Systemic lupus erythematosus is a complex, autoimmune pathology. It causes skin damage, but also to the different organs. So what are the causes and symptoms of this chronic disease? And what are the effective treatments to calm flare-ups? Explanations.

Lupus is a chronic autoimmune disease: the immune system, which protects against microbes or substances foreign to the body, goes haywire and turns against the body's own cells. It produces specific antibodies, called autoantibodies. This attack by the excessively produced autoantibodies is responsible for inflammatory reactions and damage to various tissues: vessels, skin, joints, muscles, blood cells, etc.

A person suffering from systemic lupus erythematosus can apply for recognition as a long-term condition (ALD) by their GP. Examinations and care related to the disease will then be covered at 100%, based on the reimbursement rates of the Assurance Maladie.

1. The different forms of lupus

There are two main types of lupus:

Cutaneous lupus (including chronic forms such as lupus tumidus, discoid lupus, etc.) which are mainly a dermatological condition. The initial lesions of acute lupus form a rash on the face, around the nose, and on the cheekbones (creating a "vespertilo" rash, like a wolf mask). Some lesions may leave scars and when they affect the scalp, they may cause alopecia in the affected areas.
Systemic lupus erythematosus: This form affects several organs. This form of lupus is expressed differently in different patients, with varying clinical symptoms. The most common manifestations are joint and skin disorders. Often there are blood problems, and inflammation of the lungs or heart (= seritis). More rarely, neurological, cardiac, or renal problems. And almost always latent or temporary fatigue. Other autoimmune diseases are sometimes associated with lupus, such as thyroid disease or Gougerot-Sjögren's disease.

Rarely, the disease can be triggered by medication: induced lupus, which is usually reversible.

2. Are some people more affected than others by this autoimmune disease?

"Women who are not menopausal are particularly affected by this disease," explains Dr. Guison. The figures from the Health Insurance are telling, as they reveal that the disease occurs in 90% of cases in women.

In metropolitan France, it is estimated that 41 people out of 100,000 suffer from systemic lupus erythematosus. In the West Indies, the disease is more common, affecting 94 out of every 100,000 people in Guadeloupe and 127 out of every 100,000 people in Martinique. Systemic lupus also exists in children under 19 years of age and accounts for 2% of cases.

3. How does lupus occur?

The onset of the disease can be quite sudden with the characteristic facial skin involvement. But it can also occur more slowly with the appearance of signs that are dissociated from one another and end up being associated progressively, over a period of a few weeks or months, or even longer: fatigue, sometimes fever, joint pain, skin signs, chest pain, shortness of breath, etc. "But severe joint pain associated with inflammatory joints in a young person can quickly lead one to believe that he or she has lupus," stresses Dr. Guison.

4. What are the symptoms?

Joint damage: Joint pain is one of the most common manifestations of the disease. Joints may be red, stiff, and painful during attacks. When the disease has been present for a long time, significant joint laxity and deformity may occur (Jaccoud's rheumatism), but this is rare. In general, however, the joint inflammation is intermittent and the diagnosis is often made before definitive joint damage.
Kidney damage: this is a painless condition. People with lupus should therefore be monitored regularly and have blood tests to assess kidney function in order to avoid silent progression of the disease. About 50% of lupus patients have their kidneys damaged by lupus, usually in the first 2-3 years of the disease. Kidney damage is usually minimal or moderate, but if lupus is not controlled, it can lead to kidney failure and treatment such as dialysis.
Skin involvement: Lupus can cause skin signs (erythema, plaques, etc.) on the body, particularly in areas of light exposure. The vast majority of patients are hypersensitive to the sun and must protect themselves from it.
Nerve damage: neurolupus is the name given to a heterogeneous group of psychological and psychiatric clinical manifestations. This condition is rare.
Heart attacks: "These are less common attacks", says Dr. Guison, which can cause endocarditis and pericarditis.
Vascular problems: phlebitis (formation of a blood clot in a vein) or thrombosis of an artery: cerebrovascular accident (CVA), myocardial infarction, pulmonary embolism, etc.Can inaugurate the disease.
Obstetrical manifestations: Obstetrical manifestations include early and late fetal loss. In patients with antiphospholipid antibodies, the risk of recurrent miscarriage is increased. Pregnancy may be considered, especially after 6-12 months of remission, but systemic lupus erythematosus may flare up during pregnancy and in the postpartum period. Pregnancy should be planned when the disease is in remission. During pregnancy, the patient should be closely monitored for disease flare-ups and thrombotic events by a multidisciplinary team that includes an obstetrician specialising in high-risk pregnancies. In particular, women with a certain type of antibody (anti-"SSA") should have weekly fetal ultrasound monitoring between 16 and 24 weeks of amenorrhoea to look for congenital heart damage.
Lung damage: this can be an effusion (= presence of fluid) in the pleura, which is the sheet around the lung.

5. How is the diagnosis made?

The specialist will carry out a detailed examination. Some doctors use the classification criteria for systemic lupus erythematosus developed by the European League Against Rheumatism/American College of Rheumatology. Patients are diagnosed with lupus if they meet at least 4 out of 11 criteria.

Blood samples show antibodies that are more or less specific to the disease, which can help guide the diagnosis. Sometimes biopsies are taken for diagnostic purposes, but also as part of the screening for complications of lupus (kidney, skin, etc.). Systematic tests usually include the following:

Anti-nuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA)
A complete blood count (CBC)
Urine analysis
Renal and liver biochemistry (including renal and liver enzymes) Some doctors use the classification criteria for systemic lupus erythematosus developed by the European League Against Rheumatism/American College of Rheumatology. Patients are only eligible for these criteria if they have at least 4 out of 11 criteria.

6. What are the causes or contributing factors?

Lupus erythematosus is caused by a combination of genetic, hormonal, and environmental factors. Possible environmental triggers include smoking and sun exposure. Smoking causes many of the immune alterations in lupus (reduced function of some T-cells, increased inflammation-promoting cells, etc).

7. How is lupus treated?

As we have seen, it is a disease with many clinical signs, it is a polymorphic pathology, and the therapeutic arsenal is therefore varied. Medication is essential to prevent the progression of the disease and serious organ damage.

Hydroxychloroquine (an antimalarial drug) for all lupus patients
Non-steroidal anti-inflammatory drugs (NSAIDs) in addition to antimalarials for mild disease
Corticosteroids, and immunosuppressants for moderate to severe forms. The immunosuppressants will slow down the defence reactions of the immune system and thus calm the disease.

8. How does the disease evolve?

Generally, appropriate treatment reduces symptoms, inflammation, and pain, and stops the development of serious organ damage. A person with lupus can live a normal life.

The person may have periods without any symptoms (remission under treatment) lasting several years, interspersed with periods of flare-ups. This evolution varies from one individual to another, but today well-monitored lupus has an excellent prognosis. However, in some rare cases, lupus is resistant to medication and continues to cause relapses or pain.

In parallel, alternative therapies can help to relieve patients and make it easier for them to live with their disease on a daily basis. For muscle mobility and joint damage, physiotherapy sessions can be prescribed. And in the most severe cases, corticosteroid injections into the joints can be considered to relieve severe pain.

A healthy lifestyle, serious photoprotection, regular follow-up with an internist, and, above all, stopping smoking can reduce the risk and severity of flare-ups.

Thanks to Dr. Jérôme Guison, a specialist in internal medicine and clinical immunology Hospital practitioner