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Systemic Lupus Erythematosus

Systemic Lupus Erythematosus

During the period we are in good health, our immune system produces a protein called antibodies that protects our body from any foreign attack, like inflammatory destructions of our cells seen in cancer, germs, as well as viral and bacterial infections. Systemic Lupus erythematosus (SLE) is one of the most common types of lupus where the immune system attacks its tissues. It is an autoimmune disease because the immune system cannot tell the difference between foreign invading substances and healthy body tissues. It’s also a chronic multisystem autoimmune disease that affects people of all ages but predominantly affects women of childbearing age (15-44 years old). African Americans are at higher risk with a ratio of 9:1 compared to other general populations. Women who have lupus can get pregnant and deliver healthy babies. Female with lupus who are pregnant are high-risk during pregnancy. According to the Center for Disease Control and Preventions, female patients taking strong immunosuppressive medications must stop taking the medicines before and during pregnancy to protect the unborn child from harm and complications like miscarriages, stillbirths, premature delivery, and pre-eclampsia so that the pregnancy outcomes can be improved if conceptions are delayed for at least six months and ensure that medication is adjusted.

SLE usually affects the skin, joints, brain, kidney, and blood vessels that provide strength and flexibility to surrounding structures. Though the exact cause is not known, hormonal and immunological influences and epigenetic predisposition, are considered likely causing the disease.

  • Hormones: This regulates the body’s function and metabolisms. Estrogen is the sex hormone that plays a significant role in lupus. Both men and women produce estrogen, but its higher among females. Many women can have lupus symptoms before menstrual periods or during pregnancy when estrogen production is high. It has shown that estrogen regulates the severity of lupus. However, it does not mean that estrogen is the leading cause of lupus.
  • Gene: No gene or a particular group of a gene causes lupus. However, lupus can appear in some families; just like among one identical twin have lupus, there is an increased chance that the other twin will develop lupus. Some ethnic groups are also more predisposed than other ethnic groups, showing the ethnic group implicated to have a common gene among them.
  • Environment: This acts as a trigger setting up illness or flares in cases of lupus. Some of the environmental triggers are ultraviolet rays from the sun, light bulbs, as well as any etiological factor that can trigger stress injury, drugs, emotional trauma, and infections.

The disease‚Äôs appearance varies among patients and may range from mild localized symptoms to life-threatening systemic disease. The diagnosis is often not specific to lupus. SLE Patients mostly present with fever, heartburn, as well as poor blood circulations to hands and toes. Fatigue, which is the most negative symptom that affects SLE patients’ quality of life, a malar rash typically facial butterfly rash, also myalgia, and arthritis are also present. Lupus is a disease of flares meaning that as symptoms worsen, the patient becomes ill, and when the symptoms improve, the patients feel better.

Types of Lupus
Among the four types of lupus, Systemic Lupus Erythematosus is the most common and the most severe type of lupus. Other types of lupus include the following:

  • Cutaneous lupus: typically affects the skin, for example, rash or lesions manifestations.
  • Drug-induced Lupus: This is manifested as a result of overactions to use of certain medications like Isoniazid, Hydralazine, Procainamide, Minocycline, Quinidine, TNF alpha inhibitors like Etanercept, Infliximab, Adalimumab. The symptoms are usually seen about 3 to 6 months after starting a medication. Most commonly seen among elderly individuals.
  • Neonatal or pediatric Lupus: This happens when an infant passively acquires autoantibodies from a mother with SLE. The skin, liver, and blood pathology resolve by six months, but the most severe problem seen is congenital heart block, which may require a pacemaker.

Clinical Features
SLE is a disease that affects many parts of the organ systems. Flares can be either acute or chronic; a lot of symptoms are present. They usually come and go at different stages; the following clinical features are both seen in males and females

  • Skin: mostly seen with 70% of cases presented
    • Malar rash (butterfly rash) with a sparing seen across cheeks and nose
    • Photosensitivity via sunlight
    • Discoid Rash
    • Oral ulcers: usually painless ulcers
    • Alopecia: loss of hair
  • Joints: morning stiffness
    • Arthritis and arthralgia
    • Nonerosive polyarthritis

    Other symptoms are seen in the musculoskeletal can present with myalgia and lymphadenopathy. The brain presents with seizures, psychosis with personality changes, and finally, clinical features on the heart showing the involvement of myocardium, pericardium, coronary artery, and lungs showing interstitial lung diseases, pulmonary hypertension, etc.

