Lupus

lupus summary

Lupus is a complex disease that can take many forms. It belongs to the so-called autoimmune diseases and affects women much more than men. The patient usually has characteristic skin lesions, fatigue, joint pain, swelling, among other symptoms.
It is a disease that can go years without manifesting itself, but suddenly due to a “triggering factor” that is not yet known and a supposed genetic predisposition, the disease sets in, then a series of symptoms appear that resemble those of several other diseases. , hence the difficulty in diagnosing this disease, which, until recently, only relied on the doctor linking isolated symptoms and requesting more specific examinations of the disease.

Currently, there is no known cure for lupus, despite several clinical studies that have been and are being carried out worldwide.

Treatment depends on the type of symptom presented, but in general it consists of the use of NSAIDs (non-steroidal anti-inflammatory drugs such as paracetamol, aspirin and ibuprofen), corticosteroids and use of sunscreen.

New treatments, based on antibodies, appeared on the American market in 2011 and in Brazil in July 2013, such as belimumab (Benlysta).

Women should use some type of contraceptive method until the possibility of pregnancy is discussed. In this case, it is important to consult the doctor and follow certain guidelines.

The most serious complications of the disease include organ failure.

After a certain time, it is possible to establish medication and body control, which causes the disease to go “asleep”. It is not a cure, but there are a series of known precautions and tips (see lupus tips) such as proper nutrition that can help the patient to live with the disease, to avoid crises and peaks of the disease’s symptoms.

Definition

There are actually three types of lupus: lupus erythematosus, disseminated lupus, and tuberculous lupus.
Here we will mention only lupus erythematosus and disseminated lupus erythematosus which are related diseases. Lupus erythematosus is the cutaneous manifestation of disseminated lupus erythematosus, which is an autoimmune inflammatory disease that affects several organs. Tuberculous lupus is the cutaneous localization of the lung tubercles.

Autoimmune disease is when our body’s defense cells, due to some defect, begin to recognize parts of our body as foreign bodies (antigens), causing our body to have a whole response (immune response, usually with antibodies) against his own cells, which normally would not occur.

In the United States, specialists sometimes refer to lupus as a “ great imitator ”, because the symptoms are often confused with other diseases, mainly due to a large number of different symptoms (see also below under Symptoms ). .

The term lupus comes from the Middle Ages, from the Latin loup (wolf), when a doctor named Rogerius, described as wolf bites what he would later discover to be the lesions caused by lupus.

Epidemiology

– Women are more affected by lupus than men. In a statement published in October 2017, the American College of Rheumatology (ACR ) estimated that women were 9 to 10 times more affected by lupus than men. The ACR also noted that women of color were 2 to 3 times more affected than Caucasian women.

– People aged between 15 and 40 are part of a particularly affected age group.

– Lupus can reduce life expectancy by about 25 years, as noted in a study published on October 11, 2017 in the scientific journal Rheumatology (DOI: 10.1093/rheumatologia /kex286).

– Systemic lupus erythematosus (SLE) is the most common form of lupus, according to the ACR.

– In Brazil, 150,000 to 300,000 adult patients (mostly women, in 90% of cases) have systemic lupus erythematosus (also called SLE)1.

Cause

Lupus erythematosus is a cutaneous manifestation of disseminated lupus erythematosus.

Lupus erythematosus is an autoimmune inflammatory disease of generally unknown cause.

The exact cause of lupus is still unknown, but what is known is that the disease tends to run in certain families and tends to affect certain groups more than others, suggesting a possibility of genetic inheritance. It is possible that some medications cause the disease (in 10% of cases).

A variant of the PRDM1 gene appears to be responsible for lupus, according to several studies, including one published in July 2017 in the scientific journal Nature Immunology . PRDM1 leads to the synthesis of a protein called Blimp-1 that plays a role in regulating the immune system, including T-Cells .

Sun exposure can make the condition worse. The use of sunscreen is mandatory.

It is possible that some medications cause the disease (in 10% of cases). These drugs include antiepileptics, hypotensives or antibiotics.
Studies indicate that vitamin D deficiency may be related to the onset of the disease, as well as other autoimmune diseases.
Furthermore, according to a study published in April 2009, women who take the contraceptive pill (especially the stronger ones) are 50% more likely to acquire lupus.

At the cellular level, lupus is caused by the production of antibodies by B lymphocytes, which attack the body itself. This results in different symptoms.

– Infections can trigger lupus.

– Stress and posttraumatic stress disorder (in English:  posttraumatic stress disorder ) can be causes or triggers of lupus. A study published on September 20, 2017 in the scientific journal Arthritis & Rheumatology (DOI: 10.1002 / art. 40222) showed that post-traumatic stress syndrome, but also trauma exposures were strongly associated with an increased risk of suffering from lupus. In this study of 54,763 women, researchers found that women with probable PTSD were about 3 times more likely to develop lupus.

