Neonatal lupus erythematosus
| Neonatal lupus erythematosus | |
|---|---|
| Specialty | Neonatology, Immunology |
Neonatal lupus erythematosus is an autoimmune disease in an
Pathogenesis
Ro/SSA and La/SSB are proteins found inside cells. Anti-Ro/SSA and anti-La/SSB are antibodies that form against these proteins. These antibodies can be seen in autoimmune diseases, the most common being Lupus and Sjögren's. Mothers can have these antibodies circulating in their blood without having any signs or symptoms of an autoimmune disease.
Babies born to mothers with these antibodies have a chance of developing neonatal lupus erythematosus.
Fetal susceptibility and environmental factors could also play a role in pathogenesis since not all infants develop congenital heart block even when exposed to antibodies.[2]
Clinical manifestations
Neonatal lupus can present with several signs and symptoms. The most common manifestations involve the heart and skin. Problems involving the liver, gallbladder, brain, and blood can be seen but are usually transient.[1]
Heart
Cardiac manifestations present more commonly in utero, but can also present after birth. The most common complications are varying degrees of
Common complications
- First degree heart block
- Second degree heart block
- Third degree heart block (Complete heart block)
- Endocardial fibroelastosis
Other complications seen with neonatal lupus
- Patent ductus arteriosus
- Patent foramen ovale
- Pulmonic stenosis
- Pulmonary valvular dysplasia
- Fusion of chordae tendineae of the tricuspid valve
- Ostium secundum type atrial septal defects
Skin
A rash can be seen upon delivery. It is commonly found on the head and face, but can also be found on other parts of the body. It is most commonly seen around the eyes. The rash can be described as raised, red, and ring-shaped. The rash is not always visible at birth and can become more prominent after
- Red, ring-shaped rash of face and head
- Telangiectasia
Liver and gallbladder
Severity in which the liver is affected can range from mildly elevated liver enzymes to liver failure.[1][2][4]
- Elevated transaminases
- Hyperbilirubinemia
- Cholestasis
- Hepatitis
Blood
The conditions listed below have been reported with no issues of bleeding or sepsis.[1][2][4]
Brain
Although the conditions below have been reported, it is still uncertain that these manifestations are related to anti-Ro/SSA and anti-La/SSB antibodies.[1] Majority of the neurologic conditions were found incidentally with no neurological signs or symptoms present and did not lead to physical disability or need for surgery.[1][2]
- Hydrocephalus
- Macrocephaly
- Vasculopathy
- Hypocalcemic seizures
- Spastic diplegia
Diagnosis
An infant is diagnosed with neonatal lupus if maternal antibodies, anti-Ro/SSA, anti-La/SSB, or less commonly
Screening
Screening includes testing for maternal antibodies and evaluating for heart block in utero. Universal screening is not recommended.[1][2] Screening is usually performed when there is a higher likelihood for neonatal lupus such as individuals who are more likely to have antibodies due to autoimmune diseases or individuals who have had prior pregnancies complicated with neonatal lupus. If a fetus develops heart block, screening for maternal antibodies can be considered. Monitoring for heart block can be done using a fetal echocardiogram.[1]
Management
Infants with neonatal lupus are managed with supportive care. This means treating or monitoring the symptoms that can occur from this disease. For example, avoiding sunlight so that the infant's rash won't worsen. Many of the manifestations are transient, but once complete heart block occurs, it is irreversible. Heart block can be managed in utero if diagnosed during pregnancy. Infants born to mothers with anti-Ro/SSA and anti-La/SSB should have an ECG performed to check for heart abnormalities if none were seen while in the uterus.[5]
In utero
Fetal heart block treatment varies based on the degree. First degree heart block is usually treated with glucocorticoids, but it can also reverse on its own. As of right now, treatment guidelines for first-degree heart block is controversial due to lack of evidence. Second degree heart block commonly progresses to complete heart block. Second degree heart block can also reverse on its own. Treatment includes fluorinated glucocorticoids and immunoglobulin therapy. Third degree heart block is irreversible, and many treatments have been attempted without success. Management is mainly expectant. Early delivery should be avoided unless other complications arise. In third degree heart block, if the ventricular heart rate drops below 50-55 beats per minute, maternal beta-antagonists can be given. Glucocorticoids and immunoglobulin therapy can be used for endocardial fibroelastosis, but effectiveness is still unclear.[5]
See also
References
- ^ a b c d e f g h i j k l m Buyon JP. "Neonatal lupus: Epidemiology, pathogenesis, clinical manifestations, and diagnosis". UpToDate. Retrieved March 9, 2022.
- ^ OCLC 1051140253.)
{{cite book}}: CS1 maint: location missing publisher (link - ^ PMID 22973504.
- ^ OCLC 1150240738.)
{{cite book}}: CS1 maint: location missing publisher (link - ^ a b c Buyon JP (11 August 2020). "Neonatal Lupus: Management and outcomes". UpToDate. Retrieved March 14, 2022.
- ^ Sauer WH (3 June 2021). Link MS, Yeon SB (eds.). "Etiology of Atrioventricular block". UpToDate. Retrieved March 10, 2022.
- ^ Cooper LT. "Definition and classification of the Cardiomyopathies". UpToDate. Retrieved March 10, 2022.