Summary

– Lupus: a complex disease

– Causes and symptoms of lupus

– Diagnosis of lupus

– Lupus: what treatment?

 

Lupus has long been a serious and incurable autoimmune disease. The constant progress of medicine has led it to become a chronic disease that can be partially controlled and no longer affects patients’ life expectancy.

However, lupus remains a complex and sometimes disabling disease, the causes of which are still not known, and the treatments for which have significant side effects must be considered in the context of the symptoms of the disease. Let’s take stock of what we know about lupus, specifically systemic lupus erythematosus (SLE).

Lupus: a complex disease

Definition of the disease

Lupus is a chronic autoimmune disease. This means that the immune system, which protects the body from infections and bacteria, turns against the body itself.

Thus, lupus is characterized by autoantibodies directed against different body parts and plays an important role in affecting different organs.

Lymphocytes, the main actors of immunity, are produced in excess and responsible for inflammatory reactions and damage to different tissues and organs.

Different forms of lupus

 

Here are the different forms of lupus:

– The most common form of lupus is systemic lupus erythematosus (SLE), which can affect many parts of the body, especially the joints, skin, kidneys, heart, and brain.

– It is also called “discoid lupus, ” a chronic skin disease. The lesions form characteristic, butterfly-shaped marks on the face. Most often, this lupus does not evolve into disseminated lupus.

– Induced lupus is triggered by a drug and disappears when the drug is stopped.

– Neonatal lupus concerns babies to whom the mother transmitted lupus during pregnancy or delivery.

– Pediatric lupus also exists. However, they are rare: about 5% of all lupus cases.

Causes and symptoms of lupus

Unknown causes of lupus

Although it is known that lupus tends to affect women of childbearing age, its causes remain unknown to this day and are probably multifactorial. However, several triggering factors have been established:

– a genetic predisposition;

– exposure to the sun

– stress

– pregnancy;

– Female hormones, as suggested by the distribution of the disease: 9 women for 1 man;

– Belonging to certain ethnic groups: African-American, Native North American, Asian;

– environmental factors: certain viruses (Epstein-Barr virus…), drugs, toxic substances.

Several symptoms related to lupus

A characteristic of lupus is its evolution in the form of attacks between remission phases when the treatment is well controlled at a fair and low dose.

Note: Lupus, which is not contagious, manifests itself very differently from one person to another. Not all clinical signs are always present; they appear at different times during the disease and gradually become associated with each attack.

In terms of symptoms, the following list can be made (not exhaustive):

– fever, fatigue;

– joint or muscle pain

– skin damage;

– a great sensitivity to the sun;

– an attack on different organs which are more or less damaged and injured: kidneys, lungs, digestive system, eyes (Gougerot-Soljgren syndrome or dry syndrome), arteries, brain, etc.;

– blood damage, with disturbances that can affect white blood cells, red blood cells, platelets, etc.;

– small sores in the mouth or nostrils, hair loss;

– a greater susceptibility to infections because of the damage to the immune system;

– Raynaud’s syndrome: fingers or toes turn blue easily in case of cold or stress.

Good to know: there is also an abnormality specific to lupus: the anti-phospholipid syndrome, which is characterized by the formation of clots. In addition, in women, the disease manifestations generally diminish at the time of menopause.

Diagnosis of lupus

Diagnosing lupus is particularly complex because there are no specific symptoms of lupus. The first flare-up usually occurs after a particular triggering event.

Diagnosing lupus involves 3 phases:

– confirm the diagnosis;

– localize organ damage;

– search for another autoimmune disease.

Today, there is a classification of 11 criteria to help in the diagnosis (American College of Rheumatology, 1997): 4 criteria out of 11 can guide further examinations to be carried out by making lupus suspected. However, it is rare for 4 criteria to be present immediately in the clinical picture.

The diagnosis will therefore be based on a combination of several elements: a complete clinical examination, blood tests showing the presence of antibodies, X-rays, MRI, liver, and kidney function tests, etc.

