Systemic Lupus Erythematosus ( LUPUS )

Systemic lupus Erythematosus (SLE), commonly also known as Lupus is a chronic autoimmune  inflammatory disease characterized by acute and chronic inflammation of various tissues of the body, People with lupus produce abnormal antibodies  (abnormal Defense System ) in their blood that target tissues within their own body rather than foreign infectious agents, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, lungs, brain, and other organs. Lupus is Latin for “wolf”: the disease was so-named in the 13th century as the rash was thought to appear like a wolf’s bite.

There are several kinds of Lupus:

•             Systemic lupus Erythematosus (SLE) is the form of the disease that most people are referring to when they say “lupus.” The word “systemic” means the disease can affect many parts of the body. The symptoms of SLE may be mild or serious.

•             Discoid lupus erythematosus is a chronic skin disorder in which a red, raised rash appears on the face, scalp, or elsewhere. The raised areas may become thick and scaly and may cause scarring. The rash may last for days or years and may recur. A small percentage of people with discoid lupus have or develop SLE later.

•             Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. The lesions do not cause scarring.

•             Drug-induced lupus is a form of lupus caused by medications. Many different drugs can cause drug-induced lupus. Symptoms are similar to those of SLE (arthritis, rash, fever, and chest pain) and they typically go away completely when the drug is stopped.

•             Neonatal lupus is a rare disease that can occur in newborn babies of women with SLE, Sjögren’s syndrome, or no disease at all.

Here I am going to discuss about SLE only

Fast Facts

             Lupus is an autoimmune disease.

             Lupus occurs ten times more often in women than in men.

             Treatment depends on the organs involved .

             Involvement of the kidneys or/and the brain is the most serious manifestation of lupus.

             People can live well with lupus if they actively work toward good health.

             Sun exposure can lead to lupus flares. Cover your skin or use a sun-blocking cream, SPF 50 or greater.

             Carefully plan your pregnancies; lupus can flare during pregnancy and can affect its outcome.

Prevalence

SLE can occur in both males and females, More than 90% of cases of SLE occur in women, frequently starting at childbearing age, and are affected about ten times more often than men (women men ratio 10:1). While it most commonly begins between the ages of 15 and 45, a wide range of ages can be affected. Rate of SLE varies between countries from 20 to 70 per 100,000

Cause

The precise reason for the abnormal autoimmunity that causes lupus is not known. Inherited genes, viruses, ultraviolet light, and certain medications may all play some role.

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 a person experience will depend on which body systems are affected by the disease. The most common signs and symptoms include:

Patients may present with any of the following manifestations

Common symptoms include:

Patients may present with any of the following manifestations.

•             Constitutional (eg, fatigue, low grade fever with no other cause, weight loss, General discomfort, uneasiness, or ill feeling (malaise).

•             Musculoskeletal (eg, pain and swelling in joints with early morning stiffness, muscular pain).

•             Mouth sores.

•             Skin (eg, Skin rash: A “butterfly” rash develops in about half the people with SLE. The rash is mostly seen over the cheeks and bridge of the nose. It can be widespread. It gets worse in sunlight, Sensitivity to sunlight, discoid lupus, Hair loss).

•             Fingers and toes that turn white or blue when exposed to cold or during stressful periods (Raynaud’s phenomenon)

•             Kidney (eg, acute or chronic renal failure, acute nephritic disease)

•             Brain and nervous system: Headaches, numbness, tingling, seizures, vision problems, and personality changes

•             Lungs (eg, Chest pain when taking a deep breath, pleural effusion, pneumonitis, pulmonary hypertension, interstitial lung disease)

•             Digestive System (eg, nausea, vomiting ,dyspepsia, abdominal pain)

•             Heart: Valve problems, inflammation of heart muscle.

•             Blood vessels: Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild and may not require treatment or may be severe and require immediate attention.

•             Blood: People with lupus may develop anemia, leucopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets in the blood, which assist in clotting). Some people with lupus may have an increased risk for blood clots.

•             Pregnancy : it can cause recurrent miscarriages.

In patients with suggestive clinical findings, a family history of autoimmune disease should raise further suspicion of SLE.

Diagnosis

Lupus can be hard to detect because it is a complex disease that has many symptoms, and they can come on slowly. As experts in diagnosing and treating autoimmune diseases such as lupus, Rheumatologists can best determine whether a patient has lupus and advise them about treatment options.

People with lupus often have symptoms that are not specific to lupus. These include fever, fatigue, weight loss, blood clots, and hair loss in spots or around the hairline. They may also have heartburn, stomach pain, and poor circulation to the fingers and toes. Pregnant women can have miscarriages.

A diagnosis of lupus is made based on symptoms, a physical examination and blood tests. Tests can help to rule out other conditions.

If your doctor suspects you have lupus based on your symptoms, a series of blood tests will be done in order to confirm the diagnosis. The most important blood screening test is Antinuclear Antibody (ANA).The ANA test is commonly used to look for autoantibodies that attack the components of cell’s  nucleus, or “ command” center ,triggering  autoimmune disorder like Lupus.  If ANA is negative, you don’t have lupus. However, if ANA is positive, you might have lupus and will need more specific tests. These blood tests include antibodies to anti-dsDNA and anti-Sm, which are specific to the diagnosis of lupus.

Treatment

At present, there is no cure for lupus. However, lupus can be effectively treated with drugs, and most people with the disease can lead active, healthy lives. Lupus is characterized by periods of illness, called flares, and periods of wellness, or remission. Understanding how to prevent flares and how to treat them when they do occur helps people with lupus maintain better health. Treatment for lupus depends on signs and symptoms.

The goals of the treatment plan are to :

•             Prevent flares

Treat flares when they occur and reduce organ damage and other problems

•             Treatments may include drugs to

Reduce swelling and pain

•             Prevent or reduce flares

•             Help the immune system

•             Reduce or prevent damage to joints

•             Balance the hormones

The medications most commonly used to control lupus include: NSAIDs, Antimalarial drugs, corticosteroids, immune suppressants and biologics .

Most people with lupus can live normal lives. Treatment of lupus has improved, and people with the disease are living longer. Here are a couple of tips that may help you when living with lupus:

             Form a support system. A good doctor-patient relationship and support from family and friends can help you cope with this chronic and often unpredictable illness.

             Get involved in your care. Learn as much as you can about lupus, your medications, and what kind of progress to expect. Take all your medications as your doctor prescribes, and visit your Rheumatologist often to prevent serious problems. This lets your doctor keep track of your disease and change your treatment as needed. If you do not live near a Rheumatologist, you may need to have your primary care doctor manage your lupus with the help of a Rheumatologist.

             Stay active. Exercise helps keep joints flexible and may prevent heart disease and strokes. This does not mean overdoing it. Switch off doing light to moderate exercise with times of rest.

             Avoid excess sun exposure. Sunlight can cause a lupus rash to flare and may even trigger a serious flare of the disease itself. When outdoors on a sunny day, wear protective clothing (long sleeves, a big-brimmed hat) and use lots of sunscreen.

             If you are a young woman with lupus and wish to have a baby, carefully plan your pregnancy. With your doctor’s guidance, time your pregnancy for when your lupus activity is low. While pregnant, avoid medications that can harm your baby.

Dr. Mukhtar Masoodi is Consultant Rheumatologist, Florence Hospital Chanpore Srinagar Kashmir.