“The lupus doesn’t recognize any boundaries”: this is the title of the slogan that accompanies the World Lupus Day that is celebrated on May 10. Lupus, in fact, is a disease widespread throughout the world and that knows no differences in terms of nationality, race, ethnicity, gender and age. In Italy there are more than 60 thousand patients with systemic lupus erythematosus, in particular young women from 15 years of age and it is precisely the female sex that is most affected by the disease.

It is an autoimmune disease that sees an uncontrolled activation of the immune system, with consequent chronic inflammation that can affect any organ or apparatus.

We know more about lupus thanks to the help of Professor Carlo Selmi, Head of Rheumatology and Clinical Immunology in Humanitas and lecturer at the University of Milan.

The characteristics of the disease

Systemic lupus erythematosus is a chronic autoimmune disease, not contagious and not infectious. Like other autoimmune diseases, it is caused by an immune system reaction that mistakenly attacks certain organs, apparatus or tissues in the body that are not recognized as its own. Unlike other autoimmune diseases, however, lupus can affect any organ, even if the kidneys, heart, lungs, skin, nervous system and joints are the most affected parts.

As with other autoimmune diseases, in genetically predisposed individuals lupus can be triggered by environmental factors such as exposure to the sun, severe emotional stress, certain medications, an infection or trauma. Hormonal changes, characteristics of periods such as puberty, pregnancy and menopause, are also important.

Lupus and its symptoms

The disease is often difficult to diagnose precisely because of the different possible manifestations and the presence of non-specific symptoms, which are common to many other diseases.

Symptoms can be many, for example: systemic symptoms such as fever and fatigue; skin and mucous symptoms such as butterfly rash on the face, erythematous lesions in areas of the body exposed to the sun, alopecia and hair loss; kidney problems (nephritic syndrome and nephrotic syndrome); joint disorders with arthralgitis and arthritis, but also leukopenia, hemolytic anemia, platastrinopenia, pericarditis and pleurisy.

The course of the disease may also vary from patient to patient, with stages of remission and exacerbation.

What does the treatment involve?

The treatment of acute phases of disease and the most important organ manifestations involves the use of intravenous or oral corticosteroid drugs, first at high doses and then at the lowest dose sufficient to control the disease.

Anti-malaria, such as hydroxychloroquine, are generally very well tolerated and represent the basic therapy of all patients with SLE as they reduce the frequency of flare (appearance of new manifestations or worsening of existing ones), and they are particularly effective in controlling skin and joint manifestations of disease and can also be used during pregnancy.

Anti-malarial drugs can be associated with immunosuppressants such as cyclophosphamide, previously a first-line drug in the treatment of glomerulonephritis lupica and now much less used; azathioprine, commonly used as a maintenance therapy after acute phase therapy and in the treatment of major manifestations of disease; mycophenolate mofetil, which can be used both as an induction therapy (i.e. the one that is introduced to rapidly control the disease at its onset) and as a maintenance therapy for lupic glomerulonephritis; cyclosporine, which is useful in the case of membranous glomerulonephritis; and methotrexate, in the case of prevalent arthritic manifestations that do not respond to anti-malarial drugs.

Numerous biological drugs for the treatment of lupus are also being studied. A commercially available monoclonal antibody (Belimumab) against the BAFF molecule, a survival factor of the B lymphocytes responsible for the production of antibodies, is effective in controlling active lupus despite standard therapy. In some cases rituximab, the biological drug that affects B lymphocytes, is also used although the data have not been conclusive so far.