What causes lupus?

When healthy, our immune system protects the body from germs and cancers. With lupus, the immune system misfires and attacks “self”, the patient’s own tissues, in a process called autoimmunity or "loss of self-tolerance".

In lupus as the attack goes on, all the branches of the immune system join the fight. This leads to significant and intense inflammation. The cause of lupus is unknown, as well as what drives its diverse presentation. We know that multiple factors are required, including: the “right” genetic makeup, environmental exposures and organ specific characteristics. People with lupus may also have an impaired process for clearing old and damaged cells from the body, which in turn provides continuous stimuli to the immune system and leads to abnormal immune response.

Most often, lupus starts in young females in their fertility age, although it can sometimes start during childhood (see section on Childhood-onset lupus below). The disease is more common in some ethnic groups, mainly blacks and Asians, and tends to be worse in these groups.

About 20% of people with lupus develop the disease as children or teens. When lupus starts in childhood, it is called childhood-onset SLE, or cSLE. It is rare to get lupus before age 5 years.

How is lupus treated?

There is no cure for lupus, as it is a chronic disease. The treatment goals are to suppress the overactive immune system and ultimately induce remission and prevent permanent organ damage. The types of medications required will depend on the symptoms.

Treating cSLE requires close monitoring by a rheumatology team experienced in caring for children with lupus. The team may include doctors (pediatric rheumatologists and other specialists), nurse practitioners, physician assistants, nurses, social workers, counselors, and physical and/or occupational therapists. They will partner with the family, schools and other community resources to provide a child or teen with the best care for preserving physical and psychological health.

Common treatment options include:
  • Antimalarial drugs: Hydroxychloroquine (Plaquenil), is recommended for every patient with lupus. Hydroxychloroquine was used in the past to prevent and treat malaria. It is also used to treat lupus-related arthritis, fatigue, rashes, and mouth sores, as well as prevent flares.
  • Corticosteroids and immune suppressants: Patients with serious or life-threatening problems such as kidney inflammation, lung or heart involvement, and central nervous system symptoms need additional, stronger treatment. This may include high-dose corticosteroids such as prednisone and other drugs that suppress the immune system. Immune suppressants include azathioprine (Imuran), mycophenolate mofetil (Cellcept), methotrexate, cyclophosphamide (Cytoxan) and rituximab (Rituxan). Other medications help control pain, including nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or naproxen.
  • All medications have possible side effects. For example, prednisone is associated with increased risk of infection, weight gain, stretch marks, high blood pressure, osteoporosis (thin bones), depression, glaucoma (high eye pressure) and cataracts. However, prednisone is one of the fastest acting and most effective drugs to treat active lupus and control inflammation to prevent damage to the body. You should discuss the benefits and risks of recommended medications with your doctor. Based on the response to treatment, your doctor may be able to adjust medications to lower side effects while still controlling the lupus. It is very important that you discuss with your medical team any concerns you have about your medicines, or any changes to your medicines you are considering, to ensure the lupus is treated adequately.
  • Biologics: In 2011, the FDA approved a biologic, belimumab (Benlysta), for the treatment of active SLE in adult patients and it was approved in 2019 for use in children. Benlysta has shown to be effective in mild forms of lupus and it is the first new drug approved for lupus since 1955.



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