Groups of risk

The disease can affect both sexes, however, a higher incidence was observed in young women between 20 and 30 years old.

In the US, disseminated lupus affects people of African descent even more.

Symptoms

The symptoms of lupus erythematosus differ greatly (in terms of intensity as well) from one person to another, depending on the affected organs. However, we can observe the following symptoms:

– Joint affections manifested by pain (in 90% of patients)

– Miscellaneous skin manifestations: erythema of the face in the shape of a butterfly’s wing, which appears as red plaques with crusts. This erythema usually lasts only a few days, but tends to come back frequently.

– Variation of pigmentation (increase or decrease)

– Sensitivity to ultraviolet light (60 to 100% of cases): after exposure to sunlight or fluorescent light, it usually causes the appearance of cutaneous manifestations.

– hair loss

– Fatigue

– dry eyes

– Chest pains

– Pain in the mouth and sometimes in the nose

There are, however, generalist signs that appear when outbreaks of the disease occur. These signs show an alteration in the general state, they are: fever , headaches, cold and pale or bluish hands and feet (Raynauld phenomenon), swelling, lack of appetite and weight loss. Other symptoms may appear when the disease spreads.

It is important to know that the disease is often characterized by active phases and calmer phases (we speak of remission).

Diagnosis

Diagnosis of the disease usually takes some time, as the disease can often be confused with a number of other diseases.

As the disease usually manifests itself through characteristic skin eruptions, the doctor can diagnose the disease through a first dermatological examination.

Then, he will continue his research through serological tests (identification of antibodies) and biopsies (collections) in Organs affected organs (usually skin or kidneys).

Currently, the American College of Rheumatology has developed a table of criteria (ARA criteria for diagnosis of SLE) to assist in the diagnostic process of systemic lupus erythematosus.

A new laboratory procedure has proven to be very effective, the “lupus test band”, which detects the deposit of immunoglobulins (antibodies) and complement components (immune response) in an epithelial tissue of a patient with LE. This test should not be evaluated alone, morphological findings, biopsy, serology and immunopathology should be considered.

Complications

The disease progresses slowly over several years and is remembered due to its crises, successive outbreaks, with periods of remission that can vary from a few months to years.

Complications of the disease occur due to damage to internal organs such as the kidneys, pancreas or intestines, nervous system and cardiovascular system.

– Damage to the renal system: destruction of the renal glomeruli, altering their elimination function, also called lupus nephritis. This disease can lead to kidney transplantation. It is estimated that lupus nephritis may affect up to 50% of patients with SLE2 . Dialysis is required in approximately 5% to 20% of patients with lupus nephritis, according to a Brazilian study published in July 2021 in the scientific journalSage(DOI:10.1177/09612033211030008).
Effect of physical exercise
According to a study published in 2017, regular practice of physical exercise can reduce kidney complications thanks to an anti-inflammatory effect. In a study performed on mice, a team from Ohio State University (Ohio State University) found that engaging in moderate physical exercise, such as 45 minutes of walking on a treadmill a day, helps to significantly reduce inflammation in the kidneys. While 88% of mice that did not exercise had severe kidney damage, that number was only 45% in mice that exercised. On the other hand, when the scientists exposed the mice to stress, the number of kidney problems increased. This study was published on April 26, 2017 in the scientific journal Frontiers in Physiology (DOI: 10.3389 / fphys.2017.00236).

– Pancreatic, abdominal or intestinal damage: severe abdominal pain, nausea and vomiting .

– Nervous system damage: generates seizures, migraines, paralysis and behavioral disorders.

– Damage to the cardiovascular system, which can trigger endocarditis, hypertension

– Depression .

– Type 2 diabetes .

– Thrombosis or pulmonary embolism.

Pregnancy: Women with lupus are more likely to have complications during pregnancy and have a premature delivery. If you want to get pregnant, it is advisable to have medical follow-up and that the disease is in remission, without showing symptoms for at least 6 months.

Treatments

The specialty of the doctor who will treat the patient with lupus depends on the type and symptoms that the patient presents, usually being a rheumatologist, but the possibility of care by a nephrologist, dermatologist, cardiologist, neurologist, or a perinatologist is not excluded. .

In benign cases (skin or joint damage), the patient will administer non-steroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation and pain, and antimalarials. This treatment can be accompanied by corticoids to be administered for a short duration.

Medicines
You should know that in 2021, there are no medicines to cure the disease. But it is possible to act on the symptoms of the disease.

However, in more severe cases, the patient will be treated with stronger doses of corticoids and sometimes with immunosuppressants.