Please note: the list of tests to be performed is not exhaustive due to the many possible conditions.

Association with other autoimmune diseases

Other autoimmune diseases can be associated with lupus:

– Gougerot-Sjogren’s syndrome, which is manifested by dry eyes and mouth;

– Anti-phospholipid antibody syndrome (APAS), which is characterized by the appearance of blood clots;

– Other systemic autoimmune diseases: inflammatory myopathies, rheumatoid arthritis, scleroderma, etc.

In addition, women with systemic lupus erythematosus have twice the risk of developing cervical cancer. There is also an increased risk of cervical dysplasia (pre-cancerous lesions) and invasive cancer in women whose lupus is treated with immunosuppressants rather than anti-inflammatory drugs.

Systemic lupus erythematosus is associated with refractory (treatment-resistant) hypertension in 10% of cases, twice as many as in the rest of the population (especially in black subjects, with impaired renal function, hypercholesterolemia and/or elevated biomarkers of inflammation). This form of hypertension would increase the overall mortality of lupus patients by a factor of almost three.

Source: Gandelman JS et al.: Increased Incidence of Resistant Hypertension in Patients with Systemic Lupus Erythematosus: A Retrospective Cohort Study. Arthritis Care Res (Hoboken). 2019: advanced publication online March 15. DOI: 10.1002/acr.23880.

Lupus: differential diagnoses

Given its multitude of symptoms and “polymorphism,” lupus can be confused with many other conditions:

– inflammatory joint diseases (rheumatoid arthritis, ankylosing spondylitis, reactive arthritis…);

– diseases with long-term fever (cancers, bacterial or viral infections, etc.).

Lupus: what treatment?

Lupus is characterized by alternating flare-ups and periods of remission, which can last for several years.

Well treated, lupus does not prevent a normal active life if some precautions are taken:

– avoid sun exposure;

– respect all the instructions inherent to the treatment, including special diets;

– keep a moderate level of physical activity;

– stop smoking completely;

– take the advice of your doctor before any vaccination, operation, or pregnancy project;

– be particularly vigilant when traveling, especially to countries where malaria is endemic.

Please note: the treatment of lupus will most often be personalized according to the different symptoms and conditions.

Medications that can be used are

– corticosteroids (especially prednisone)

– immunosuppressants;

– aspirin or non-steroidal anti-inflammatory drugs

– antimalarials;

– Monoclonal antibodies.

Tip: Remember to have a serious discussion with your doctor about the benefits and risks of the side effects of treatments (at doses above 30 mg/day, prednisone causes nephritis and thrombocytopenia, among other things). It is advisable to consult a rheumatologist for the management of your lupus.

It is important to note that in lupus, the first month of treatment with corticosteroids is predictive of the next 11 months of treatment. Therefore, every effort should be made to limit the number of oral steroids taken early in the course of the disease to ensure that the need for them can be reduced later.

Regular follow-up with biological examinations to monitor the disease’s evolution and the possible involvement of new organs is also essential. Gynecological follow-up is also essential for the early detection of cervical cancer.

Today, although the precise causes are still unknown and the individual evolution of lupus cannot be predicted, the development of a curative treatment seems to be getting closer.

Good to know: biotherapy (and in particular that based on anti-TNF alpha), which targets a biological actor of immunity, could give rise to new drugs in the years to come (paradoxically, anti-TNF alpha can themselves induce the appearance of systemic lupus erythematosus or other autoimmune diseases (vasculitis, rheumatoid arthritis, hepatitis or thyroiditis, for example).

At this time, we can talk about the following:

– belimubab, which is directed against lymphocyte growth factor;

– lupuzor, a peptide therapy currently in phase 3 clinical trials, has many advantages.

Research is, therefore, very active in considering treatments adapted to each patient and offers hope for treatments and a better quality of life for people who have lupus. Nevertheless, due to their high cost, these molecules (rituximab, infliximab, adalumimab, etanercept, and abatacept) are only used when other treatments fail.