Lupus

 

lupus

 

In this article, we want to introduce you to one of the rheumatic diseases that can start with skin problems and rashes and lead to permanent kidney failure and sometimes pulmonary hemorrhage and even death. Lupus, also called systemic lupus erythematosus (SLA), is an uncontagious disease meaning that you will never get lupus from an affected person.

 

Is Lupus Hereditary?

Who will get lupus the most?

Mechanism of lupus pathogenesis

Are there factors that trigger the onset of lupus symptoms in a susceptible person?

What are the symptoms of lupus?

How is lupus diagnosed?

What are the types of lupus?

The main treatments of lupus 

Some types of adjuvant and alternative treatments for lupus

Necessary care in people with lupus

 

Is Lupus Hereditary?

In general, children receive a number of predisposing factors for lupus from their parents. But having these factors doesn't imply that a child will become affected in the future. For example, if one of two identical twins has lupus, the probability of the other one getting lupus will be only fifty percent. Interesting to know that there is no genetic test that can accurately determine the probability of getting Lupus throughout your life.

 

Who gets lupus the most?

Although lupus is more common in native Americans, African Americans, Spanish, and Asians, but it has also been seen in all races all over the world.  However, lupus rarely happens before the age of 5 and it is also very uncommon before puberty. The most common age group for the onset of lupus is fifteen to forty-five years old. Before puberty, four out of five patients who are diagnosed with lupus are male, but this ratio is reversed to one male out of every ten patients after puberty. 

 

Mechanism of lupus pathogenesis

As mentioned above, the lupus is a disease that affects several organs and systems of the body. It is usually considered as one of multi-organ destructive disease beside other rheumatoid diseases like rheumatoid arthritis. Lupus is an autoimmune disease, meaning that it affects the immune system which is responsible for fighting foreign and pathogenic aliens such a way that it will start fighting body's internal proteins and cells. During this process, many inflammatory substances are produced by the cells of the immune system, which lead to many inflammatory reactions in various arteries and organs of the body such as skin, joints, lungs and kidneys.

 

Are there any factors that trigger the onset of lupus symptoms in a susceptible person?

The following factors may trigger the onset of symptoms of lupus in people who are prone to get the disease:

  • Hormonal changes in puberty
  • direct sunlight
  • Some viral infections
  • Emotional and psychological stress
  • Medicines:
    • Isoniazid (anti-TB drug)
    • Hydralazine (antihypertensive drug)
    • Procaine amide (cardiac antiarrhythmic drug)
    • Some anticonvulsant drugs

 

What are the symptoms of lupus?

Before introducing the diagnostic criteria for lupus, let's first talk a little bit about the signs and symptoms of this disease. The onset and the course of lupus's disease will vary from patient to patient. In other words, you will not find two lupus patients whose symptoms and their durations are exactly the same. Sometimes the symptoms are more persistent in one person than in the other. Also, the organ involved in lupus and the severity of this involvement is different among lupus patients. Some people experience only a series of mild and limited symptoms, while others have a more severe experience having most parts of their body involved.

Common signs and symptoms of systemic lupus erythematosus include:

  • Fever
  • Joint involvement appeared in the form of:
  • A red, butterfly-shaped lesion that forms on the cheeks and the bridge of the nose in the presence of sunlight.
  • Skin lesions that are caused or exacerbated by sunlight (in other words, skin lesions happening in the presence of sunlight)
  • Fatigue
  • Headache
  • Impaired memory and consciousness
  • Finger discoloration in cold or stressful conditions (Raynaud's phenomenon of white or blue fingers)

People who develop symptoms of lupus with medication (drug-induced Lupus) usually have their symptoms go away when they stop taking the medication and these symptoms will rarely reappear after stopping the medication.

 

How is lupus diagnosed?

Many signs and symptoms of lupus overlap with a large number of diseases, so the diagnosis of lupus may happen long after its onset. Sometimes it takes the patient years to develop all the criteria and symptoms needed for diagnosing lupus. In the following, we will describe the criteria for erythematous systemic lupus. These criteria can appear in the patient at the same time or over time. When a patient has the following criteria, we can say that he or she has lupus.

