World Lupus Day – Lupus: the loss of tolerance to the body itself

Systemic Lupus Erythematosus is a chronic systemic autoimmune disease, caused by a loss of tolerance to the body itself, with consequent aggression to various organs.

The cause is unknown, but several factors are known to be involved: genetic, immunological, endocrine and environmental, such as sun exposure or medication.

The disease has a wide spectrum of clinical manifestations, and may evolve chronically or by outbreaks, with periods of worsening and improvement. It predominantly affects women of childbearing age with an average age of 16 to 55 years, although it can appear at younger ages or in the elderly population and is more common in blacks, Hispanics and Asians.

The alteration of the immune system, possibly in response to an infectious or environmental stimulus in a predisposed individual, leads to a dysregulation of the immune response with the production of antibodies and the beginning of an exaggerated inflammatory response. Loss of tolerance against one’s own cells is a characteristic and can affect any organ, with the consequent different manifestations.

The most frequent complaints are constitutional symptoms (90%), such as fever, tiredness, weight loss, or are related to musculoskeletal (80-90%) and cutaneous (over 80%) involvement, with pain joints or muscles and skin or mucosal lesions, often triggered by sun exposure, the best known of which is the butterfly wing rash. Very common are also oral or nasal canker sores. Hematological involvement is also common, with the appearance of anemia, leukopenia or thrombocytopenia, often associated with antibodies against these cells, which lead to their destruction.

The kidney is affected in a high percentage of patients, with manifestations of variable severity, which can lead to significant inflammation, protein loss, arterial hypertension and severe kidney disease, which can end in chronic renal failure and hemodialysis. Inflammation of the heart, pericardium, or heart muscle or changes in heart valves can lead to major heart disease. Pulmonary involvement can manifest in the lung itself or in the leaflets that cover it – the pleura, sometimes associated with pleural effusion.

There may also be several neuropsychiatric manifestations, from headaches, epilepsy, psychosis and memory disorders, of varying severity.

The digestive tract may be involved, although less frequently, with variable manifestations.

Finally, pregnancy can be associated with a worsening of the disease, especially if it is not well controlled at the beginning, with possible complications for both the mother and the fetus.

The diagnosis can be difficult and is based on the integration of characteristic clinical manifestations, laboratory alterations and the presence of specific antibodies. The treatment involves general measures, physical and emotional support, sun protection and can be based on general immunosuppressive drugs or directed to Organs affected organs, depending on its importance and the severity of the situation. It can range from anti-inflammatory drugs, immunomodulation with hydroxychloroquine, use of corticosteroids to more potent immunosuppression, eventually with the use of biological drugs.

Currently, with earlier diagnosis, better control of the disease and the use of new, more effective drugs with fewer long-term side effects, the disease has a good prognosis. The main causes of morbidity and mortality are cardiovascular diseases, infections and kidney disease.

It is very important to choose a doctor who has experience in this area, who you trust, who can manage the disease and eventually guide you to the various specialties when necessary.

Text by Bruno Grima – Unit of Systemic Immune-mediated Diseases of the Hospital Professor Doutor Fernando Fonseca / Member of NEDAI of SPMI