Lupus, Part 1 — Introduction, incidence, and symptoms
What is systemic lupus erythematosus?
Systemic lupus erythematosus (SLE) (lupus) is an episodic multi system
disease characterized by widespread inflammation of the blood vessels
and connective tissues. Lupus is a chronic disease but the symptoms may
come and go after the initial diagnosis. Lupus can be unpredictable and
dangerous and require regular care from doctors who are skilled in treating
children. We do not know what causes lupus but sun exposure can trigger
lupus in individuals prone to getting lupus. More females than males get
lupus and female hormones which reach high levels during puberty may allow
lupus to occur more easily. Certain medications can also cause an illness
similar to lupus that usually goes away when the mediations are stopped.
Incidence and prevalence
Incidence of this disease varies by location and ethnicity. Incidence
rates among children younger than 15 years have been reported to be between
0.5 and 0.6 per 100,000 persons. Prevalence rates of 4-250 per 100,000
persons have been measured, with greater prevalence in Native American,
Asian, Latin American, and black patients. In one study of adults, the
incidence in African American females was estimated at 1 in 500. The 5-year
survival rate for children with SLE is more than 90%. Most deaths of children
with SLE are the result of infection, nephritis, renal failure, CNS disease,
or pulmonary hemorrhage. Myocardial infarction may occur in the young
adult years as a complication of long-term corticosteroid use. Prevalence
rates are higher in Native American, Asian, Latin American, and black
patients. Prevalence rates are higher in females than in males. A female-to-male
ratio of approximately 2:1 occurs before puberty, and a ratio of 4:1 occurs
after puberty. Approximately 20% of patients with SLE initially present
by the second decade of life. Disease onset has been reported as early
as the first year of life. However, SLE remains uncommon in children younger
than 8 years.
Symptoms
Presenting symptoms: The most frequent presenting symptoms of SLE are
prolonged fever and malaise with evidence of multisystem involvement.
The children often present with a history of fatigue, joint pain, rash,
and fever. However, children may present with a variety of acute symptoms,
including memory loss, psychosis, transverse myelitis, hemoptysis, edema
of the lower extremities, headache, and painful mouth sores. Many children
develop a rash on their face or chest, particularly after being in the
sun. Eleven criteria are used for the classification of lupus in adults.
The same criteria can serve as a guideline in children. Any 4 criteria
are sufficient and should be sought in the history. Of note, ANA is almost
always present. Diagnosis is not difficult in the child who presents with
many manifestations, such as malar rash, pleuritic chest pain, nephritis,
and a positive ANA. Some patients present over longer periods and require
careful consideration. Occasionally, a definite diagnosis never develops
or the patient may have an overlap syndrome with manifestations of several
rheumatic diseases. Joints may become swollen and stiff. Mouth ulcers
may come and go. Sometimes more hair falls out than the normal amount.
The hands may turn red, white and blue when exposed to cold such as gripping
a cold drink or going into air conditioning or outside in the cold. Children
may also present with internal organ involvement in the kidneys, brain,
heart, or lungs. Lupus is more serious if involvement is in the internal
organs.
Ongoing symptoms: Children with inflammation of the kidneys develop nephritis;
those with brain involvement may have seizures, serious mood changes or
hallucinations; others may have fluid around the heart or lungs.
Lupus, Part 1 — Introduction, incidence, and symptoms
For more information, please contact:
Kathy Davis, MSEd, PhD
kdavis2@kumc.edu
(913) 588-6305