The first criteria: butterfly or malar rash

Redness of the skin on the cheeks and bridge of the nose, which resembles the open wings of a butterfly can be a sign of lupus. It is important to note that the nasolabial folds, which extend from the corner of the nose to the side of the lips, are not involved in this criteria. Being exposed to sunlight or ultraviolet sources is essential for this rash to develop. Interestingly, the word lupus is a Latin word that means "wolf" since the face of the lupus patient may looks like a wolf due to these malar rash.

The second criteria: discoid rashes

This skin rash on the face, neck and arms is also caused by sunlight or ultraviolet radiation. These chronic rashes are prominent plaques that can get injured. Sometimes these are the only sign of lupus. People with discoid lupus develop a discoid rash without affecting other parts of the body (less than five percent of people with lupus discoid develop systemic lupus). Studies have shown that lupus is usually milder when accompanied by these discoid lesions and there is no kidney involvement or renal failure in these subtype of lupus (the reason of why it is considered as a benign form of lupus).

The third criteria: photosensitivity

Sensitivity of the skin to sunlight and ultraviolet light causing skin rashes that usually last for two days. 

The fourth criteria: oral ulcers and nasal ulcers

They are usually painless and should be seen and examined by your doctor.

The fifth criteria: joint involvement (arthritis)

It occurs as swelling, pain, and stiffness that affect two or more joints in the body.

The sixth criteria: called serositis (inflammation of the serous membranes of the body)

Inflammation of the pericardium (the pericardium is the lining that surrounds the heart from the outside) or the inflammation of the pleura (pleura is a membrane that surrounds the lungs.)

The seventh criteria: disorders in urine analysis

  • Increased protein excretion by the kidneys
  • Existence of RBC casts (RBC masses)
  • Existence of renal cysts

The eighth criteria: abnormalities in blood cell count

  • Decrease in the number of RBCs (anemia)
  • Decrease in the number of white WBCs
  • Decrease in platelet count

The ninth criteria: the increase in the amount of antibodies against normal cells

The tenth criteria: positive antinuclear-antibody (ANA) which is an anti body working against the nucleus of our cells.

 

What are the types of lupus?

The main types of lupus are:

Erythematosus systemic lupus:

The most common form of this disease is systemic lupus, which, as its name implies, affects several systems or organs of the body. The criteria for diagnosing this disease are described above.

Cutaneous lupus:

Symptoms in these patients are limited to the skin, such as butterfly rash, discoid skin lesions (which are sometimes chronic and wounded) and photosensitivity.

Medicated lupus or drug-induced lupus:

Symptoms of lupus in these patients appear after taking stimulant drugs such as hydralazine procaine amide and antibiotics such as isoniazid and anticonvulsant drugs. With the discontinuation of the drug, the symptoms subside and it is rare for patient to have symptoms after stopping the drug.

This type of lupus is more common in men, and the important point is that anyone who is taking these drugs will not necessarily get this disease. People with the disease usually experience flu-like symptoms and can meet the criteria for systemic lupus, including Joint involvement, changes in laboratory tests, and pericarditis and pleuritis.

The patient is usually a man who has been taking medication for months or years, and it should be noted that by stopping the medication for six months, all the symptoms of this lupus-like syndrome will disappear.

Neonatal lupus:

Fetuses of mothers with lupus who test positive for antibodies (anti SSA or anti SSB), can be affected by these antibodies as well. The babies of these mothers have the following problems at birth:

  • Skin rash
  • Decrease in the number of blood cells
  • Liver disorders
  • Heart block (they may have some kind of heart problems which is called heart block)

The first three complications usually go away without permanent problems within six months. Among all symptoms, the most important and most serious complication is the heart block. Babies of mothers with lupus that have these antibodies are at high risk for heart block, usually starting around 18 to 24 weeks of gestation. Heart block is characterized by the fetus' heartbeat being too slow. This complication is permanent and newborns will need to have a pacemaker implanted. Today, doctors identify high-risk mothers and begin the treatment for the fetus even before it is born. Most babies born from mothers with lupus are healthy, and neonatal lupus is very rare.

 

What laboratory tests help diagnose lupus?

Blood cell count test:

Anemia, decreased white blood cell count, and decreased platelet count are common in lupus.