Then it will be necessary to complete the treatment according to the affected organ. In case of kidney damage, hemodialysis will be necessary (blood purification through machines, as the kidneys are very affected and cannot work as required).

Antimalarial drugs, such as hydroxychloroquine (Plaquenil®), are often used to relieve some symptoms of lupus.

Since July 2013, an antibody-based drug has been available in Brazil, belimumab (sold under the reference name of Benlysta, marketed by GSK). It is a protein that inhibits a cytokine called BAFF, which plays a role in B lymphocytes.
This is one of the rare drugs specifically indicated for systemic lupus erythematosus. This drug is sold in some countries at a high price (about US$ 30,000 for a year’s treatment), which can naturally limit its use, especially if it is not covered by health insurance. In Brazil, according to Folha de S. Paulo on July 22, 2013, the annual cost of treatment for a person weighing 60 kg is 57 thousand reais.
This treatment is administered intravenously (according to information from 2013). Belimumab is particularly indicated in severe cases of lupus. Read the leaflet and consult your doctor or pharmacist.

Possible hope with CAR-T therapy
Some patients have been successfully treated with CAR-T therapy, a commonly used methodology against cancer, according to a German study published in September 2022 in Nature (DOI: 10.1038/s41591-022 -02017-5 ). CAR T-Cell ( Chimeric Antigen Receptor T-Cell ) or CAR T-Cell therapy is a method of genetic engineering that also belongs to immunotherapy. These are the basis of T lymphocytes (T cells or T-cell) into which a chimeric antigen receptor has been introduced. But it is important to know that CAR-T therapy is an expensive therapy, in 2022 in the United States it cost about 400,000 dollars per patient3 . Indeed, its manufacture is labor-intensive and must be customized for each patient.

Tips

– A patient with lupus must obligatorily protect himself from the sun with an appropriate sunscreen.

– In addition, in case of outbreaks of the disease it is important to avoid the sun.

 Certain medications are photosensitizing and can worsen the existing problem in the patient suffering from lupus, therefore, patients should be informed about this eventuality when self-medicating.

– Aesthetic treatments, such as the application of botulinum toxin (botox) for the correction of expression wrinkles, fillers for the correction of facial furrows, wrinkles and facial resizing, lasers for depilation and rejuvenation, are not recommended for people with lupus.

– It is important to maintain a diet low in fat, moderate in proteins (meat, chicken and fish), always prefer lean meats, avoiding spicy foods, rich in fruits, vegetables and grains. 

– It is important to always stay active, as too much rest can weaken the muscles, which can make the condition worse later on. A study in rats also showed that regular moderate exercise, such as walking for 45 minutes a day, could reduce inflammation and significantly reduce kidney damage (read more in Complications). Scientists also point out that Tai chi seems to be an effective way to fight lupus, as it helps to deal with stress during physical exercise.

– Whenever you are going to undergo any medical treatment, make it clear that you are a patient with lupus, as some medications can worsen your condition.

– Vaccines to prevent pneumonia and flu are recommended for people with lupus, although they are less effective than in a person without lupus; vaccines that contain live viruses such as rubella, polio, chickenpox and chicken pox are not recommended for people with lupus.

– Women with lupus should undergo a contraceptive method due to the factors mentioned in the lupus complications section. The choice of contraceptive pills should be discussed with the physician in charge, as the feasibility of choosing the type of pill varies according to the patient’s clinical condition.

– It is important to rest well, especially to get enough sleep every day, because lupus often causes fatigue .

– Eat more omega-3s and less omega-6s . Eating omega-3s in your diet can help with some of the symptoms of lupus. In fact, a high intake of omega-3 fatty acids is associated with better sleep quality and decreased depressive symptoms in lupus patients, according to a study presented at the American College of Rheumatology (ACR) annual conference, held earlier this month. November 2017 in San Diego, California. The study also took into account the amount of omega-6 consumed by the participants.
Omega-3 fatty acids have an effect on inflammation in the body, with omega-3s generally acting as anti-inflammatory and omega-6s as pro-inflammatory, i.e. promoting inflammation. In general, Western diets are very high in omega-6 fatty acids and are suspected of contributing to chronic disease. Omega-3s are found in fatty fish that live in cold waters, such as salmon, sardines and trout, as well as nuts.

Fontes e referências:
Mayo Clinic, Nature Immunology, Frontiers in Physiology (DOI : 10.3389/fphys.2017.00236), Arthritis & Rheumatology (DOI : 10.1002/art.40222), Rheumatology (DOI : 10.1093/rheumatology/kex286), American College of Rheumatology (ACR).

Jeanne Kenney
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I’m a stylist trainer, a content creator, and an entrepreneur passion. Virgo sign and Pisces ascendant, I move easily between my dreams, the crazy world I want, and my feet on the ground to carry out my projects.

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