Systemic Lupus Erythematosus – Vasculitis/DAH
Lupus is a disease that occurs when your body's immune system attacks your own tissues and organs (autoimmune disease). Inflammation caused by lupus can affect many different body systems — including your joints, skin, kidneys, blood cells, brain, heart and lungs.
Lupus can be difficult to diagnose because its signs and symptoms often mimic those of other ailments. The most distinctive sign of lupus — a facial rash that resembles the wings of a butterfly unfolding across both cheeks — occurs in many but not all cases of lupus.
Some people are born with a tendency toward developing lupus, which may be triggered by infections, certain drugs or even sunlight. While there's no cure for lupus, treatments can help control symptoms.
Signs and Symptoms
No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe, and may be temporary or permanent. Most people with lupus have mild disease characterized by episodes — called flares — when signs and symptoms get worse for a while, then improve or even disappear completely for a time.
The signs and symptoms of lupus that you experience will depend on which body systems are affected by the disease. The most common signs and symptoms include:
❖ Fatigue
❖ Fever
❖ Joint pain, stiffness and swelling
❖ Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body
❖ Skin lesions that appear or worsen with sun exposure
❖ Fingers and toes that turn white or blue when exposed to cold or during stressful periods
❖ Shortness of breath
❖ Chest pain
❖ Dry eyes
❖ Headaches, confusion and memory loss
As an autoimmune disease, lupus occurs when your immune system attacks healthy tissue in your body. It's likely that lupus results from a combination of your genetics and your environment.
It appears that people with an inherited predisposition for lupus may develop the disease when they come into contact with something in the environment that can trigger lupus. The cause of lupus in most cases, however, is unknown. Some potential triggers include:
❖ Sunlight. Exposure to the sun may bring on lupus skin lesions or trigger an internal response in susceptible people.
❖ Infections. Having an infection can initiate lupus or cause a relapse in some people.
❖ Medications. Lupus can be triggered by certain types of blood pressure medications, anti-seizure medications and antibiotics. People who have drug-induced lupus usually get better when they stop taking the medication. Rarely, symptoms may persist even after the drug is stopped.
Risk Factors
Factors that may increase your risk of lupus include:
❖ Your sex. Lupus is more common in women.
❖ Age. Although lupus affects people of all ages, it's most often diagnosed between the ages of 15 and 45.
❖ Race. Lupus is more common in African Americans, Hispanics and Asian Americans.
Treatments
Treatment for lupus depends on your signs and symptoms. Determining whether you should be treated and what medications to use requires a careful discussion of the benefits and risks with your doctor.
As your signs and symptoms flare and subside, you and your doctor may find that you'll need to change medications or dosages. The medications most commonly used to control lupus include:
Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others), may be used to treat pain, swelling and fever associated with lupus. Stronger NSAIDs are available by prescription. Side effects of NSAIDs may include stomach bleeding, kidney problems and an increased risk of heart problems.
Antimalarial drugs. Medications commonly used to treat malaria, such as hydroxychloroquine (Plaquenil), affect the immune system and can help decrease the risk of lupus flares. Side effects can include stomach upset and, very rarely, damage to the retina of the eye. Regular eye exams are recommended when taking these medications.
Corticosteroids. Prednisone and other types of corticosteroids can counter the inflammation of lupus. High doses of steroids such as methylprednisolone (Medrol) are often used to control serious disease that involves the kidneys and brain. Side effects include weight gain, easy bruising, thinning bones, high blood pressure, diabetes and increased risk of infection. The risk of side effects increases with higher doses and longer term therapy.
Immunosuppressants. Drugs that suppress the immune system may be helpful in serious cases of lupus. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep, others), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer.
Biologics. A different type of medication, belimumab (Benlysta) administered intravenously, also reduces lupus symptoms in some people. Side effects include nausea, diarrhea and infections. Rarely, worsening of depression can occur.
Rituximab (Rituxan, Truxima) may be beneficial for some people in whom other medications haven't helped. Side effects include allergic reaction to the intravenous infusion and infections.
In clinical trials, voclosporin has been shown to be effective in treating lupus.
Other potential drugs to treat lupus are currently being studied, including abatacept (Orencia), anifrolumab and others.