Lupus, a complex autoimmune disease, presents a formidable challenge in the field of medicine. Its intricate interplay of genetic, environmental, and immunological factors make it a truly perplexing enigma.
Timely detection and effective treatment of Lupus play a pivotal role in mitigating its destructive consequences. Early diagnosis enables clinicians to intervene promptly, minimizing the progression of the disease and preventing irreversible organ damage. Therapeutic interventions, including immunosuppressive agents and targeted biologics, have revolutionized the management of Lupus, reducing disease activity, averting flares, and enhancing patients' quality of life. Furthermore, regular monitoring and follow-up assessments ensure optimal disease control and allow for adjustments in treatment regimens.
What remains of highest importance is the integration of patient education, psychological support, and multidisciplinary collaboration in all phases of the disease. All of the above can definitely ensure optimum patient care.
The first step, however? Get informed!
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The word ‘lupus’ (Latin for ‘wolf’) is attributed to the thirteenth century physician Rogerius who used it to describe erosive facial lesions that resembled a wolf's bite. The butterfly distribution of the facial rash, however, was noted by von Hebra in 1846, and the first published illustrations of Lupus erythematosus were included in his text, "Atlas of Skin Diseases."
At the time, the manifestations of the disorder were believed to be solely cutaneous, and it was not until 1872 that Kaposi proposed that there were two types of Lupus erythematosus: the discoid form and a disseminated (systemic) form.
Perhaps the most important event in the modern era of Lupus was the discovery of the LE cell by Hargraves and colleagues in 1948. The scientists theorized that the cell was “the result of phagocytosis of free nuclear material with a resulting round vacuole containing this partially digested and lysed nuclear material...”. This discovery enabled the diagnosis of individuals with a much milder form of the disease and promoted early treatment.
Two other immunologic markers of Lupus were also recognized in the 1950s: the biologic false-positive test for syphilis and the immunofluorescent test for antinuclear antibodies.
Lupus is a chronic inflammatory autoimmune disease with a wide range of clinical presentations due to its effect on multiple organ systems. There are four main types of Lupus: Neonatal and Pediatric Lupus Erythematosus (NLE), Discoid Lupus Erythematosus (DLE), Drug-induced Lupus (DIL), and Systemic Lupus Erythematosus (SLE), the latter affecting the majority of patients.
Patients with Lupus experience abnormal immunological function and the production of autoantibodies, which leads to the formation of immune complexes that target healthy tissue.
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Lupus occurs predominantly in young women with a female to male ratio of 10:1. It is less prevalent in whites than in other ethnic groups and the age at which it is typically diagnosed is 15 to 45. However, Lupus can affect any age, race and gender.
At least four susceptibility genes (first-degree relatives are about 20 times more likely to develop SLE)
Major histocompatibility complex (MHC) genes, particularly HLA genes
Ig receptor genes
Exposure to sunlight (i.e. ultraviolet light)
Exposure to chemicals such as hydrazine (found in tobacco) and aromatic amines (found in hair dyes)
Diet
Infection with certain viruses or bacteria
Androgens may inhibit while estrogens may enhance the expression of SLE
Elevated circulating prolactin
Lupus affectsEnvironmental factors serve as triggering agents for Lupus in genetically and hormonally susceptible individuals. They induce a state of immune dysregulation, and lead to hyperactive T-helper type 2 lymphocyte and Blymphocyte function.
Hyperactive B lymphocytes leads to excessive autoantibody production, and Immune regulatory mechanisms whose function is to downregulate autoantibody formation and suppress T-lymphocyte function, fail, which leads to excessive cytokine production and no suppression of autoantibody production.
The autoantibodies formed become pathogenic and form immune complexes that damage host tissue.
Multiple mechanisms to B-cell hyperactivity take place:
High antigenic load; environmental and self-antigens presented to B cells by other B cells or specific APC
Defective T-helper cells that increase B-cell antibody production and decrease defective B-cell clearance
Impairment of other immune regulatory processes including suppressor T cells function, cytokines (interleukins, interferon-γ, TNF-α, transforming growth factor-β)
Autoantibodies are directed against nuclear constituents of the cell and are thus called antinuclear antibodies. Lupus patients may have more than one antigenspecific antinuclear antibody:
dsDNA and ssDNA: Antibodies to dsDNA are highly specific for Lupus and are present in 70% to 80% of patients.
