Systemic Lupus Erythematosus (SLE)
Introduction and Facts
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease in which organs, tissues, and cells undergo damage mediated by tissue-binding autoantibodies and immune complexes. The clinical picture of SLE can change, both in terms of disease activity and organ involvement. The immunopathogenesis of SLE is complex and is associated with diverse clinical features. No single mechanism of action can explain all cases, and the initial events that triggered it are still unknown.
In accordance with the theory, in this case there is also multisystem involvement, namely the mucocutaneous system (malar rash), musculoskeletal (arthritis), hematology (anemia), neurology (cerebri) and kidneys (nephritis).
There is still no definite data on the prevalence of SLE in Indonesia. In the US, the most reliable figure is 0.05 – 0.1% of the population, but different figures exist in various reports. Some races, such as blacks, Native Americans, and Hispanics, are at higher risk for SLE and can develop more severe disease.
Pathophysiology
The pathogenesis of SLE consists of three phases, namely the initiation phase, the propagation phase, and the peak phase (flares). The initiation of lupus begins with events that initiate apoptotic cell death in a pro-immune context. This event is caused by various agents that are actually fairly common exposures in humans, but can initiate disease because of the susceptibility of SLE patients. The prophage phase is characterized by autoantibody activity in causing tissue injury. Autoantibodies in lupus can cause tissue injury by (1) formation and generation of immune complexes, (2) binding to extracellular molecules in target organs and activating inflammatory effector functions at these sites, and (3) directly inducing cell death by ligation of surface molecules. or penetration into living cells. The peak phase reflects the immunological memory, appearing in response to the immune system fighting with the antigen that first appeared. Apoptosis occurs not only during cell formation and homeostasis but also in various diseases, including SLE. So, various stimuli can provoke the peak of the disease.
Clinical Symptoms and Complications
The clinical picture of SLE varies widely, both in terms of organ involvement at a time and the severity of disease manifestations in these organs. In addition, the course of the disease differs between patients. Severity can vary from mild to moderate to severe or even life threatening. Because of the multisystem differences in clinical manifestations, lupus has replaced syphilis as a great imitator.
The clinical picture of SLE is complicated for two reasons. First, although SLE can cause a variety of symptoms and signs, not all symptoms and signs in patients with SLE are caused by the disease. Many diseases, especially viral infections, can mimic SLE. Second, the side effects of treatment, especially long-term use of glucocorticoids, must be distinguished from the symptoms and signs of SLE
Constitutional Manifestation. Fever is present in the majority of patients with active SLE, but infectious causes must be considered, especially in patients on immunosuppressive therapy. Weight loss may occur early in the disease, where weight gain, particularly in patients treated with glucocorticoids, may become more pronounced at a later stage. Fatigue and malaise are among the most common symptoms and are often aggravating symptoms of the disease. The exact cause of these symptoms remains unclear. Disease activity, medication side effects, neuroendocrinological disorders, and psychogenic factors are involved in the onset of constitutional symptoms.
Mucocutaneous Manifestations. Photosensitivity can be recognized by rash formation, exacerbation of a pre-existing rash, reactions to excessive sunburn (exagerrated sunburn), or symptoms such as itching or paresthesias after exposure to sunlight or artificial light sources. Photosensitivity is common and can occur in all racial and ethnic groups, although there have been no studies on its prevalence in the general population. A characteristic butterfly-shaped rash, which is a reddish rash in the malar area of the cheeks and the nasal junction that divides the nasolabial folds, is better known as the malar rash or butterfly rash. This rash can be found in 20-25% of patients.
Musculoskeletal Manifestations. SLE arthritis is usually inflammatory and presents with synovitis and pain, is nonerosive and nondeforming.
Cardiovascular Manifestations. Pericarditis is characteristic, with positional substernal pain and occasionally a rub may be present. Echocardiography may show an effusion, or in chronic cases thickening and fibrosis of the pericardium.
Lung Manifestations. Pleurisy is often found in SLE. Typical chest pain of pleuritic, rub, and effusion with radiographic evidence may be present in some patients, but in others it may be symptomatic without objective findings.
Kidney Manifestations. Lupus nephritis occurs in some patients with SLE. The spectrum of pathological involvement may vary from mesangial proliferation that is completely asymptomatic to aggressive diffuse membranoproliferative glomerulonephritis leading to renal failure.
Neurological and Psychiatric Manifestations. Central nervous system (CNS) involvement occurs in 5-15% of patients and sometimes refers to neuropsychiatric SLE or lupus cerebritis. Patients may have objective manifestations such as asepsis meningitis or meningoencephalitis, seizures, chorea, ataxia, stroke, and transverse myelitis.
Gastrointestinal Manifestations. Nonspecific gastrointestinal symptoms, including diffuse abdominal pain and nausea, are typical for SLE patients.
Hematological Manifestations. Splenomegaly and diffuse lymphadenopathy are frequent but nonspecific findings in active SLE. Anemia is a characteristic finding, may be due to hemolysis, with a positive Coombs test, low haptoglobin, and high lactate dehydrogenase levels, or with myelosuppression.
Eye Manifestations. Retinal exudates and infarctions (cytoid bodies) are relatively rare and are nonspecific findings. Conjunctivitis and episcleritis can sometimes be found in active disease. etc
Diagnosis
Systemic lupus erythematosus usually begins with nonspecific or specific signs and symptoms, but may also first manifest with bruising, splenomegaly, peripheral neuritis, myoendocarditis and endocarditis, interstitial pneumonitis, aseptic meningitis, or a positive Coombs test. The presence of anemia (71%), leukopenia (56%), thrombocytopenia (11%), proteinuria, hematuria, pyuria, azotemia, hypergammaglobulinemia, immune complexes, cryoglobulins, antiphospholipid antibodies, and Biologic False-Positive Serologic Test for Syphilis also make a person suspect. SLE.
Management and Care
There is no permanent treatment for SLE. The goals of therapy are to reduce symptoms and protect organs by reducing inflammation and/or the level of autoimmune activity in the body.
Pharmacological Therapy. Mild or remittance disease can be left without treatment. If needed, NSAIDs and anti-malarials can be used. NSAIDs help reduce inflammation and pain in muscles, joints, and other tissues. Example: NSAIDs are aspirin, ibuprofen, naproxen, and sulindac. In some circumstances it is not recommended to give COX-2 selective agents because they can increase cardiovascular risk.
Corticosteroids are better than NSAIDs in treating inflammation and restoring function when the disease is active. Corticosteroids are more useful especially when internal organs are also affected.
Hydroxychloroquine is an antimalarial drug found to be effective for SLE patients with weakness, skin and joint diseases. Side effects include diarrhea, upset stomach, and changes in eye pigment.
Immunosuppressant treatment is used in patients with severe SLE manifestations and internal organ damage. Examples are methotrexate, azathioprine, cyclophosphamide, chlorambucil and cyclosporine. All immunosuppressants cause the blood cell count to decrease and increase the risk of infection and bleeding
Non-Pharmacological Therapy. Avoiding the sun or covering it with sun-protective clothing can be effective in preventing photosensitive problems. Weight loss is also advised in obese and overweight patients to reduce some of the effects of this disease, especially when there are problems with the joints.
End-stage kidney damage from SLE requires dialysis or a kidney transplant
Spleen removal surgery is sometimes performed to increase the platelet count
Reference:
Laporan Kasus. Systemic lupus erythematous (SLE). [Internet] [Cited 28/8/2021]. Available from: http://erepo.unud.ac.id/id/eprint/14766/1/4b5af7f9d2503f55a347e689e5d7f2ab.pdf
