Systemic Lupus Erythematosus

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Systemic Lupus Erythematosus (SLE) (In Phoenix, AZ, USA)

Perhaps no other rheumatic condition causes as much anxiety and perplexity among patients and medical professionals as SLE does. SLE is a disease that can have a wide range of problems, from unpleasant arthritis, rash, & fatigue to potentially fatal involvement of vital organ systems including the kidneys and brain. It is challenging to diagnose, assess, and manage.

One in every 2,000 Caucasians is affected by Systemic Lupus Erythematosus SLE, although other races and ethnic groups, such as Afro-Americans and Asians, are more likely to be affected. Women are affected 8–10 percent more frequently than men, and symptoms frequently develop when they are most likely to have children, greatly lowering their quality of life. While survival rates have steadily improved over the past several decades, 5% of patients with Systemic Lupus Erythematosus SLE die within 5 years of being diagnosed, while nearly 10% die in the first 10 years from time of diagnosis.

While the cause of SLE is unknown, it is believed that a combination of genetic factors and exposure to either infectious triggers or chemical agents is necessary to initiate the disease. While first-degree relatives of SLE patients have an approximate 5% chance of developing the same illness, an identical twin of an SLE patient has a nearly 50% chance.

We consider SLE to be an autoimmune disease. This means that the body’s immune system, which is ordinarily designed to recognize and destroy everything that is foreign to the body (such as infections) unexpectedly starts attacking numerous bodily parts and generating inflammation, but leaves the body's organs alone. Due to this, SLE symptoms appear inside the skin, joints, and maybe everywhere else.

Interesting Features:

While it is challenging to condense the wide range of SLE consequences, arthritis, rash, & general symptoms like fatigue and symptoms such as fever are the most prevalent ones. The tiny joints in the hands, feet, or, sporadically, the knees, ankle, or elbows are commonly affected by arthritis. While often troublesome, the arthritis of SLE does not typically cause joint destruction such as what we can see in rheumatoid arthritis (RA). The rash is typically triggered by exposure to sunlight and occurs most frequently in sun-exposed areas. Blistering or scarring may complicate skin lesions of SLE in severe cases. When deep layers of the skin are involved, this may be called a discoid rash.

Inflammation

from around lining of the heart and lungs (referred to as pleuritis & pericarditis, respectively), a decrease in the number of white or red haemoglobin, platelets, & mouth ulcers are further signs of SLE, an increased tendency to form blood clots, inflammation of the kidneys (nephritis), and inflammation of the brain (cerebritis) causing seizures or changes in mental functioning. Even after looking through this list of side effects, it can be claimed that SLE can affect any portion of the body. Nephritis appears to have the greatest effect on overall outcomes or survival of all SLE symptoms, especially if dialysis is necessary due to renal failure.

Atherosclerosis is a significant late consequence of SLE (hardening of the arteries). Patients with SLE, like those with RA, have a higher risk of strokes & strokes, which is thought to be connected to how inflammation affects the blood vessel lining. Women with SLE specifically have a 40–50% higher risk of developing heart disease than women of the same age with in overall population. This is why it's important to address and, if possible, reduce other heart disease risk factors like smoking, high cholesterol, high blood pressure, and diabetes.

Diagnosis:

Even in the hands of experienced physicians, the diagnosis of SLE is often very challenging. Most of the common symptoms patients with SLE exhibit may mimic a number of other illnesses. Making a diagnosis is more complex than just doing a blood test; it requires a thorough evaluation of each patient and consideration of their symptoms, physical characteristics, and laboratory anomalies. Because of this, it could take numerous visits to establish or rule out all the diagnosis, but making this time commitment is essential to prevent over diagnosing or underdiagnosing SLE.

The presence of SLE can be determined by laboratory results, however this is not enough if there are no characteristic symptoms or physical examination results.

The anti-nuclear antibody (ANA) is positive in about 99% of patients with SLE, and depending on the presence or absence of other features, a negative test can rule out SLE. A positive test, however, does not mean that one has “tested positive for lupus.” Up to 10% of the general population may express a positive ANA, and by some estimates just over 10% of individuals who show a positive ANA when a physician is suspicious enough to order the test actually have SLE.

Other antibody tests, such as anti-Smith, anti-RNP, anti-Ro/SSA, anti-La/SSB, or anti-dsDNA are more specific, and when present are much more supportive of a diagnosis of SLE. Of these antibodies, anti-dsDNA seems to be the best marker for disease activity, particularly in those patients with nephritis. Complement proteins, produced by the immune system, may be present with active inflammatory in SLE patients and can serve as reliable indicators of active disease. Those who exhibit an elevated risk for blood clots frequently have anticardiolipin antibodies or a "lupus anticoagulant" in their bodies. Regular lab tests like blood counts, plasma chemistries, including urine tests are indeed helpful in identifying SLE and screening for the disease's consequences. Patients with nephritis do not typically experience pain over the kidneys or other specific symptoms other than fever or active disease in other locations, so urine tests are particularly useful in picking up this complication at an early stage.

To assist doctors in making the diagnosis of SLE, criteria have been devised. These standards are not ideal and do not take into consideration all the characteristics that a particular patient may have, although they least provide a useful framework.