RNA-associated antigens: Four RNA-associated antigens exist; the Smith (Sm) antigen, the small nuclear ribonucleoprotein (snRNP), the Ro (SS-A) antigen and the La (SS-B) antigen.
Histone: A basic component of chromatin and nucleosomes.
Lupus anticoagulant: The phospholipid moiety of the prothrombin activator complex.
Cardiolipin
Manifestations include psychosis, depression, anxiety, seizure, stroke and neuropathy. It is unclear whether they indicate CNS involvement or are secondary to the distress of living with a chronic illness.
Manifestations include normochromic, normocytic, low iron concentration, or hemolytic anemia, leukopenia as well as thrombocytopenia.
Manifestations include an increase in serum creatinine and proteinuria.
Progression to endstage kidney disease is a major cause of morbidity and mortality, but the course of the disease is variable and many Lupus nephritis patients do very well.
Arthralgia and arthritis are frequently the chief complaint upon initial presentation.
Arthritis presents as joint stiffness, pain and inflammation, affects small and large joints, its pattern is usually recurrent and its duration is short.
Manifestations include cough, rales, tachypnea, dyspnea, pneumonitis and pleural effusion.
Pulmonary hypertension has also been reported and people with it usually have a poor prognosis.
Manifestations range from hypertension to pericarditis, myocarditis, heart murmur and coronary artery disease. The use of corticosteroids and underlying renal disease may be contributing factors.
Manifestations include dyspepsia, abdominal pain, difficulty swallowing as well as oral or nasopharyngeal ulceration.
A Malar rash, also known as butterfly rash, is seen in half of the patients.
Photosensitivity is common.
Manifestations include discoid lesions, Raynaud’s phenomenon, alopecia and vasculitis.
In addition to the above, patients with Lupus may experience nonspecific signs and symptoms such as fatigue, fever, anorexia and weight loss, which are seen frequently in patients with an active disease.
The American College of Rheumatology developed criteria for identifying Lupus patients and patients have to meet 4 or more of the 11 criteria. The criteria, however
Are 95% specific and 85% sensitive, Do not encompass all clinical manifestations, and Are used to distinguish SLE from other collagen–vascular diseases.
For a more proper diagnosis, and in addition to meeting 4 of the criteria, additional serologic, immunopathologic, and clinical evaluations are needed.
Nonsteroidal anti-inflammatory drugs (NSAIDs) can reduce pain and inflammation and help with problems of joint pain and swelling, especially in the fingers, wrists, or knees.
Over-the-counter anti-inflammatory drugs that are often used include
Ibuprofen
Naproxen
Indomethacin
Celecoxib
Aspirin can also be used and it can help prevent blood clots. Many patients with Lupus take a daily low-dose aspirin, also called baby aspirin, 75 to 100 mg, to lower their risk of blood clots.
Side effects of anti-inflammatory drugs:
Anti-inflammatory drugs can irritate the stomach, so some patients are advised to take them with food or milk.
At higher doses, NSAIDs can increase the risk of a heart attack or stroke.
Anti-malarial medications help control Lupus in several ways. They Modulate the immune system without predisposing the patient to infections, Protect against UV light and treat skin lesions that do not respond to treatment with topical therapy, and Prevent the activation of plasmacytoid dendritic cells, a component of the immune system that is responsible for making interferon.
Side effects of antimalarial drugs:
Most people do not notice any side effects, but antimalarial drugs may cause stomach pain and digestive tract problems such as nausea or diarrhea, which are self-limiting.
In rare cases, prolonged use of antimalarial drugs at high doses may damage the eyes.
Hydroxychloroquine is the most commonly used antimalarial drug for Lupus.
Dosage: 200-400mg/day orally as a single dose or in two divided doses. If the patient cannot take hydroxychloroquine, chloroquine may be recommended instead. It usually takes 1 to 3 months for the drug to start working.
