Systemic lupus erythematosus

Libman-Sacks disease
Lupus is a chronic inflammatory disease that affects various parts of the body, especially the skin, joints, blood, and kidneys. For most sufferers, lupus is a mild disease affecting only a few organs. For others, it may cause serious and even life-threatening problems.

Lupus appears in one of two forms: (1) Discoid lupus erythematosus (lupus vulgaris), which can be chronic but is less severe, may cause a red, scaly rash, usually on the face; (2) Systemic lupus erythematosus (SLE or lupus) is the most common and severe form of lupus.

Systemic lupus erythematosus (SLE) is an autoimmune disease. It can affect many body systems and is extremely variable in severity. It is also a rheumatic (arthritic) disease and can affect the joints, muscles, skin, kidneys, nervous system, lungs and heart. But manifestations are individual and sometimes vague and fluctuating with time, and the disease is is difficult to diagnose. In the 1950s, an average person diagnosed with lupus had a 50% probability of death within 5 years. Diagnosed today, and with improved understanding and management practices, a lupus patient has an 80% to 90% chance of living a normal life span.

Nearly all people with lupus experience changes in disease activity. At times, the disease may flare; at other times, there may be no evidence of lupus at all (remission). Fever, weight loss and fatigue may be among the first signs of the illness. A skin rash may develop on the face, neck or arms, especially after exposure to the sun. This rash may involve the nose and cheeks and appear as a butterfly-shaped rash. Ulcers, usually painless, can occur in the mouth or nose. Painful swelling of the joints and prolonged stiffness in the morning can occur. Inflammation of the surface of some organs (serositis) such as the heart and lungs can cause painful breathing or shortness of breath. The kidneys can be affected without producing symptoms, although swelling (oedema) of the legs can occur. Lupus affecting the kidneys may cause protein and blood in the urine and high blood pressure. People with lupus may experience depression or difficulty in concentrating, either due to the disease itself or as a reaction to living with a chronic disease. Rarely, seizures can occur.

Lupus affects women eight to ten times more frequently than men and often first appears in women of child-bearing years (ages 18 to 45). Women of colour (African-American, Native American, Asian and Hispanic) are affected more often than Caucasian women. Lupus also can affect children, the elderly and men. It is rare in children under 5 years; it more commonly affects teenagers but is still an uncommon condition for younger people. Rashes, kidney inflammation (nephritis) and joint inflammation are the most likely problems seen in children with SLE.

There are an estimated 500,000 to 1.5 million people in the USA with lupus. In Korea it is estimated that there are about 150,000 women and men suffering from the disease. However, there are only 100,000 that have been recognized/diagnosed. Other 50 thousand are either not treated or not diagnosed.

(G) Very specific problems