Diagnosis and Lab Test
SLE is one of the illnesses that are not easy to diagnose. However, providers have particular technique skills and tests to detect signs of SLE.

SLE is an imitator because its symptoms look like ones seen in bone disorders, diabetes, blood disorders, rheumatoid arthritis, fibromyalgia, and even in cases of thyroid problems. Diagnosis is a combination of physical symptoms presented to the providers by the patients and laboratory results. Most times, in clinical settings, it’s not a onetime diagnosis. More often, it’s a diagnosis narrowed down to asking whether the patient met the criteria for a diagnosis of lupus. The American College of Rheumatology has revised these criteria.

If a provider suspects lupus’s symptoms, a series of blood tests should confirm the diagnosis. So, the most important blood screening test is antinuclear antibodies (ANA). So, when the antinuclear antibodies are negative, you do not have lupus, but you may have lupus when the antinuclear antibodies come out positive. However, to have a better specific test, the presence of anti-double-strand DNA (anti-dsDNA) and Anti-Smith (anti-smith) should be monitored. Antiphospholipid presence on the blood test will lead to increased risk factors like blood clots and miscarriages. Complement factors C3 and C4 should also be measured that way to detect the disease and its progressions. Another diagnostic method used is the radiography, which helps to assess the involvement of the joints, renal ultrasounds to elicit the size of the kidney, and any obstructions with glomerular filtration ratios, chest radiography has also been used to monitor lungs involvement.

According to the American College of Rheumatology and Systemic Lupus International Collaborating Clinics, more than four criteria out of the eleven listed (at least one clinical and one laboratory criteria or biopsy-proven Lupus nephritis with positive ANA or Anti-dsDNA) must be met for the diagnosis of SLE. The following are the list of the clinical tests: acute cutaneous lupus, chronic cutaneous lupus, oral, nasal ulcers, non-scarring alopecia, arthritis, serositis, renal, neurological, hemolytic anemia, leukopenia, and thrombocytopenia. Also, the list of Immunological Criteria includes ANA, Anti-dsDNA, Anti-Sm, Antiphospholipid antibody, Low Complement, and Direct Coombs test.

Treatment and Management
Treatment of SLE can be painful at times due to the involvement of multi-organ systems. Although there is no cure for the autoimmune disease, the medications and therapies are available to help control flares, maintain the remissions, and manage symptoms.

The management consists of supportive care, such as ensuring patients avoid prolonged sun exposure, UV light, smoking cessation, moderate alcohol consumption, monitoring blood pressure, cholesterol level, and aerobic exercise. Proper education by providers to patients can improve functions and quality of life. Long-term pharmacotherapy typically involves hydroxychloroquine, which has shown to reduce flares, articular involvement by 50%. It also reduces mortality, and for acute flares, low dose, short term oral glucocorticoids are given as induction therapy. In extreme cases, there has been an addition of immunosuppressants like Mycophenolate (Cellcept) and Azathioprine (Imuran). Other medications implicated in SLE treatment include Methotrexate, which reduces arthralgias by 79% but produces about 70% adverse effects like gastrointestinal symptoms, infections, and elevated transaminase.

According to the European League Against Rheumatism and the American College of Rheumatology, clinicians should try NSAID like Diclofenac, Ibuprofen, Naproxen for a limited period on patients with a lower risk of drug interactions. Omega 3 fatty acids are also known to reduce arthralgias related to SLE by almost 35%. The FDA in 2011 approved a drug named Belimumab (Benlysta) for the treatment of active SLE in adult patients and also used among children. They stated that Benlysta has shown to be effective in mild forms of lupus. Belimumab is the first new drug approved for lupus since 1955.

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