Corticosteroids such as prednisone may help reduce swelling, tenderness, and pain that are triggered by Lupus attacks by "calming" the immune system, which is overactive in Lupus. They can ease symptoms in most organs including the heart, lungs, kidneys, brain or blood vessels and can ease swelling, warmth, and soreness in joints. Thus, they can prevent or decrease the risk of long-term organ damage. Corticosteroids come in different forms such as pills, a shot, or a cream to apply to the skin and Lupus symptoms usually respond very quickly to them.
Types of steroids:
Prednisone 1-2 mg/kg is the most commonly prescribed steroid in Lupus. In the case of liver problems, other steroids may be prescribed such as prednisolone or methylprednisolone.
The lowest effective dose is often used and when no longer needed, the prescribed steroid is tapered down, as corticosteroids should never be discontinued abruptly.
Side effects of steroids:
Steroids can cause a wide range of side effects, including
Increased appetite.
Weight gain.
Changes in mood.
Muscle weakness.
Blurred vision.
Increased growth of body hair.
Easy bruising.
Lower resistance to infection.
Swollen, "puffy" face.
Acne.
Osteoporosis (bone weakness).
Onset of, or worsening of, diabetes.
Onset of, or worsening of, high blood pressure.
Stomach irritation.
Nervousness, restlessness.
Difficulty sleeping.
Cataracts or glaucoma.
Water retention, swelling.
Like corticosteroids, these drugs curb the immune system, help control symptoms, and help prevent long-term organ damage. They may be prescribed if corticosteroids have not helped manage symptoms.
Common immunosuppressive drugs used in Lupus include:
Methotrexate
Azathioprine
Cyclophosphamide
In some cases, immunosuppressive drugs may be prescribed concomitantly with corticosteroids, which leads to lower amounts of each type of drug being used, hence less side effects.
Side effects of immunosuppressive drugs:
Immunosuppressants can make it hard for the body to fight infections and raise the risk of some kinds of cancer.
Azathioprine may cause pancreatitis or hepatitis.
Cyclophosphamide may lead to bladder problems, hair loss, or fertility problems.
Methotrexate can cause nausea, headaches, mouth sores, photosensitivity, lung infections and liver damage.
Anticoagulants make the blood thinner and help prevent clots, a life-threatening complication of Lupus.
Commonly used blood thinners include:
Heparin
Warfarin
Low-dose aspirin
Side effects of anticoagulants:
The most common side effects of anticoagulants are associated with an increased risk of bleeding and include: passing blood in the urine and the stools, severe bruising, prolonged nosebleeds, bleeding gums, vomiting or coughing up blood, heavy periods in women in addition to bloating, diarrhea, upset stomach and loss of appetite.
A monoclonal antibody is a synthetic protein developed to target only one type of substance in the body. Many kinds of monoclonal antibodies can be used to treat multiple diseases, but for Lupus, the below monoclonal antibodies are approved:
Belimumab (Benlysta®)
Rituxan (Rituximab®)
Anifrolumab-fnia (Saphnelo®)
Belimumab (Benlysta®), which is administered intravenously or subcutaneously, is restricted to the treatment of Lupus. It may help reduce the patient's need for corticosteroid treatment, but it has not been tested thoroughly for more severe forms of Lupus. Rituxan (Rituximab®) is another monoclonal antibody that has been used to treat Lupus when other treatments have not worked. Anifrolumabfnia (Saphnelo®) is used to treat adults with moderate to severe SLE who are receiving other Lupus medicines.
Side effects of monoclonal antibodies:
The antibodies themselves are proteins, so their administration may cause allergic-like reactions. This is more common while the drug is first being given.
Other side effects include fever, chills, weakness, headache, nausea, vomiting, diarrhea, low blood pressure and rashes.
Acthar Gel (repository corticotropin injection) is a prescription medication used for the treatment of flares as well as maintenance therapy in patients with Lupus. It works by stimulating the outer layer of cells of the adrenal gland, which promotes the production of natural cortisol that reduces inflammation.
Side effects of repository corticotropin injection include: Injection site reactions, fatigue, physical weakness, and lack of energy, fluid retention, insomnia, headache and high blood sugar.
With Lupus, the immune system attacks instead of protecting the body. Lupus does not discriminate: it attacks all organs, including the brain and nervous system. To add to the above, treatments themselves can impair mental and emotional health.
