Systemic lupus erythematosus (SLE) or systemic lupus (LS)

What is it about ?

Lupus erythematosus is an inflammatory disease of the connective tissue. The term “lupus” means “wolf”, and “erythematosus” “red”. These are characteristic reddish rashes (spots) in the face, in butterfly wings, reminiscent of the design of a wolf’s mouth.

Systemic lupus erythematosus (SLE) is an autoimmune disease, i.e. that the body makes antibodies that work against its own cells, and in this case, those in connective tissue. As we have connective tissue almost everywhere in the body, it is also referred to as systemic disease, i.e. that disease can occur in “systems” (eg organs) throughout the body.

It is still unclear exactly how SLE occurs in some people and not in others. It is known that genetic and environmental factors contribute, the disease occurring more often within certain families. However, as far as is known, it is not hereditary. If a family member in the first degree has the disease, there is a good chance of getting it too.

What is its frequency?

The numbers vary. Across the planet, the disease affects 4 to 250 people in 100,000. The disease mainly affects women (90%) and manifests itself mainly between the ages of 18 and 45 years. In half of the cases, the diagnosis is made before the age of 30.

How to recognize it?

Initially, there is inflammation of the connective tissue. We have connective tissue all over our body. Symptoms of SLE vary depending on the organs affected. The course of the disease is very variable, sometimes with sudden flares, and at other times a rather slow and insidious progression. Often, general symptoms also occur: fever, headache, fatigue and weight loss.

Also, depending on the affected systems and organs, the following symptoms may occur:

• the musculoskeletal system: joint pain and swelling, muscle pain.
• the skin: reddish rashes (spots) in the face in the shape of a butterfly, disc-shaped lesions, hypersensitivity to sunlight (photosensitivity), ulcers in the mouth (canker sores).
• internal organs:
  • inflammation of the kidneys (nephritis) with protein and blood in the urine, which sometimes progresses to kidney failure with greatly reduced urine production,
  • inflammation of the pericardium (pericarditis),
  • inflammation of the pleura (pleurisy) with shortness of breath, chest pain and coughing.
• psychiatric symptoms: confusion, hallucinations, memory impairment.
• blood vessels: inflammation of the capillaries in the skin, increased risk of blood clots (thrombosis), “dead” fingers (Raynaud’s disease).
• pregnancy: miscarriage, premature birth, low birth weight, pre-eclampsia (high blood pressure in pregnant women).

It is especially abnormalities in the joints and skin that are particularly frequent, other problems occur much more rarely.

How is the diagnosis made?

The medical history and symptoms will help to point to a case of SLE. If SLE is suspected, a blood test and urinalysis will be done. If it is still presumed that it is SLE after the first examinations, you will always be referred to the specialist (rheumatologist) for further examinations and initiation of treatment. Then, the general practitioner can take control of the follow-up.

There are no tests that can diagnose LED with certainty.

What can you do ?

Treatment is a specialist’s responsibility. At your level, you should avoid exposing yourself to the sun too much, exerting too much effort, and taking medications that can trigger or worsen symptoms.

What can your doctor do?

The treatment aims to contain the symptoms but also to control your immune system well. To slow down (or “inhibit” if we want to use the technical term) inflammatory reactions, the doctor may prescribe anti-inflammatory drugs such as naproxen and ibuprofen, possibly with high-dose cortisone. The greater the organic damage, the higher the doses.

To neutralize the immune system in its action, we use so-called immunosuppressants such as azathioprine, ciclosporin, methotrexate, etc. But these drugs weaken the body’s normal defense responses. Reason for which, it is necessary to follow the treatment very carefully through blood tests. Hydroxychloroquine is an antimalarial medicine used in the treatment of rheumatic conditions. Hydroxychloroquine is sometimes used as the basic medicine for SLE.

Of course, the doctor will also treat any heart failure and kidney anomalies, thrombosis, vascular problems as well as psychological symptoms.

Source

Foreign clinical practice guide ‘Systemic lupus erythematosus (SLE)’ (2000), updated on 07/31/2017 and adapted to the Belgian context on 06/14/2019 – ebpracticenet