SLE is defined as a prototypic autoimmune disease that has a broad range of clinical manifestations. In SLE, tissue damage in multiple organs is caused by autoantibodies and immune complexes.
Because other autoimmune diseases, infectious diseases, central nervous system diseases, and fibromyalgia can also manifest with multisystem disease involvement, these other conditions need to be taken into account when considering a diagnosis of SLE.
SLE most commonly develops in adult females between the ages of 15 to 40. There are eight to 10 cases in women for every one case in men. Black and Asian women are mostly affected.
Immune system activation in SLE is characterised by exaggerated B-cell and T-cell responses and loss of immune tolerance against self-antigens. Production and defective elimination of antibodies, circulation and tissue deposition of immune complexes, and complement and cytokine activation contribute to clinical manifestations that range from fatigue and joint pain to severe, life-threatening organ damage.
- Each person with lupus has slightly different symptoms that can range from mild to severe and may come and go over time. New symptoms may continue to appear years after the initial diagnosis, and different symptoms can occur at different times.
- Some of the most common symptoms of lupus include painful or swollen joints (arthritis), with a frequency of 48% in patients followed for 10 years, unexplained fever, and extreme fatigue. Arthralgia, expressed by the patient as pain and stiffness, is more common than objective arthritis.
- A characteristic red skin rash – the so-called butterfly or malar rash – may appear across the nose and cheeks. Rashes may also occur on the face and ears, upper arms, shoulders, chest, and hands and other areas exposed to the sun. Because many people with lupus are sensitive to sunlight (called photosensitivity), skin rashes often first develop or worsen after sun exposure.
- Other symptoms include:
- Chest pain.
- Hair loss.
- Mouth ulcers.
- Pale or purple fingers and toes from cold and stress.
- Kidneys: Inflammation of the kidneys (nephritis) can impair their ability to get rid of waste products and other toxins from the body effectively. There is usually no pain associated with kidney involvement. Most often, the only indication of kidney disease is an abnormal urine or blood test; however, some patients may notice dark urine and swelling around their eyes, legs, ankles, or fingers. Because the kidneys are so important to overall health, lupus affecting the kidneys generally requires intensive drug treatment to prevent permanent damage.
- Lungs: Some people with lupus develop pleuritis, an inflammation of the lining of the chest cavity that causes chest pain, particularly with breathing. Patients with lupus also may get pneumonia.
- Central nervous system: In some patients, lupus affects the brain or central nervous system. This can cause headaches, dizziness, depression, memory disturbances, vision problems, seizures, stroke, or changes in behavior.
- Blood vessels: Blood vessels may become inflamed (vasculitis), affecting the way blood circulates through the body. The inflammation may be mild and may not require treatment or may be severe and require immediate attention. People with lupus are also at increased risk for atherosclerosis (hardening of the arteries).
- Blood: People with lupus may develop anaemia, leukopenia (a decreased number of white blood cells), or thrombocytopenia (a decrease in the number of platelets in the blood, which assist in clotting). People with lupus who have a type of autoantibody called antiphospholipid antibodies have an increased risk of blood clots.
- Heart: In some people with lupus, inflammation can occur in the heart itself (myocarditis and endocarditis) or the membrane that surrounds it (pericarditis), causing chest pain or other symptoms. Endocarditis can damage the heart valves, causing the valve surface to thicken and develop growths, which can cause heart murmurs. However, this usually doesn’t affect the valves’ function.
A clinical diagnosis of SLE is made using the medical history, physical examination, and supporting laboratory data to determine whether an individual meets four of 11 classification criteria as defined by the American College of Rheumatology (ACR).
Although the SLE classification criteria were created to facilitate enrollment of individuals with phenotypically similar disease into clinical trials, they also are used in clinical practice.
Treatment options include steroids, hydroxychloroquine, dapsone, azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, and rituximab.
Because SLE has a wide spectrum of symptoms and organ involvement, treatment strategies must be individualised for each patient, the organ system involved, and the severity of involvement.
Immunosuppressive agents should be used only after infection and malignancy have been ruled out. In patients undergoing immunosuppressive treatment, physicians must continue to be vigilant for infections, both common and opportunistic, and for malignancies. Care of patients with SLE involves ongoing assessment of the patient’s lupus activity and other comorbid conditions.
Osteoporosis prevention and treatment (especially in patients taking steroids), lipid and blood pressure control, antibiotic prophylaxis, and vigilant ongoing age-appropriate malignancy screenings are all treatment measures that are essential for the care of patients with SLE.
There are many potential causes of pain in SLE. Many people with SLE have secondary fibromyalgia, and individuals occasionally present with widespread pain as the initial manifestation of SLE.
Other organ system involvement can be painful, such as serositis (pleuritis or pericarditis). Neurologic system involvement may create painful peripheral neuropathy or headache syndromes. Raynaud’s phenomenon occurs in many people with SLE and can be painful.
Avascular necrosis (AVN) is a frequent complication both of SLE. In patients with SLE, AVN most commonly involves hips, shoulders, and knees, though other joints may be involved. The treatment of each painful condition involves treating the acute SLE process, if it is present, and then treating the pain symptomatically.
This may involve the more commonly used agents such as non-steroidal anti-inflammatory drugs (NSAIDs), steroids, anti-malarial agents, or cytotoxic agents. Treatment should include education, pharmacological intervention, physical therapy, behaviour modification, family counseling, as well as nerve blocks and procedures if appropriate.
NSAIDs are the most commonly used pain medicines in adults. NSAIDs also can decrease inflammation, such as in arthritis. According to Lahita and Shao, NSAID use in SLE dates back to the early 1950’s. Although NSAIDs are not approved by the FDA for the management of pain in SLE, a US survey has shown that 84% of rheumatologists prescribe the drug to patients. In addition, studies show that up to 80% of SLE patients are treated with NSAIDs for musculoskeletal symptoms, serositis and headache.
NSAIDs collectively inhibit the synthesis of prostaglandins, a group of active lipid compounds that have diverse effects in various organ systems. The cellular membrane contains phospholipids for the synthesis of prostaglandins and thromboxane.
Phospholipids are metabolised by phospholipase A into arachdonic acid. Arachdonic acid is then further metabolide by cyclooxygenase to form prostaglandins. NSAIDs inhibit COX and the rate-limiting step of prostaglandin synthesis.
Criscione-Scheiber L. Managing Pain in Active or Well-Controlled Systemic Lupus Erythematosus. Practical Pain Management, 2016.
Lahita RG, Shao C. Nonsteriodal anti-inflamatory drugs in Systemic Lupus Erythematosus: Systemic Lupus Erythematosus: Basic, Applied and Clinical Aspects, 2015.
Wright B and Bharadwaj S. Systemic Lupus Erythematosus. Cleveland Clinic Publication, 2010.