For those reasons, life with Lupus can be very challenging. With symptoms that come and go, disease flares and remissions, and the uncertainty of what each day will bring, it is normal to experience unhappiness, frustration, anger, and sadness. It is also normal to grieve for the loss of life before Lupus.
However, there is a difference between temporary negative feelings and those that become overwhelming and long-lasting, which may signal a serious illness called clinical depression.
People are considered clinically depressed when they have the below symptoms that last for more than a few weeks and are severe enough to disrupt daily life. However, if recognized and properly treated, these symptoms can improve.
These are among the most common psychological and physical symptoms of clinical depression:
Feelings of helplessness, hopelessness, uneasiness, anxiety, irritability or guilt
Sadness and crying, often without reason
Insomnia, restless sleep or sleeping too much
Changes in appetite leading to weight loss or weight gain
Lowered self-esteem or feelings of worthlessness
Inability to concentrate or difficulty thinking and indecisiveness
Lack of energy and interest in things formerly enjoyed
General slowing and clouding of mental functions
Diminished sexual interest and/or performance
Recurrent thoughts of death or suicide
Clinical depression may not be recognized in people with Lupus because its symptoms and those of active Lupus are very similar. Additionally, clinical depression may also lead to anxiety, which may in turn aggravate the physical symptoms of Lupus such as headache and stomach pain...
A variety of factors can contribute to clinical depression in people with chronic illnesses. The most common is the emotional drain that results from the need to cope with the illness in addition to the economic, social, and workplace repercussions. Also, many medications used to treat Lupus, especially corticosteroids, may precipitate clinical depression. A Lupus flare can also trigger clinical depression, both because the patient feels more ill, and because flares bring about a sense of "never being free of Lupus".
In fact,
Clinical depression generally improves with a combination of psychotherapy and medications. Patients should not feel embarrassed or hesitant about asking for a referral to a psychiatrist or psychologist as psychotherapy can help them learn to understand their feelings and illness and cope more effectively with stress. Cognitive behavioral therapy, a special type of psychotherapy, can be very helpful.
Support groups led by a therapist or trained counselor can play an essential role in helping patients deal with symptoms of clinical depression.
Certain medications such as anxiolytics and antidepressants can help patients manage their symptoms, but proper guidance from a physician is needed. If used properly, improvement is evident in a matter of weeks once the medication is started.
It is important for patients to discuss all herbs and supplements taken with their physicians as certain ingredients can interact with already prescribed Lupus medications.
Volunteerism can provide real emotional benefits, and helping others can have a positive impact on the patient's own well-being.
Chronic pain plays a major role in the development of clinical depression. Besides medications, experts recommend non-pharmacological approaches to conquer, or at least reduce, chronic pain. These include yoga, Tai Chi, Pilates, acupuncture, biofeedback, meditation, behavioral changes, play therapy as well as chiropractic care.
If physically able, the patient should be advised to take part in some sort of physical activity every day.
Not getting enough restful sleep can bring about multiple health problems, including clinical depression. Strategies that aim to improve sleep include:
Getting 7 to 8 hours of sleep in a 24-hour period.
Performing aerobic exercises every day.
Avoiding caffeine, nicotine and alcohol several hours before bedtime. Knowing which medications induce sleep disturbances and taking them earlier during the day.
Ensuring a proper sleep environment by having a good mattress, comfortable bed linens, the right room temperature and the right amount of darkness.
Staying in touch with family and friends, making phone calls, joining Facebook or adding an animal companion to the family can help alleviate symptoms of clinical depression.
Words of anger and self-pity such as "It is not fair. I have not done anything wrong. Why me?" can bring about unproductive and harmful thoughts. Patients should be encouraged to replace negative thoughts with positive ones such as “Despite my illness, I still have many blessings.”
Just as clinical depression develops over time and not overnight, conquering it is a gradual process. However, most patient with Lupus find that, with time, their overall attitude and sense of well-being are greatly improved.
Other widely famous celebrities suffer from Lupus such as Other widely famous celebrities suffer from Lupus such as Toni Braxton, Nick Cannon, Paula Abdul… Toni Braxton, Nick Cannon, Paula Abdul…
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