Systemic lupus erythematosus, also called SLE or lupus, is a chronic inflammatory disease that can affect the skin, joints, kidneys, lungs, nervous system, and/or other organs of the body. The most common symptoms include skin rashes and arthritis, often accompanied by fatigue and fever. Lupus occurs mostly in women, typically developing in individuals in their twenties and thirties – prime child-bearing age. Sjögren’s syndrome is an inflammatory disease that can affect many different parts of the body, but most often affects the tear and saliva glands. Patients with this condition may notice irritation, a gritty feeling, or painful burning in the eyes. Dry mouth or difficulty eating dry foods, and swelling of the glands around the face and neck are also common. Some patients experience dryness of other mucous membranes (such as the nasal passages, throat, and vagina) and skin.

These results led researchers to conclude that, in these high-risk pregnancies, hydroxychloroquine may decrease the risk for delivering a second child with cardiac neonatal lupus.

Anti-SSA/Ro antibodies are present in about one-third of women with systemic lupus erythematosus, almost all with Sjogren’s syndrome, and even in some healthy women. Pregnancy in mothers with anti-SSA/Ro antibodies—whether they have a disease or not—can be challenging for patients and the doctors who treat them since, despite weekly monitoring, some children born to these women can have the heart complications of neonatal lupus, which are associated with additional diseases and even death. In addition, some children are born with the transient skin rash of cutaneous neonatal lupus.

Previous research suggested that fetal exposure to hydroxychloroquine may decrease the risk of heart problems associated with neonatal lupus in mothers with lupus and anti-SSA/Ro antibodies. Recently, researchers have taken this one step further and have studied the use of hydroxychloroquine as a way to lower the chances of these cardiac complications in subsequent pregnancies of women who have already given birth to a child with neonatal lupus.

Women who have one child with cardiac neonatal lupus are 10 times more likely to experience the same complications with additional children than women who have not had an affected child.

According to data from the Research Registry for Neonatal Lupus (a U.S. registry dedicated to collecting and analyzing neonatal lupus data) the recurrence rate of cardiac neonatal lupus after giving birth to one child is 18 percent and the occurrence of cardiac neonatal lupus after giving birth to one child with cutaneous neonatal lupus is 13 percent. Based on this information, researchers tried to find out whether this recurrence rate was lower in women taking hydroxychloroquine.

Twenty-four pregnancies of 22 women who had previously given birth to one child with lupus (either with or without cardiac complications) were followed. Sixty-eight percent of the mothers were Caucasian, 41 percent were diagnosed with lupus, and 32 percent were diagnosed with Sjogren’s Syndrome. Nineteen of the 22 mothers had previously given birth to a child with cardiac neonatal lupus, and eight of those infants died of complications.

Researchers studied the effects of taking hydroxychloroquine daily (with an average dose of 352.6mg) started by six weeks of, and continued throughout the entire, pregnancy to determine if this would positively affect the outcomes of the pregnancies. In addition to taking hydroxychloroquine, 25 percent of the mothers received low dose intravenous immune globulin, 75 percent received non-fluorinated steroids (17 of these women were on prednisone), and 8.3 percent received fluorinated steroids (one of these women started taking these after the diagnosis of congenital heart block).

Based on the above recurrence/occurrence rates, researchers expected the rate of recurrent cardiac neonatal lupus among the study’s participants to be 17.2 percent. Of the 24 pregnancies where the mothers took hydroxychloroquine, only one child with was born with cardiac neonatal lupus – a recurrence/occurrence rate of only 4.2 percent, about 75.7 percent less than expected.

These results led researchers to conclude that, in these high-risk pregnancies, hydroxychloroquine may decrease the risk for delivering a second child with cardiac neonatal lupus.

“This case series, made possible by the U.S. Research Registry for Neonatal Lupus and our collaborators in Europe, suggests that for women with anti-SSA/Ro antibodies who have had a previous child with neonatal lupus, the use of hydroxychloroquine during a subsequent pregnancy may reduce the risk of having a child with serious life-threatening heart disease,” explains Peter M. Izmirly—assistant professor, New York University School of Medicine, New York and lead investigator in the study—who also notes that additional studies should be completed to confirm this observation.

Patients should talk to their rheumatologists to determine their best course of treatment.