Systemic Lupus Erythematosus is a chronic, inflammatory autoimmune collagen disease that has protean manifestation resulting from a disturbed immune cycle that causes an exaggerated production of autoantibodies. It follows a relapsing and remitting course. The prevalence of SLE is estimated to be 1/2500 persons, which occurs 10 times more in women than in men. SLE is a result of disturbed immune regulation that causes an exaggerated production of antibodies. Although the specific cause of SLE is unknown. This disturbance is brought by the combination of genetic factors, hormonal factors (the usual onset during the childbearing years), and environmental factors for instance sunlight, thermal burns. Specifically, B cells and T cells both contribute to the immune response in SLE. B cells are instrumental in promoting the onset and flares of the disease. Some of the Medication such as isoniazid, chlorpromazine, procainamide and certain anti-seizure medications have been associated with induction of SLE.
Signs and symptoms of Systemic Lupus Erythematosus
Systemic Lupus Erythematosus can affect any organ system, and mostly involves the skin, joints, kidneys, blood cells and nervous system. Its presentation and course are highly variable where the onset is insidious or acute. It can go undiagnosed for many years. The clinical features are as per the affected body system such as:
- Musculoskeletal system: Arthralgia and arthritis (synovitis) are common features characterized by swelling of joint, tenderness and pain on movement and stiffness in the morning.
Initial or classic symptoms: Fatigue, nausea, fever, weight loss.
- Integumentary (skin) system: Different types of skin disorder are seen ( subcutaneous lupus erythematosus, a butterfly rashes across the bridge of the nose and cheeks) which occurs in the majority of patients. Lesions worsen during exacerbation (flares) which may be aggravated by sunlight or artificial ultraviolet light. Accompanied by oral ulcers might involve buccal mucosa or hard palate.
- Cardiovascular system: It includes pericarditis which is the common clinical manifestation. Women diagnosed with SLE are also a risk for early atherosclerosis. Papular erythematosus and purpuric lesions may occur on fingertips, elbows and lateral sides of hands which may later progress to necrosis.
- Respiratory (pulmonary) system: It involves pleural effusion, pulmonary oedema, pneumonia, pulmonary hypertension, lung disease and pleurisy.
- Gastrointestinal: Nausea, Dyspepsia and abdominal pain are some the gastrointestinal clinical features.
- Urinary (renal) system: Acute or chronic renal failure, nephritic
disease, glomerulonephritis, and urinary tract infection. Early detection can prevent severe renal damage. Renal involvement may lead to hypertension.
- Neuropsychiatric: A Central nervous system is widespread, encompassing the entire range of Neurologic disease. It is presented by Changes in behaviour or cognitive ability, depression or anxiety due to the disease, seizure and psychosis.
- Hematologic: It involves severe disorder such as leukaemia, leukopenia, anaemia and thrombocytopenia. The clinical features and its severity are varied upon the affected body system.
Diagnostic finding and assessment
Systemic Lupus Erythematosus is based on a complete medical history including genetic history as well, physical examination, systemic tests and blood tests. Usually, assessment reveals constitutional symptoms (fever, fatigue, weight loss, arthritis, pleurisy and pericarditis). The skin is inspected for erythematous rashes (butterfly rashes). A set of questions are asked of the patient about skin changes, early signs and symptoms, photophobia, mouth ulcers and any other problems related to different systems. Cardiovascular assessment includes auscultation for cardinal friction rub associated with myocarditis and pleural effusion. The Neurologic assessment is directed towards identifying and describing any central nervous system changes. No single lab test confirms SLE but can assist in diagnosing it. Some of the laboratory tests that may be used to diagnose are:
- Liver function test
- Haematology test
- Renal function test
- Cardiac enzyme test
- Autoantibody tests
Other diagnostic immunologic tests support but do not confirm the diagnosis whereas imaging may be used to diagnose patients with suspected SLE:
- Chest X-ray
- Joint radiography
- CT or MRI in brain
- Cardiac MRI
Treatment of Systemic Lupus Erythematosus
Treatment of SLE often depends on the individual patient’s disease severity and manifestation of a disease. SLE can be managed by medical, pharmacologic, and supportive therapy.
It includes management of an acute and chronic disease. The treatment focuses on preventing progressive loss of organ function, reducing the likelihood of acute disease, minimizing disease-related disabilities and preventing complications occurred from therapy. Although SLE can be a life-threatening advance in its treatment have improved survival and reduced morbidity. Management of the chronic condition involves periodic monitoring and recognition of clinical changes requiring an adjustment in therapy.
Medication therapy for SLE is based on the concept that local tissue inflammation is mediated by the exaggerated immune response. Corticosteroids are the single most important medication available for treatment. Non-steroidal anti-inflammatory drugs (NSAIDs) are used with corticosteroids to minimize corticosteroids requirements. Immunosuppressive agents (alkylating agents and purine analogues) are used because of their effect on immune function. Corticosteroids are used topically for cutaneous manifestation. Iv administration of corticosteroids is an alternative to traditional high dose oral use. Cutaneous, musculoskeletal and mild systemic features of SLE are managed with antimalarial drugs. Immunosuppressive agents are generally reserved for the most serious forms of SLE that have not responded to conservative therapies. B-cell depleting therapies are the latest treatment for SLE.
Supportive therapy: Be sensitive to the psychological reactions of the patient due to the changes and unpredictable course of SLE. Provide appropriate information regarding the disease, daily management tips and social support. Teach patient to avoid sunlight and UV exposure and encourage the health promotion activities. A dietary consultation may be indicated to ensure that the patient is acknowledged about the dietary recommendation, given the increased risk of cardiovascular disease. Detailed information about the medication its use, doses, potential side effects and complication if it’s discontinued. Help patient to have a positive environment. Remind them of the follow-up and assess them to carry out a diagnostic investigation.