Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes and other organs in the body, in various combinations, especially in young women, and which is characterised by immune abnormalities, in particular antibodies to nuclear constituents, on a background of genetic and environmental risk factors. The clinical course of SLE is variable and may be characterised by periods of remissions and of chronic or acute relapses. Treatment is based on both preventive measures and alleviation of symptoms, but in particular antiinflammatory agents including nonsteroidal antiinflammatory drugs, corticosteroids and immunosuppressive medications. Key Concepts Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown cause that can affect the skin, joints, kidneys, lungs, nervous system, serous membranes and/or other organs of the body. Immunologic abnormalities, especially the production of a number of antinuclear antibodies, are another prominent feature of the disease. The clinical course of SLE is variable and may be characterised by periods of remissions and chronic or acute relapses. Women, especially in their 20s and 30s, are affected more frequently than men. The most common pattern is a mixture of constitutional complaints with skin, musculoskeletal, mild haematologic and serologic involvement. However, some patients have predominately haematologic, renal or central nervous system manifestations. The pattern that dominates during the first few years of illness tends to prevail subsequently. Fatigue, fever and weight loss are typically present at some time during the course of the disease, occurring in 50–100% of patients. Other symptoms may include myalgia and weight gain. Fever due to active disease is seen in over 50% of patients with SLE; fever may also represent infection or a drug reaction. The course is marked by remissions and relapses and may vary from mild to severe. Major manifestations include: Arthritis, with joint symptoms in >90% of patients Mucocutaneous lesions, including skin lesions in most patients, hair loss, and oral and/or nasal ulcers, which occur in 12–45% Raynaud phenomenon, which is present in 16–40% Renal involvement, with histopathologic change in most patients but with clinically apparent disease in ∼50% Gastrointestinal tract disease, which is more often due to complications of medications than to active SLE Pulmonary diseases, including pleurisy, pleural effusion, pneumonitis, interstitial lung disease, pulmonary hypertension and alveolar haemorrhage Cardiovascular diseases, including pericarditis (which is relatively common), verrucous endocarditis and increased risk of coronary artery disease Neurologic diseases, including cognitive defects, organic brain syndromes, delirium, psychosis, seizures, headache, peripheral neuropathies and thromboembolic disease Ophthalmologic manifestations, particularly keratoconjunctivitis sicca as well as more uncommon manifestations Haematologic manifestations, including cytopenias, thrombophilia, thromboembolic disease, lymphadenopathy and splenomegaly Autoantibody production The onset of SLE is infrequently attributed to a single event, although most clinicians have seen patients in which the disease began shortly following some memorable occurrence. Precipitating factors may include exposure to ultraviolet light, infections, stress, surgery and pregnancy. Keywords: autoimmune disease; rheumatic disease; immunology; genetics; corticosteroids