Systemic lupus erythematosus, SLE for short, is a chronic autoimmune disease of unknown cause grouped under lupus disorders. It is most frequently seen between the ages of 20-30 years with a high predominance of the female sex (as are most autoimmune diseases). You may even know a celebrity who has been open about her lupus diagnosis, Selena Gomez.
When we come to the cause of SLE, it's no surprise to hear that it's most commonly unknown. However, there are predisposing factors that are linked to its development. These include: being of the female sex, specific gene mutations, and environmental factors such as UV light exposure, smoking, and EBV infections.
So how does this disease affect the body? SLE is a type III hypersensitivity reaction mediated by immune complexes (a combination of free-floating antigens and antibodies). These complexes deposit at various parts of the body, leading to the subsequent clinical manifestations. The most affected areas are the skin, kidneys, joints, serosa, CNS, heart, blood vessels and the list goes on. Hence, the clinical manifestations include rash, nephritis, arthritis, serositis, coma/ seizures, carditis, clotting disorders to name a few.
We currently use the SLICC (Systemic Lupus International Collaborating Clinic) criteria to diagnose SLE. It has 17 components that are broadly categorized into clinical and immunological. It is quite detailed so we will only have a brief outline of the criteria. The clinical manifestations are of those mentioned above, as well as the infamous malar rash, also known as the butterfly rash, non-scarring alopecia, oral or nasal ulcers, and elements of anemia, leukopenia, and thrombocytopenia. The immunologic consists of detecting antibodies such as ANA (anti-nucleic acid), Anti-dsDNA, Anti-Sm (anti-smith), and antiphospholipid as well as low complements and positive direct Coombs test. Now to diagnose SLE, the patient should fulfill at least 4 of the 17 including one from the clinical as well as immunologic. Another way to confirm the diagnosis is by biopsy-proven lupus nephritis.
Treatment depends on the current presentation of the patient. However, since it is an autoimmune disorder, our mainstay of treatment is immunosuppressant drugs. If they seek medical attention with life-threatening symptoms such as late stages of lupus nephritis, neuropsychiatric disorders, or coagulation disorders, the regimen is usually high-dose corticosteroids with mycophenolate mofetil. If the above manifestations are absent, the treatment would be low-dose corticosteroids. When we come back to the rash, although the butterfly rash is more acute, the chronic ones such as the discoid rash can be treated with antimalarial drugs.
In conclusion, SLE is a disease that affects almost every part of the human body. Although it may be well-known for the infamous butterfly rash, it has much more dire manifestations especially ones to the brain and kidneys. Hence, it is wise to be aware of the possible signs and symptoms so as to detect the disease early in its course for a better future outcome for a patient.
References
Daniel, W., & Dafna, G. (2023). Clinical manifestations and diagnosis of systemic lupus erythematosus in adults. UpToDate Retrieved August 28, 2024 from https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-systemic-lupus-lupus-erythematosus-in-adults
Loscalzo, J., Fauci, A. S., Kasper, D. L., Hauser, S., Longo, D., & Jameson, J. L. (2022). Harrison’s Principles of Internal Medicine, Twenty-First Edition (Vol.1 & Vol.2). McGraw Hill Professional.
Lupus malar rash (no date) WHIMSICAL WARRIOR. Available at: https://www.sharonkeil.com/blog/lupus-malar-rash (Accessed: 28 August 2024).
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