Lupus Erythematosus

Systemic lupus erythematosus is perhaps the most characteristic of autoimmune diseases. It is a rich in clinical disease in spite of this little understood. Understand what is lupus and know which organs are most affected.

What is lupus?

Lupus disease is characterized by the production of various antibodies, ie antibodies which attack the body itself. Between lupus autoantibodies are common against each cell nucleus and another against the patient’s own DNA. With that, you can imagine the havoc that can cause disease, since it creates antibodies against structures essential for life. It is not known why the body starts producing antibodies. There is probably a combination of genetic factors, because the disease is more common when there is a positive family history and environmental factors not yet identified. Lupus attacks skin, kidneys, joints, lung, heart, blood vessels, blood cells, nervous system, gastrointestinal tract, among other organs and tissues. The disease is nine times more common in women than in men and occurs in all ages, being most prominent between 20 and 40. Lupus is a disease difficult to treat and in severe cases require the use of powerful immunosuppressive drugs. While it is challenging to treat, is a disease that shows how much medicine has advanced in recent decades. In the 50’s, over 60% of patients died before completing five years of illness. Today, 80% survive for at least twenty years.

Symptoms of lupus erythematosus

We will describe the most common symptoms of lupus. That does not mean that all patients with lupus manifestations have necessarily all explained below. There are cases of lupus cases with few symptoms and many symptoms.

Joints

The joint damage occurs in up to 95% of patients with lupus. The two main manifestations are arthritis (joint inflammation) and arthralgia (joint pain without inflammatory signals). Often these symptoms appear years before the definitive diagnosis of lupus.

Arthritis and arthralgia of lupus have some characteristics that differentiate them from other diseases that affect the joints:

Are migratory, ie change joint pains within 24-48 hours. One day knees hurt the next day his fist, and the third day the shoulder, then returns to the knees, etc..

The implication is symmetrical, ie when a knee pains, the other also.

Usually presents as polyarthritis or polyarthralgia, which means that several joints hurt at the same time. The acometimiento of a single joint is an indication for other diagnoses such as gout (read: DROP | Uric Acid | Symptoms and diet) or septic arthritis.

In general, the joint hurts more than the appearance may suggest for whom the review.

The joints most commonly affected are the hands and fingers (phalanges), wrist and knees.

Skin

Another very commonly affected organ is the skin. Up to 80% of lupus patients have some type of skin problem, especially in sun-exposed areas. Typical lesions include malar rash or rash in butterfly wings. It is a reddish area that covers the cheeks and nose as can be seen in the pictures below. The malar rash (malar) appears in at least 50% of patients, usually lasting several days and there is always sun exposure. Prolonged exposure to fluorescent lights can also trigger skin lesions of lupus. Another common dermatological lesion discoid lupus, which is characterized by round, reddish plaques, most commonly on the face, neck and scalp. Discoid lupus may be part of systemic lupus box, or be the only manifestation of the disease. In the latter case, the prognosis is better, since there is no involvement of other organs.

Patients with discoid lupus have isolated 10% chance of evolving into systemic lupus. The more numerous discoid lesions, the greater the risk of progression to other organs.

Other common dermatological lesion is loss of hair, which can affect not only the scalp, but also eyebrows, eyelashes and beard.

Oral ulcers similar to canker sores are common, but with the difference being, usually painless (read: Cold Sores | Causes and treatment).

Raynaud’s phenomenon is a change in coloration of the members caused by spasm of blood vessels. The artery spasm causes a sudden lack of blood, leaving her pale hand. If the spasm persists, the lack of blood causes the hand that was pale begins to be red. This ischemia can cause great pain. When the spasm disappears, the rapid return of blood leaves the skin hot and flushed it.

Raynaud’s phenomenon is not unique to lupus and can occur even in people without any disease diagnosed. Cold, snuff and caffeine can be triggers for that symptom.

Kidneys

Up to 75% of lupus patients develop renal injury during the course of their disease.

The most common finding is the loss of protein in the urine, called proteinuria.

Other important findings are:

Hematuria (blood in urine), which can be macroscopic or microscopic.
Elevation of blood creatinine, indicating renal failure.
Hypertension.

The most common renal acometimiento glomerulonephritis (glomerular injury) caused by autoantibodies. There are basically five types of lupus glomerulonephritis, gathered for the term lupus nephritis.

They are:

Lupus nephritis class I – minimal mesangial glomerulonephritis.

Lupus nephritis class II – mesangial proliferative glomerulonephritis.

Class III lupus nephritis – focal proliferative glomerulonephritis.

Lupus nephritis class IV – diffuse proliferative glomerulonephritis.

Class V lupus nephritis – membranous glomerulonephritis.

Classes III, IV and V are the most serious, with diffuse proliferative glomerulonephritis (class IV) which has a worse prognosis. Minimal mesangial glomerulonephritis (class I) is the mildest.

Lupus nephritis is classified into classes because each of these lesions has different prognoses and treatments. Therefore, the identification of what type of lupus nephritis is the patient is of great importance.