The standards comprise:

  1. A doctor's observation of mouth or nasal ulcerations
  2. Malar or "butterfly" rash covering the cheeks
  3. Rash triggered or worsened by sun exposure (photosensitive rash)
  4. Discoid rash
  5. Arthritis of 2 or more different joints
  6. Inflammation of the lining of the heart or lungs (pericarditis or pleuritis)
  7. Nephritis
  8. Cerebritis
  9. Abnormalities of blood cells (low white blood cells, red blood cells, or platelets)
  10. Abnormal Anti-Nuclear Antibody (ANA)
  11. Other abnormal immunologic tests (Anti-Smith, Anti-dsDNA, Anti-Cardiolipin, Lupus Anticoagulant, or false + syphilis test)

4 of 11 of these criteria are required for a diagnosis of SLE.

Treatment:

Therapy must be tailored to the unique characteristics of each patient because SLE can manifest itself in many different ways. The difficulty is in identifying disease complications through screening and in giving the right drugs to effectively reduce SLE activity while limiting side effects. The risks of vigorous therapy are typically justified in cases of severe illness symptoms that could be either fatal or harmful to multiple organs. Conversely, SLE that only affects the skin and joints is typically best managed with less dangerous extended maintenance medications (see Medications section).

The mainstay of treatment for mild SLE, or those people who don't have significant multiple organ involvement, is antimalarial medication like hydroxychloroquine (HCQ, brand name Plaquenil). Rashes usually start to clear up in 1-2 months, but it may take 3-6 months of treatment to fully alleviate the symptoms of arthritis. The impact of anti-malarial medication on fatigue as well as other SLE symptoms varies. According to one study, patients on HCQ who had previously been stable and stopped taking their medicine saw their disease activity worsen as opposed to patients who maintained taking it. The only severe side effect of HCQ is a one in 1,000 chance of retinal damage to the region of the eye responsible for colour vision, which is usually treated once it is discovered.

The use of sunscreen, avoiding ultraviolet (UV) light, and wearing protective clothes is advised, especially for people with SLE skin symptoms that are active. Any UV light, even many fluorescent lights, can trigger a disease flare. Therefore, avoidance of concentrated exposure to such light is advisable, and special screens or shields are available to limit UV exposure from fluorescent bulbs at one’s place of work. Sunscreens with SPF 15 or above usually provide protection from UV light as well and should be worn every day that significant light exposure is anticipated.

Non-steroidal anti-inflammatory drugs (NSAIDs) can be prescribed to treat pleuritis/pericarditis or joint problems, but it may be advisable to avoid them in people with nephritis since they have the potential to decrease blood flow to the kidneys. Additionally, NSAIDs raise the risk of ulcer development, especially in people taking corticosteroids (see below). In patients at risk for stomach complications, COX-2 selective NSAIDs may minimize this problem.

Corticosteroids Prednisone and other corticosteroids are frequently used to treat SLE symptoms and consequences. Steroids, while generally beneficial, must be taken carefully to prevent adverse effects, such as weight gain, blood sugar elevation, cataracts, increased vulnerability to infection, and bone thinning that increases the risk of fracture, among many others. It is crucial to fight the impulse to immediately treat every symptom an SLE patient exhibits with steroids. In light of this, low doses of prednisone (10 mg/day) may be used to treat joint complaints, although large concentrations of steroids (oral or intravenous) are necessary to treat major disease presentations quickly while awaiting the effects of other treatments that are usually started in this situation.

Methotrexate (MTX) When HCQ and other treatments are ineffective for SLE patients with more active arthritis, methotrexate (MTX) may be utilised. Similar to RA patients, MTX is administered once weekly alongside folic acid daily to lessen adverse effects, and usually takes effect within one to three months. Although MTX is frequently highly helpful in the treatment of inflammatory arthritis, it needs to be regularly checked for adverse effects such infections, reduced blood counts, and raised liver enzymes.

Immunosuppressive drugs Immunosuppressive medications are normally saved for severe SLE symptoms such nephritis, cerebritis, or other organ system involvement. Instances of such drugs are azathioprine (Imuran), cyclosporine, and cyclophosphamide (Cytoxan) (Neoral). These drugs function by more effectively reducing the activity of white blood cells that are responsible for injury and inflammation in SLE patients. Many medical professionals believe that Cytoxan is the best option for treating SLE that is life-threatening, especially nephritis. This drug has been demonstrated to lower mortality & kidney failure in severe lupus nephritis and is frequently administered intravenously in high dosages (referred to as "pulses") on a monthly basis.

Novel agents DHEA, mycophenolate mofetil (Cellcept), rituximab, & abatacept are recent drugs used to treat SLE. DHEA, a hormone made by the adrenal gland, may help SLE patients feel less worn out and/or operate better mentally. As a standard treatment following treatment initiation with Cytoxan or in certain individuals as a substitute to Cytoxan, Cellcept is quickly gaining acceptance for the management of nephritis. An intravenous drug called rituximab is administered in 4 once-weekly infusions. Rituximab, which is approved to treat lymphoma but is not yet FDA-approved for SLE, has showed considerable promise in recent studies for treating several SLE indications. An forthcoming injectable drug called abatacept disrupts communication between immune system cells. In the upcoming years, it is hoped that this drug as well as other "biologic response modifiers" would be helpful for treating SLE.

It is crucial that all doctors communicate with each other and that everyone is aware of decisions to change therapy because SLE patients may see many doctors. Ideally, confusion is avoided by making it clear which clinician is in charge of treating various aspects of the disease. SLE care can be adjusted for optimal benefit to patients dealing from this difficult and complicated illness by collaborating with skilled medical professionals and making choices between patient and doctor together.

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