HEALTHLINE
The extracellular matrix glues the body together, holds it up against gravity, gives it and all its organs their unique shapes, and cushions cells and organs by absorbing water.
Symptoms and Causes
Because the proteins of the extracellular matrix vary in structure and function and because different autoimmune disorders cause the immune system to selectively attack different proteins, each of the autoimmune connective tissue disorders has its own unique set of symptoms. The names of the various disorders reflect these symp- toms. The formal name of the most common form of lupus, systemic lupus erythematosus, for example, describes a disc-shaped skin rash that is characteristically red (erythematous) and heals from the inside out. During the thirteenth century, a physician named Rogerius likened the physical appearance of this rash to the bite of a wolf and gave the condition the name lupus, which means “wolf” in Latin. The word “systemic” describes the potential for the ailment to affect any organ system in the body, producing fever, joint pain, fatigue, sores in the nose or mouth, painful breathing, hair loss, skin rashes, abdominal pain, epilepsy, and even psychoses.
Different names are applied to some lupus cases, but the disease
process in all types is the same. Lupus can be very mild and involve only the skin. Patients at this end of the spectrum of the disease are said to have discoid lupus, and while most of them will not progress to more severe forms, there is no way to predict the long-term course of the disease. Drug-induced lupus can be triggered by prolonged use of hydralazine (a drug used to treat high blood pressure), procainamide and quinidine (drugs used to treat irregular heart- beats), isoniazid (a drug used to treat tuberculosis), and less com- monly by over two dozen other drugs. Neonatal lupus affects new- born children of mothers with lupus.
Who Is at Risk?
How common is lupus? Estimates place the number of Americans with the disease at less than one-half of one percent, and each year, more than 16,000 new cases are reported in the United States. Worldwide, close to 5 million people have some form of lupus. Racial differences in susceptibility occur, with black people and Asians being at higher risk than white people. Lupus, like many other autoimmune diseases, is also more common in women, especially during childbearing years and menopause, suggesting that the fe- male XX combination of chromosomes renders the body more sus- ceptible to immune attack than the male XY pair does. Susceptibility to acquired connective tissue disorders can also run in families, but genetic predisposition alone does not produce signs or symptoms of these diseases, which are activated by external triggers. While the triggers in acquired connective tissue disorders
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