Just with clinical data is not possible to establish what type of lupus nephritis are dealing, as proteinuria, hematuria and renal failure are common findings in the nephritis type II, III, IV and V. Therefore, all patients with lupus showing signs of kidney disease should undergo renal biopsy to identify what type of injury in the glomerulus are causing antibodies.

It is perfectly possible that a patient has more than one class of lupus nephritis at the same time.

Classes I and II do not usually require specific treatment, however classes III, IV and V, and have worse prognosis and high risk of terminal renal failure are usually treated with powerful immunosuppressive drugs. The most common are steroids (cortisone), cyclophosphamide, cyclosporine, mycophenolate mofetil and azathioprine.

Untreated patients or do not respond well to drugs, inevitably end up needing hemodialysis.

Blood

Autoantibodies can also attack the blood cells produced by the bone marrow. The most common abnormality is anemia, which occurs not only by the destruction of red blood cells but also by inhibition of the production in the bone marrow.

Another common blood disorder is the decrease in white blood cells (leukocytes), called leukopenia. The mechanism is the same as anemia, destruction and inhibition of its production. Following the same reasoning, we can find the reduced number of platelets, called thrombocytopenia.

When we drop the three bloodlines while (erythrocytes, leukocytes and platelets) give the name of pancytopenia.

Any of these alterations can be fatal, either by severe anemia, infections due to low white blood cells or spontaneous bleeding due to the drop in platelets.

The increase in lymph nodes and spleen are also a common finding in lupus and can be confused with lymphoma.

Another common problem in lupus is the emergence of thrombosis. Antiphospholipid antibody syndrome is a disease that occurs frequently in patients with lupus and is associated with the formation of multiple thrombi in both arteries and in veins, which may cause a stroke pictures (stroke), renal infarction, limb ischemia, venous thrombosis of the legs and pulmonary embolism.

Blood vessels

In addition to the thrombosis that occurs with the antiphospholipid antibody syndrome, lupus autoantibodies may attack the blood vessels directly, causing what is called vasculitis. Vasculitis can affect any vessel in the body and can damage skin, eyes, brain, kidneys …

Nervous system

Lupus can present with neurological and psychiatric syndromes.

Neurological injuries occur due to thrombosis and vasculitis, which eventually lead to a stroke.

Psychiatric disorders can also occur due to lupus. The most common psychosis, in which the patient begins to have strange thoughts and hallucinations, and dementia, with progressive loss of memory and the ability to perform simple tasks.

Other bodies

Virtually any organ can be affected by lupus antibodies. Pericardial effusion and pleural effusion (water on the pleura and pericardium) are common findings. Attacks the gastrointestinal system, lungs, heart attacks, heart valve lesions and pancreatitis may also occur.

Other findings very prevalent in lupus are tiredness (fatigue), involuntary weight loss and constant low fever.

Diagnosis of Lupus

The diagnosis of lupus is made by clinical findings and measurement of antibodies in the blood. The highest is the ANA (antinuclear antibodies), antibody to core proteins of the cell.

The ANA is almost always present in cases of lupus, but can also occur in other autoimmune diseases such as Hashimoto’s hypothyroidism, rheumatoid arthritis, scleroderma, etc..

The ANA, meanwhile, may occasionally be positive in normal people. Therefore, their presence does not necessarily confirm the disease, but their absence exclude the diagnosis of lupus in nearly 100%. The cases of discoid lupus isolated, acometimiento systemic, are the exception and can not have positive ANA antibodies.

The presence of two antibodies are highly associated with lupus: Anti-Sm and anti-dsDNA. The presence of typical symptoms, positive ANA and one of these two antibodies closed the diagnosis of lupus.

Treatment of lupus

Treatment is usually with corticosteroids, chloroquine and anti-inflammatory. In more severe cases are needed stronger drugs such as cyclophosphamide, mycophenolate mofetil, azathioprine and corticosteroids in itself very high doses.

In 2011 over a drug approved for lupus, called Belimumab (Benysta ®). This medicine is a synthetic antibody against our B lymphocytes, cells that produce defense autoantibodies of lupus. The Belimumab should be used in conjunction with existing drugs and appears to reduce disease activity and frequency of relapses. Importantly, studies with this drug is not included patients previously using cyclophosphamide or had renal or neurological injury due to lupus. This means that the drug was tested only in patients with milder cases of the disease. Another fact is that in the two studies conducted, the drug was less effective in patients of African descent, which apparently is not a good choice for this ethnic group.

In severe cases, diffuse vasculitis, pulmonary hemorrhage, nerve damage, etc.., You may need treatment with plasmapheresis to reduce the number of circulating autoantibodies.

Patients with lupus can alternate phases of crisis and remission phases. Some patients are able to go years without symptoms of the disease. Some factors contribute to the reactivation of the disease in patients in remission:

Sun exposure.
Physical or mental stress.
Pregnancy.
Infections.
Snuff.
Abandonment of treatment.