ESR or erythrocyte sedimentation rate:

In this test, the rate of sedimentation (settling down) of blood cells is measured. Normally, they should settle completely in one hour in a specific and standard tube. If they precipitate faster than this time, it is a sign of a systemic disease. It should be remembered that rising ESR is not a specific disease indicator and may occur in many inflammatory and infectious diseases and even cancers.

Liver function tests:

like AST or ALT enzymes

Kidney function tests:

such as creatinine and 24-hour urine protein

Urine analysis:

  • Study of urinary sediment
  • Study to find the increase in RBCs
  • Viewing kidney cysts

Blood tests to evaluate antibody titers:

Anti-nuclear antibody (ANA)

 

The following may also be used to diagnose lupus:

  • Simple chest x-ray (x-ray)
  • Echocardiography
  • Biopsy of suspected skin rash
  • Kidney biopsy (to determine the extent of kidney involvement due to lupus and to determine the appropriate treatment according to the severity of this involvement)

 

How is lupus treated?

The type of treatment and medication used in lupus depends on the signs and symptoms as well as the degree of involvement of your organs and systems. Usually, people experience periods of dormancy and asymptomatic between periods of recurrence of their symptoms. These periods in which symptoms are active or symptoms become worse are called flare-up of the disease. The types of drugs used during the time symptoms are active may differ from the time when patient doesn't have any obvious symptoms. You may see the following types of drugs used for systemic lupus treatment:

NSAIDs:

These drugs which are abbreviation of Non Steroidal Anti-Inflammatory Drugs, especially the over-the-counter ones, are commonly used to treat mild forms of lupus. However, you should know that sometimes these drugs are very effective in one patient and not very satisfactory in another one, and sometimes a single patient may receive different responses to this drug at different flare-ups and treatment periods. Over-the-counter NSAIDs such as naproxen and ibuprofen, besides a variety of stronger NSAIDs that are only available with a prescription, are in this class of drugs.

Antimalarial drugs:

By acting on the immune system, hydroxychloroquine (plaquenil) reduces the risk of flare-ups of lupus. Regular ophthalmologic examination is essential when taking this drug.

Immune system inhibitors:

There are drugs that weaken the activity of the immune system and thus reduce the symptoms of lupus. Some of these drugs are:

  • Azathioprine (Imuran)
  • Mycophenolate mofetil (cellcept)
  • Methotrexate (Trexall)

By weakening the body's immune system, these drugs increase the risk of various infections.  liver disorders and decreased fertility are among their side effects.

Biological drugs:

Mononuclear antibodies (drugs that deactivate the destructive elements of immune system responsible for the creation of lupus) that are a type of immunotherapy such as:

  • Belimumab (Benlysta): which is injected intravenously to reduce the symptoms of lupus in some patients. Diarrhea, nausea and infections are some of the side effects of this drug.
  • Rituximab (Rituxan): This monoclonal antibody has been very useful in resistant forms of systemic lupus erythematosus (SLE). Intravenous injection of this drug can provoke allergic reactions.

 

Some types of adjuvant and alternative treatments for lupus include:

Fish oil

Omega-3 seems to be helpful as an anti-inflammatory agent in the adjuvant treatment of lupus. This treatment never replaces the other key drugs and further studies are needed to confirm its effectiveness.

Dehydroepiandrosterone:

Medications containing this hormone are useful in reducing fatigue and muscle weakness. This medicine may cause acne in women. Do not use this medicine arbitrarily.

Acupuncture:

Acupuncture may be helpful in reducing muscle weakness but it has not been fully approved yet.

Avoid using the treatments listed here without consulting with your physician.

 

Necessary care in people with lupus

  • Regular sun protection:

Sunlight or any light source containing ultraviolet rays can cause lupus flare-ups. Therefore, wear covered clothes, hats with a brim and appropriate sunglasses and sunscreen, and it is better not to go out during the day when there is direct and intense sunlight. Your sunscreen should have an SPF of at least fifty-five.

  • Keep in touch with your doctor:

Regular checkups with your doctor are very helpful in preventing lupus flare-ups.

  • Exercise regularly

  • Avoid emotional stress
  • Avoid smoking
  • Eat a healthy diet
  • Take oral supplements (such as calcium and vitamin D) regularly if prescribed:

Studies have suggested that patients with lupus benefit from daily calcium and vitamin D intake to maintain their skeletal health.

 

 

 

 

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2020/11/24
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