Lupus

Lupus is a chronic condition where symptoms tend to cycle in flares and remissions. There is no known cure but numerous treatments are available.

What is lupus?

Systemic lupus erythematosus (SLE), commonly known as lupus, is an autoimmune illness that affects many organs and systems in the body. Lupus is a chronic condition, but symptoms tend to cycle in alternate periods of "flares" (or "flares-ups") and remissions. There is no known cure, but numerous treatments are available.

In a person with lupus, the immune system, which normally protects the body from foreign invaders and infection, malfunctions and instead attacks a person's own healthy body tissues. Lupus can lead to numerous health complications and, in serious cases, can even become life-threatening.

What causes lupus?

The exact cause of lupus is unknown, but most scientists believe that genetics, combined with outside triggers – such as infections, medications or other environmental factors – lead to the development of this disease.

What are the different types of lupus?

There are multiple recognized forms of lupus:

• Systemic lupus erythematosus (SLE) is the disease most commonly mentioned, and the most serious since it involves the entire body.
• Discoid lupus is a skin-only illness in which a specific rash, mostly a scarring rash of circular-shaped lesions, occurs without other symptoms (SLE patients sometimes have the same rash).
• Subacute cutaneous lupus involves a more widespread rash that is often worsened by sun exposure. It is associated with a limited form of SLE in about 50% of cases.
• Drug-induced lupus is relatively rare and is triggered by certain medications, such as hydralazine and some anti-seizure medications. Drug-induced lupus causes joint pain in about 90% of cases, rash in 20%, and inflammation of the lining of the heart or lung in 15%. It typically goes away if the drug is stopped.
• Neonatal lupus occurs in infants whose mothers have specific blood test findings, but not in infants of mothers who have SLE but do not have these blood test findings. Its symptoms usually consist mainly of a transient, unimportant rash and blood test findings that disappear within a few months. In rare cases, it can lead to a serious form of heart disease.

What are the risk factors for lupus?

Sex, race and ethnicity, and age are all key factors. Younger women, and especially younger women of color, are most at risk.

• Sex: Most people with lupus are female. The ratio of women to men who have lupus is about 9 to 1.
• Race and ethnicity: Women of color have higher incidences of lupus than do White women.
  • The incidence of lupus in Black women is three times the incidence in White women.
  • The incidence of lupus in Asian women is twice that of the incidence in White women.
  • The incidence of lupus in LatinX women is twice that of incidence in White women.
• Age: Symptoms that lead to a lupus diagnosis most commonly appear in people between 15 and 44 years of age.

What are the symptoms of lupus?

Symptoms vary from person to person, but the typical person with lupus is a young woman who develops arthritis of the fingers, wrists or other small joints, hair loss, a rash (often on the face, in butterfly pattern over the nose and cheeks). These may be accompanied by fevers, swollen lymph nodes (glands), chest pain, and/or protein in the urine.

Often, the first clinical indication that a person has lupus is a blood test result, whether or not they are experiencing common lupus symptoms. When symptoms do occur, they usually begin in only one or two areas of the body, but more may develop over time.

The most common signs and symptoms of lupus are:

Each person with lupus will likely have their own, specific patterns of symptoms and flares. These patterns may change over time, however.

A doctor may suspect a person has lupus if they display some of the classic signs, such as recurring rashes and fevers. In cases where these more obvious symptoms are absent, the diagnosis may be less overt. For example, a patient may experience only mild forms of some of the classic symptoms, such as fatigue and swollen lymph nodes, and not seek medical attention until a serious complication occurs, such as pericarditis (inflammation of the lining around the heart).

If your primary care doctor suspects you may have lupus, contact a rheumatologist to confirm the diagnosis and get appropriate treatment. HSS is top-ranked for rheumatology by U.S. News & World Report and offers a dedicated team of specialists and resources at the Lupus and APS Center of Excellence.

How is lupus diagnosed?

A diagnosis of lupus is generally based on laboratory tests that exclude other diseases that may have similar symptoms, and specific serologic tests – blood tests that determine the presence of certain antibodies.

