more likely to be a symptom of scleroderma or some other connective tissue disease if it develops after age 30, if it is severe, and if it is accompanied by other symptoms (such as skin changes and arthritis).
Course of Typical Skin Changes. The primary symptoms of scleroderma occur in the skin. They often take the following course:
Other Skin Changes. The following skin symptoms may also occur:
Changes in bones, joints, and muscles can cause the following symptoms:
Complications in the Upper Digestive Tract.
Complications in the Lower Digestive Tract. Complications in the lower digestive tract are uncommon. If they do occur, they can include the following:
Many patients, however, have few or even no lower gastrointestinal symptoms.
In severe cases, the lungs may be affected, causing shortness of breath or difficulty in taking deep breaths. Shortness of breath may be a symptom of pulmonary hypertension, an uncommon but life-threatening complication of systemic scleroderma.
Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in scleroderma patients. Two major lung conditions associated with scleroderma, pulmonary fibrosis and pulmonary hypertension, can occur either together or independently.
Interstitial Pulmonary Fibrosis. Scleroderma involving the lung causes scarring (pulmonary fibrosis). Pulmonary fibrosis occurs in about 70% of scleroderma patients, although its progression is very slow and patients have a wide range of symptoms:
Pulmonary fibrosis also places the patient at higher risk for lung cancer. This condition may be due to severe dysfunction in the esophagus, which causes patients to aspirate tiny amounts of stomach acid.
The most important indication of future worsening in the lungs appears to be inflammation in the small airways (alveolitis). Doctors detect alveolitis by using a lung test called bronchoalveolar lavage.
Pulmonary Hypertension. Pulmonary hypertension is the narrowing of the pulmonary arteries in the lung. The narrowing of the arteries creates resistance to blood flow and increases the workload of the heart. The heart becomes enlarged from pumping blood against this resistance. Some symptoms of pulmonary hypertension include shortness of breath, chest pain, weakness, and fatigue. Shortness of breath,the primary symptom of pulmonary hypertension, worsens over time. The goal of treatment is to control the symptoms, although the disease usually develops into congestive heart failure.
Pulmonary hypertension can develop in one of two ways:
Click the icon to see an image of cor pulmonale.
Signs of kidney problems, such as increased levels of protein in the urine and mild high blood pressure (hypertension), are common in scleroderma. As with pulmonary hypertension, the degree of severity depends on whether the kidney problems are acute or chronic.
Slow Progression. The typical course of kidney involvement in scleroderma is a slow progression that may produce some damage but does not usually lead to kidney failure.
Renal Crisis. The most serious kidney complication in scleroderma is renal crisis. It is a rare event that occurs in a small number of patients with diffuse scleroderma, most often early in the course of the disease. This syndrome includes a life-threatening condition called malignant hypertension, a sudden increase in blood pressure that can cause rapid kidney failure. This condition may be fatal. However, if the condition is successfully treated, it rarely recurs.
Until recently, renal crisis was the most common cause of death in scleroderma. Aggressive treatment with drugs that lower blood pressure, particularly those known as ACE inhibitors, is proving to be successful in reducing this risk.
Many patients with even limited scleroderma have some sort of functional heart problem, although severe complications are uncommon and occur in only about 15% of patients with diffuse scleroderma. As with other serious organ complications, they are more likely to occur within 3 years after the disease begins.
Fibrosis of the Heart. The most direct effect of scleroderma on the heart is fibrosis (scarring). It may be very mild or it can cause pain, low blood pressure, or other complications. By damaging muscle tissue, the scarring increases the risk for heart rhythm problems, problems in electrical conduction, and heart failure. The membrane around the heart can become inflamed, causing a condition called pericarditis.
Pulmonary hypertension and hypertension associated with kidney problems in scleroderma can also affect the heart.
Other complications of scleroderma may include the following:
Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917-1928.
Feldman M, Friedman LS, Brandt LJ. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders; 2006.
Henness S, Wigley FM. Current drug therapy for scleroderma and secondary Raynaud's phenomenon: evidence-based review. Curr Opin Rheumatol. 2007;19:611-618.
Knobler RM, French LE, Kim Y, Bisaccia E, Graninger W, Nahavandi H, et al. A randomized, double-blind, placebo-controlled trial of photopheresis in systemic sclerosis. J Am Acad Dermatol. 2006;54:793-799.
Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440-447.
Nash RA, McSweeney PA, Crofford LJ, et al. High-dose immunosuppressive therapy and autologous hematopoietic cell transplantation for severe systemic sclerosis: long-term follow-up of the US multicenter pilot study. Blood. 2007;110:1388-1396.
Nihtyanova SI, Denton CP. Current Approaches to the Management of Early Active Diffuse Scleroderma Skin Disease.Rheumatic Dis Clin North Am. 2008;34(1):34(1):161-79; viii
Ostojic P, Cerinic MM, Silver R, Highland K, Damjanov N. Interstitial lung disease in systemic sclerosis. Lung. 2007;185:211-220.
Rubin LJ. Treatment of Pulmonary Arterial Hypertension Due to Scleroderma: Challenges for the Future.Rheumatic Dis Clin North Am. 2008;34(1):191-197; viii.
Schachna L, Medsger TA Jr., Dauber JH, Wigley FM, Braunstein NA, White B, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2006;54:3954-3961.
Shoenfeld Y, Katz U. IVIg therapy in autoimmunity and related disorders: our experience with a large cohort of patients. Autoimmunity. 2005 Mar;38(2):123-37.
Steen VD. Pregnancy in scleroderma. Rheum Dis Clin North Am. 2007;33:345-358.
Tashkin DP, Elashoff R, Clements PJ, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006; 354(25):2655-66.
Thombs BD, Taillefer SS, Hudson M, Baron M. Depression in patients with systemic sclerosis: a systematic review of the evidence. Arthritis Rheum. 2007;57:1089-1097.
Tyndall A, Furst DE. Adult stem cell treatment of scleroderma. Curr Opin Rheumatol. 2007;19:604-610.
Wigley FM. Scleroderma (Systemic Sclerosis). In: Goldman L, Ausiello D. Goldman: Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders, 2008. pp. 2032-2041.
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