Types of Lupus
Reviewed by: HU Medical Review Board | Last reviewed: January 2020 | Last updated: March 2021
Lupus is a chronic, autoimmune disease that causes the immune system to attack healthy tissue, which results in inflammation and pain. It can be mild or quite severe. The skin, joints and internal organs are most often affected but any part of the body can be damaged by the inflammation that lupus generates.
Lupus strikes women 9 times more than men, and women of color, especially African American women, the most. It tends to develop between the ages of 15 and 44. Approximately 1.5 million people in the U.S. and 5 million worldwide have lupus. There are 4 different types of lupus and a condition known as pre-lupus:1
Systemic lupus erythematosus (SLE)
Systemic lupus erythematosus (SLE) is by far the most common form of the disease, accounting for roughly 70 percent of people diagnosed with lupus. It is called “systemic” because it can affect any area of the body or the whole system. The kidneys, joints, and skin are most often involved. In 50 percent of people with lupus, a major organ such as the heart, kidneys, lungs or brain, are affected.1
Some common complications of this form of lupus are:
- Lupus nephritis, or inflammation of the kidneys
- Inflammation of the nervous system and brain, which can cause headaches, memory problems, confusion, fever, seizures, and strokes
- Hardening of the arteries (arteriosclerosis) which can cause heart attacks
Cutaneous lupus erythematosus (CLE)
While skin disease is a common symptom of lupus, cutaneous lupus erythematosus (CLE) is a different type of lupus that impacts just the skin. CLE sometimes occurs by itself and sometimes in addition to SLE. Some researchers believe that CLE is actually more common than SLE.
There are 3 types of cutaneous lupus:
- Acute cutaneous lupus erythematosus (ACLE) which includes the distinctive malar (butterfly) rash
- Chronic cutaneous lupus erythematosus (CCLE) which includes discoid lupus erythematosus (DLE) and chilblain lupus erythematosus
- Subacute cutaneous lupus erythematosus (SCLE)
Lupus panniculitis (lupus profundus) is a rare subtype of CLE. Most are adults between 20 and 60 years of age, with firm, long-lasting nodules, and plaques on the head and body.2
Each form of cutaneous lupus has its own unique type of skin lesions and patterns. Uncontrolled CLE may cause long-term damage to the skin such as scarring or loss of pigment. Up to 30 percent of people with one form of CLE have multiple types of CLE. 1,3,4
Drug-induced lupus erythematosus
Drug-induced lupus accounts for approximately 10 percent of all those with lupus. As the name implies, it is caused by high doses of certain medicines. The drugs that most often cause lupus-like symptoms include hydralazine and diltiazem (for high blood pressure), procainamide (for cardiac arrhythmia), isoniazid (for malaria), sulfa drugs and minocycline (antibiotics), and TNF inhibitors (for inflammation).
The symptoms of drug-induced lupus are similar to SLE lupus except that the symptoms tend to go away when the drug is stopped. This is a rare form of lupus and only occurs in about 10 percent of cases.3-6
Neonatal lupus is very rare and represents about 10 percent of lupus cases. It occurs when the mother’s antibodies attack the baby in the womb. Only about 40 percent of women who give birth to children with neonatal lupus have lupus themselves, but they may go on to develop it or Sjogren’s syndrome (dry eye) later in life. What the mothers do carry are the antibodies anti-Ro, anti-La or anti-RNP.3
Only 1 to 2 percent of infants born with neonatal lupus experience congenital heart block, which is often treated with a pacemaker. The heart problems, rash, liver problems, and low blood cell counts that appear at birth usually disappear after six months. Congenital heart block is largely preventable with close monitoring and treatment when mothers are known to be at risk. Despite the name, neonatal lupus is not considered true lupus.1,3
Doctors now believe that some people progress from a sort of pre-lupus, or asymptomatic autoimmunity, to incomplete lupus erythematosus, to full-blown SLE lupus. They show some of the signs and symptoms of SLE lupus but do not meet the official criteria for lupus. About 20 percent of those with pre-lupus eventually develop lupus, though it is not understood why. These people tend to be older than those with SLE lupus and do not have high ANA titers, but do have arthritis in more than 4 joints and blood disorders.6
Some patients with pre-lupus, who have some lupus symptoms but do not meet the full criteria, may be diagnosed with undifferentiated connective tissue disease (UCTD).
Diagnosing lupus can be tricky since so many of its symptoms mimic other conditions. However, once lupus is diagnosed, there are many treatments available that can slow the progression of the disease.