Excerpt from Systemic Lupus Erythematosus and Pregnancy

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Background

Systemic lupus erythematosus (SLE) is one of the most common autoimmune disorders that affect women during their childbearing years. Typical clinical symptoms of SLE include fatigue, fever, arthralgias, arthritis, a photosensitive rash, serositis, Raynaud phenomenon, glomerulonephritis, vasculitis, and hematologic abnormalities. Flares of SLE are uncommon during pregnancy and are often easily treated. The most common symptoms of these flares include arthritis, rashes, and fatigue.

SLE increases the risk of spontaneous abortion, intrauterine fetal death, preeclampsia, intrauterine growth retardation, and preterm birth. Prognoses for both mother and child are best when SLE is quiescent for at least 6 months before the pregnancy and when the mother's underlying renal function is stable and normal or near normal.

The mother's health and fetal development should be monitored frequently during pregnancy. In addition, an obstetrician with experience in high-risk care should conduct the follow-up of pregnant women with SLE.

Pathophysiology

The pathophysiology of disease activity during pregnancy remains unknown.

Increased SLE disease activity is expected during pregnancy because of increased levels of estrogen, prolactin, and T–helper cell 2 cytokines. The incidence of exacerbations during pregnancy and the postpartum period, especially in women in remission at the beginning of pregnancy, has been progressively diminishing in the last 30 years. Possible causes for flare-ups during the postpartum period include decreased levels of anti-inflammatory steroid, elevated levels of prolactin (ie, proinflammatory hormone), and changes in the neuroendocrine axis.

Frequency

United States

The prevalence of SLE is 14.6-50.8 cases per 100,000 general population.

The frequency of exacerbation or persistently active disease varies with disease activity at conception. Rates range from 7-33% in women who have been in remission for at least 6 months to 61-67% in women who have active disease at the time of conception.

In the United States, the overall average incidence of SLE between 1950 and 1990 was estimated to be 1.8-7.6 cases per 100,000 person-years.

International

Incidences in 4 European cohorts from Iceland, England, and Sweden were similar to those observed in the United States. Rates in these cohorts were 3.3-4.8 cases per 100,000 person-years.

Mortality/Morbidity

Over the past 50 years, the survival rate in patients with SLE has improved dramatically. In 1955, the 5-year survival rate was only 50%, whereas, in the 1990s, the 10-year survival rate approached or exceeded 90%, and the 20-year survival rate approached 70%. Factors contributing to this improvement include early diagnosis, increased potency of pharmaceutical agents, and improved treatments (eg, dialysis, kidney transplantation).

Race

• Evidence suggests that SLE is more common in African American groups than in white populations. In general, prognoses are worse in African American or Hispanic patients with SLE than in white patients.
• In North America and Europe, prognoses are worse in patients with SLE who are of Asian, Indian, African Caribbean, or Hispanic race than in white patients.
• When the prevalence of SLE is stratified by race, the prevalence among African Caribbean individuals was approximately 5 times the rate observed in people of white descent.
• In the United States, the prevalence of SLE in female African Americans ranges from 17.9-283 cases per 100,000.
• A West Indian study of female patients with SLE reported a prevalence of 83.8 cases per 100,000.

Sex

The incidence of lupus is dramatically higher in women than in men. The race- and sex-specific incidence rates of definite SLE per 100,000 persons were 0.4 (95% CI, 0.2-0.7) in white males, 3.5 (95% CI, 2.9-4.2) in white females, 0.7 (95% CI, 0-2) in African American males, and 9.2 (95% CI, 6.8-12.5) in African American females.

Age

SLE in pregnancy affects female adolescents and women of reproductive age.

• The incidence peaks between ages 15 and 45 years, ie, the childbearing years, when the female-to-male ratio is about 12:1.
• In patients with SLE that begins during childhood or later, the female-to-male ratio is approximately 2:1.

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