Systemic sclerosis (scleroderma): supplement

Article Abstract:

There has been a great deal of research dealing with every aspect of scleroderma, so it is appropriate that the November 1991 supplement to the Annals of the Rheumatic Diseases on systemic sclerosis and scleroderma is being published. Coverage of every aspect has not been possible in the accompanying articles. Rather, the effects of the disease on organ systems has been outlined, with particular attention to the lungs. Medical concerns are addressed as well. Information on mechanisms underlying the disease are discussed in the articles on immunogenetics, endothelium (cells that form the lining of blood vessels), and genes controlling collagen (major structural protein in the body). The word scleroderma means ''hard skin'' and now refers to a number of syndromes ranging from localized scleroderma (morphea) to generalized scleroderma and chemical-induced disorders. Generalized scleroderma, or systemic sclerosis, is characterized by overproduction of connective or fibrous tissue containing collagen, combined with widespread vascular (blood vessel) damage. Symptoms of skin involvement can vary, making classification of subtypes of the disease difficult. Early diagnosis is important, as full-blown disease is difficult to stabilize or reverse. Most cases of generalized systemic sclerosis occur in patients with Raynaud's phenomenon, in which the hands become white and then blue (cyanotic) on exposure to cold or emotion. Two tests in these patients are highly predictive of whether they will develop systemic sclerosis. The ability to identify patients at risk for systemic disease, the methods of evaluating the disease, and the ability to accurately estimate future organ involvement need to be improved. It is likely that multiple factors cause systemic sclerosis, and the genetic and environmental influences are discussed in this supplement. In addition, a specific immune dysfunction occurring at disease onset also seems likely. Before logical treatment of systemic sclerosis at different stages can be developed, much basic knowledge about the cause and development of the disease needs to be known. (Consumer Summary produced by Reliance Medical Information, Inc.)

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Systemic sclerosis: is there a treatment yet?

Article Abstract:

Systemic sclerosis is a disease characterized by the hardening of organs and tissues due to the formation of fiber-like tissue. This disease is difficult to treat because: (a) it varies in severity, progression, and nature; (b) it may improve spontaneously in the later stages, particularly among a large proportion of patients with benign disease; (c) the cause and disease process are not understood; and (d) criteria for evaluating improvement or deterioration of the disease are lacking. A logical approach to the use of drugs in treating systemic sclerosis is discussed. Before treatment, patients must be classified by type of systemic sclerosis. This disease has been divided into the diffuse cutaneous form, involving widespread effects, and the limited cutaneous subtypes, involving localized effects. Treatment should be based on the stage of the disease. The disease stage can be assessed by evaluating the levels of circulating antibodies, which are immune proteins in the blood, and the production of cytokines, which are regulatory factors. The identification of risk factors will help to prevent the progression of complications of systemic sclerosis, such as lung disease. The development of scleroderma, the thickening of the skin and other tissues, involves several processes including inflammation, abnormal immune reactions, and blood vessel damage. Various agents used to treat systemic sclerosis may be able to alter the physiological processes of this disease, but the disease is at this time considered untreatable, because no effective therapy has been found. (Consumer Summary produced by Reliance Medical Information, Inc.)

Drug therapy, Identification and classification

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Soluble thrombomodulin concentration is raised in scleroderma associated pulmonary hypertension

Article Abstract:

Patients with pulmonary hypertension caused by scleroderma have elevated blood levels of thrombomodulin. Thrombomodulin is an anticoagulant produced by blood vessels. It may be involved in the development of pulmonary hypertension, which is high blood pressure in the lungs.

Pulmonary hypertension

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