Systemic lupus erythematosus

Article Abstract:

Systemic lupus erythematosus, or simply lupus, is a disease with a wide range of clinical manifestations, including a characteristic facial rash, oral ulcers, abnormal kidney function, a tendency toward certain inflammatory processes such as pleurisy, and abnormalities of blood products. Lupus falls into the category of autoimmune disease, in which the body's immune system suddenly ceases to recognize parts of the body (or more accurately, cells of the body) as being part of itself. The immune system then develops antibodies to parts of itself; these autoantibodies attack those parts as if they were foreign. While the exact cause of lupus has yet to be determined, its precipitants are being recognized. Some genetic predisposition exists, in that the disease is three to four times more common in blacks than in whites. Women are far more prone than men to develop the disease, and studies have suggested that the presence of androgens, or male sex hormones, is protective against lupus in individuals who might otherwise be susceptible. People with lupus have an overproduction of B cells, the white blood cells that produce antibodies. They have a relative shortage of T cells, another group of white blood cells that can modify the immune response. Cytokines, substances that act in concert with immune cells, are often present at abnormal levels in people with lupus. Certain environmental triggers can precipitate lupus flares or attacks. These include ultraviolet light, certain drugs, including procainamide and hydralazine, and some evidence suggests a role for viruses. People with lupus show greater evidence of exposure to certain viruses, but none has yet been shown to cause the disease. Hair dyes contain certain chemicals that can be absorbed through the scalp and trigger a lupus flare. The evidence to date suggests that certain people have a very strong genetic predisposition to lupus, and they need little in the way of environmental triggers for their disease to become active. Others have a weaker genetic predisposition, and they are more likely to have their disease activated by strong environmental triggers. (Consumer Summary produced by Reliance Medical Information, Inc.)

Physiological aspects, Development and progression, Autoimmune diseases, Panel Discussion, Lupus erythematosus

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The acute lupus hemophagocytic syndrome

Article Abstract:

Systemic lupus erythematosus (SLE) is a connective tissue disease that causes inflammation, and can affect the skin, joints, blood, heart, kidneys, and lymph nodes. Common symptoms include fever, joint pain, skin lesions, weakness and fatigue. Changes that occur in the blood of SLE patients include the destruction of red blood cells (hemolysis) and an alteration in the ability of the blood to clot (coagulopathy). Unusual features of this disease include a rapid and severe reduction in all types of blood cells (fulminant pancytopenia), and the destruction of red blood cells (hemophagocytic syndrome). When these features occur in association with SLE, the condition is referred to as lupus hemophagocytic syndrome. This article describes the clinical features and treatment of six patients with lupus hemophagocytic syndrome. All the patients fulfilled the criteria of the American Rheumatism Association for the diagnosis of SLE. Their symptoms included fever, skin rash and abdominal pain, and they had abnormally low levels of blood platelets (a type of blood cell involved in blood clot formation). Tissue samples revealed inflammation of the lymph nodes in two patients, inflammation of the kidneys in two patients and inflammation of the blood vessels in the skin and appendix in two patients. Treatment with prednisone restored the number of blood cells to normal in all patients within three to seven days. Hemophagocytic syndromes have also been identified in association with viral, bacterial and fungal infections, and with tumors. Patients with hemophagocytic syndrome should be tested for SLE to allow early diagnosis; patients with SLE should be treated with steroids. Hemophagocytic syndromes caused by infections should not be treated with steroids. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Case studies, Complications and side effects, Systemic lupus erythematosus, Prednisone

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