Language
Country/Area
No result found
A hidden crisis of the heart: How does neonatal lupus erythematosus lead to irreversible cardiac conduction problems?
Neonatal lupus erythematosus is an autoimmune disease that usually occurs in mothers with anti-Ro/SSA antibodies and with or without anti-La/SSB antibodies. The disease most commonly presents as a rash resembling subacute cutaneous lupus erythematosus and may be accompanied by systemic abnormalities such as complete heart block or hepatosplenomegaly. Although neonatal lupus erythematosus is usually benign and self-limiting, some heart problems may be permanent.
Diagnosis mainly relies on maternal antibodies and clinical manifestations.
Etiology
Anti-Ro/SSA and anti-La/SSB are proteins present in cells, and these proteins induce the production of corresponding antibodies. Generally, these antibodies are observed in autoimmune diseases, the most common of which include lupus and Sjögren's disease. Even if the mother has these antibodies in her blood, she may not show any symptoms of an autoimmune disease. When maternal antibodies cross the placenta and enter the fetal circulation, there is a chance that they can lead to the development of neonatal lupus erythematosus.
These antibodies can affect heart cells, causing cell damage and ultimately fibrosis of the heart's conduction system.
Clinical manifestations
The clinical manifestations of neonatal lupus erythematosus can involve many aspects such as the heart and skin. Although the liver, gallbladder, brain, and blood may also be affected, these problems are usually temporary.
Heart
Clinical signs of the heart are more common during the fetal period but can also appear after birth. The most common complications are heart block and endocardial fibroelastosis. Many newborns are diagnosed with congenital heart block, often associated with neonatal lupus erythematosus. This type of heart block occurs when there is a malfunction in the heart's conduction system, preventing the passage of impulses from the atria to the ventricles.
Heart block may first manifest as a slow heartbeat during the second trimester of pregnancy.
Skin
A rash can appear during childbirth, usually on the head and face, but may appear on other parts of the body. This rash is often red, raised, and ring-shaped. Although this rash is sometimes not obvious at birth, it becomes more obvious with UV exposure.
Liver and gallbladder
The degree of liver damage can range from mild liver enzyme elevations to liver failure, including elevated transaminases and high bilirubin.
Blood
Reported blood problems include anemia, neutropenia, and thrombocytopenia, but these conditions are not usually accompanied by problems with bleeding or sepsis.
Brain
Although some neurological conditions are present, it is currently unclear how these manifestations are related to anti-Ro/SSA and anti-La/SSB antibodies.
Diagnosis
The diagnosis of neonatal lupus erythematosus is based on the presence of maternal antibodies (such as anti-Ro/SSA or anti-La/SSB) and any clinical manifestations. Screening includes testing for maternal antibodies and evaluation for early fetal heart block.
Management
Management of neonatal lupus erythematosus depends primarily on supportive care. Many manifestations are transient, but once complete heart block occurs, it is often irreversible. This requires timely electrocardiography and appropriate management if heart block is discovered during fetal life.
Management of heart block during fetal life depends on its extent.
Heart problems caused by neonatal lupus erythematosus not only affect the immediate health of the baby, but may also have a profound impact on their future health risks. Faced with these challenges, how should we improve awareness and early screening of neonatal lupus erythematosus?