Patients with lupus characteristically have the following lab test results:

• Antibodies against their own cells. Almost all people with lupus test positive for antinuclear antibodies (ANA).
• Positive signs of one or more of the following, which may be present along with a positive ANA:
  • anti-double-stranded DNA (anti-dsDNA)
  • anti-Smith (anti-Sm) antibody
  • anti-Ro/SSA antibody
  • anti-La/SSB antibody
  • anti-RNP antibody
  • antiphospholipid antibodies (anticardiolipin antibody, anti-Beta-2-glycoprotein-I (aβ2GPI) antibody)
• Blood (hematologic) abnormalities, such as:
  • leukopenia (low white blood cell count)
  • lymphopenia (low lymphocyte count)
  • thrombocytopenia (low platelets)
  • various forms of anemia, including autoimmune hemolytic anemia
  • evidence of inflammation in the blood, usually measured by an increased erythrocyte sedimentation rate (ESR)

People with lupus sometimes also have false-positive test results for syphilis. This has nothing to do with the disease syphilis, and a more specific test for syphilis will be negative in people who have this test result.

What is the treatment for lupus?

Depending on the symptoms, blood test results and the particular organs involved, a person with lupus may receive one or more of the following:

• nonsteroidal anti-inflammatory drugs (NSAIDs)
• hydroxychloroquine (Plaquenil)
• corticosteroids
• immunosuppressive drugs such as azathioprine (Imuran), methotrexate (Rheumatrex), cyclosporine (Sandimmune, Neoral), and mycophenolate mofetil (CellCept), cyclophosphamide (Cytoxan) and tacrolimus (Prograf)
• biological agents such as belimumab (Benlysta), rituximab (Rituxan)
• (for special circumstances) intravenous immunoglobulin, vitamins/antioxidants or drugs used for other purposes but which may be effective, such as:
  • eculizumab (to inhibit complement)
  • angiotensin converting enzyme inhibitors (to control blood pressure and protect kidneys)
  • antidepressants
  • anti-seizure medications

With proper treatment, most people with lupus can expect to have a normal lifespan. Blood count abnormalities often do not require any treatment, while rashes, fatigue, arthritis and other symptoms usually respond to one or more of the various forms of treatment described above.

Aggressive treatment is required for more dangerous health complications of lupus, such as renal (kidney) disease and neurological complications.

What are the health complications of lupus?

The severity of lupus varies from mild to life-threatening. After many years of having lupus, patients may develop:

• osteoporosis (especially in those who are treated with corticosteroids)
• hypertension (high blood pressure)
• kidney failure
• atherosclerosis (also known as atherosclerotic cardiovascular disease)
• forms of heart and lung diseases

In addition, a significant number of lupus patients also have the antiphospholipid antibody (aPL) associated with antiphospholipid syndrome.

Are people with lupus at a higher risk for blood clots?

Lupus patients who are positive for antiphospholipid antibody (aPL) can develop blood clots and heart valve disease and may require additional medications to reduce the risk of clots. These include:

• aspirin
• warfarin (Coumadin)
• heparin or low-molecular-weight heparin (Lovenox or Fragmin)

(See also antiphospholipid syndrome.)

Can I get pregnant if I have lupus?

Pregnancy is possible in most patients with lupus, but complications are frequent, especially if the disease is active. Anyone with lupus should be closely evaluated before they become pregnant, especially to see if there is lupus disease activity that can be better controlled before pregnancy. When a woman with lupus becomes pregnant or is planning to become pregnant, she should get a referral for specialized care.

Medications may need to be changed to protect the fetus. Patients with high blood pressure or kidney disease are at risk to have this worsen during pregnancy. Lupus patients who are positive for aPL are at higher risk of miscarriage, while patients with anti-Ro/SSA and anti-La/SSB antibodies are at risk for delivering a child with neonatal lupus.

Does lupus lead to kidney disease?

About one-third of patients with systemic lupus erythematosus (but not those with cutaneous, drug-induced, or neonatal lupus) develop kidney disease, known as lupus nephritis.

Many cases of lupus involving the kidney are mild, and treatments have significantly improved, but up to 10% of patients with lupus nephritis can l develop kidney failure, with a need for dialysis and, possibly, a kidney transplant. About one-third of patients who start dialysis during an acute lupus flare will improve and be able to discontinue it within the first year. Patients with severe kidney disease and lupus need close follow-up with a rheumatologist and kidney specialist, since new treatments continue to be developed.

Get more detailed information about lupus from the articles and other content below, or find a rheumatologist at HSS who treats lupus. The Lupus and APS Center of Excellence offers multidisciplinary patient care, support and education.