Overview
Lupus is associated with multisystemic inflammation resulting from abnormal immunological function. Patients experience periodic flares of varying severity or instances in which no observable signs or symptoms are present. The four main types of lupus are neonatal and pediatric lupus erythematosus (NLE); discoid lupus erythematosus (DLE); drug-induced lupus (DIL); and systemic lupus erythematosus (SLE).
As a rare form of lupus observed in newborns, NLE is thought to result from maternal autoantibodies passing through the placenta. However, of those pediatric patients who have positive maternal autoantibodies, only about 1% develop NLE. Common clinical presentations involve the heart, liver, and skin. Significant morbidity and mortality, along with cardiac manifestations, have been noted; however, in most NLE patients with other organ involvement (e.g. skin, liver, and blood), signs and symptoms sometimes resolve spontaneously within to 6 months.1
DLE is manifested as a chronic scarring and atrophic photosensitive dermatosis, which may progress to SLE or may occur in patients with SLE. The cause is thought to be genetic, with the highest prevalence in women, African-Americans, and persons between 20 and 40 years of age. The diagnosis is frequently made by biopsy of a rash on the scalp, face, neck, or arms. Chemical and physical sunblocks, topical corticosteroids, or antimalarial agents are commonly used to prevent disease flares and to manage the clinical manifestations associated with DLE.2
DIL occurs after exposure to a medication, causing an autoimmune response. Various organ systems may be affected, but clinical manifestations usually subside upon discontinuation of the responsible agent
SLE is the most common type of lupus and is therefore the focus of this review. SLE is commonly referred to simply as “lupus,” but it is differentiated from other types by its multi-organ system effects. SLE is diagnosed in approximately 20 to 150 persons per 100,000 and is typically seen in females of child-bearing age; however, it may affect male or female patients at any age.4–6 SLE is more commonly observed in African-Americans, Asians, Hispanics, and Native Americans.
Arriving at the correct diagnosis of lupus is a challenge, considering the multitude of clinical presentations observed. The disease can affect the kidneys, lungs, skin, nervous system, and musculoskeletal system as well as other organs of the body. If SLE is suspected, patients’ subjective complaints, as well as laboratory abnormalities and demographic characteristics, may help to pinpoint the diagnosis.
In recent decades, mortality rates attributed to SLE have declined as a result of earlier disease detection and advances in treatment. The average 10-year survival rate now exceeds 90%; three decades ago, the 10-year average survival rate was 76%. The most common causes of death are related to early active SLE include SLE-induced and immunosuppressant-induced infectious complications. A common cause of late mortality related to SLE is an accelerated atherosclerosis that is associated with either the disease or the treatment.9