Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus

José Salvador García Morillo, Spain

From: Salud (About Health)
PABLO PÉREZ MARTÍNEZ, JOSÉ MANUEL RAMOS RINCÓN, ANA MAESTRE PEIRÓ

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease, which is characterized by a dysregulation of the immune system. It results in  loss of tolerance to one’s own antigens, so that the body recognizes them wrongly as foreign, producing an attack on the different organs and tissues by antibodies (autoantibodies).

Prevalence rates generally range from 20 to 70 cases per 100,000 people, with more frequent prevalence in young women between 20 and 40 years of age.

Its origin is unknown, but it is believed that there is an interrelationship between genetic and environmental factors. Genetically, an association has been found with certain HLAs such as B8, DR3, DQW2, C4AQ0.

In addition to genetic factors, environmental factors such as infections (Epstein-Barr virus) or exposure to xenobiotics (UV light or tobacco) appear to be related to the development of the disease.

These genetic and environmental factors will culminate in the production of autoantibodies and immune complexes. The main autoantibodies present in SLE are antinuclear antibodies (ANA), a marker that identifies patients at risk of developing or having this disease, as well as others such as anti-DNA or anti-Sm antibodies, which can be diagnostic of the disease.

The clinical picture of SLE is very variable, depending on which organs are involved. It can affect several organs such as skin, kidneys, central nervous system (CNS), lungs, vascular system, and serous membranes such as pleura or pericardium, joints, etc. Lupus nephritis is a common, serious, and feared complication in women with SLE.

Its diagnosis is based on clinical and serological criteria and depends very much on clinical suspicion. The presence of typical clinical manifestations, especially cutaneous or nephritis, photosensitivity, or hair loss in a young woman of childbearing age, guide the diagnosis of SLE.

The clinical course is usually chronic, with periods of activity or flare-ups and others of remission, although complete remission of the disease is rare.

The prognosis varies not only because of the severity of the activity in the different organs, but also because of the accumulated chronic damage which may result  from the immunosuppressive treatments used, such as hydroxychloroquine, glucocorticoids, methotrexate or cyclophosphamide.

To avoid the side effects of immunosuppressive drugs, new biological treatments are being developed, targeting B lymphocytes, such as rituximab and belimumab or anifrolumab, which are the only biologicals with a specific indication for SLE.

Book cover Comunicar Salud (Health Communications)
Comunicar Salud
(Health Communications)

PABLO PÉREZ MARTÍNEZ, JOSÉ MANUEL RAMOS RINCÓN, ANA MAESTRE PEIRÓ

1ª edición: febrero 2023
© Sociedad Española de Medicina Interna
ISBN: 978-84-09-47805-7
eISBN: 978-84-09-48195-8

This is a Spanish book full of short articles on a wide range of diseases and clinical conditions written for lay people and patients by various authors.

The book contains useful information and it has been made available in English to serve European readers by the courtesy of SEMI, the Spanish Society of Internal Medicine, in cooperation with FDIME. We will publish chapters as short reports regularly over the coming months.

We hope you will enjoy reading them.

10 key points
  1. 9 out of 10 adults with lupus are women. Nephritis is one of the most serious complications. Early diagnosis with renal biopsy and rapid and intensive treatment with steroid pulses and immunosuppression are recommended.
  2. The association with other autoimmune diseases is frequent. 1 in 3 patients have or report another immune-mediated disease and nearly half had a family member with lupus.
  3. There are four clinical forms: systemic SLE (70%), lupus exclusively cutaneous (10%), drug-induced lupus and neonatal lupus, due to the transplacental passage of antibodies from the mother to the fetus.
    The evolution is due to flare-ups or relapses, which can be frequent, leading to chronic tissue damage.
  4. Clinical assessment of activity is just as important as chronic damage in systemic autoimmune diseases.
  5. Complete remission is rare, despite intensified treatment. For this reason, it is sometimes necessary to accept a situation of low disease activity.
  6. Sun protection and smoking cessation are keys to avoiding recurrences or flare-ups of the disease.
  7. Pregnancy is a risky situation for SLE. Advice should be made on the best time for pregnancy, and it is recommended that patients should be in remission for at least 6 months.
  8. Hydroxychloroquine (HCQ) is a mainstay of SLE treatment; 100% of SLE patients should be on HCQ therapy.
  9. Corticosteroids treatment should be kept to a minimum, because the cumulative dose of corticosteroids is one of the main predictors of chronic damage that affects the quality of life of people with lupus.
  10. It is believed that between 10% and 15% of people with lupus will die prematurely due to complications of lupus. A study funded by the Lupus Foundation of America found that, overall, lupus was among the top 20 causes of death in women between the ages of 5 and 64. However, due to better diagnosis and management of the disease, most people with the disease will now live a normal life.

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