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Congenital heart block and familial connective tissue disease
The Journal of Rheumatology
|October 1, 1983
Insights
Maternal systemic lupus erythematosus can lead to congenital heart block in children, who may later develop connective tissue diseases. This rare association is documented in only a few cases.
Area of Science:
- Rheumatology
- Pediatrics
- Cardiology
Background:
- Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect multiple organs.
- Congenital heart block (CHB) is a rare condition where the electrical signals controlling the heart rate are impaired from birth.
- Neonatal lupus is a condition that can occur in infants born to mothers with certain autoantibodies.
Observation:
- Two cases are presented of children born to mothers with SLE.
- Both children were diagnosed with congenital heart block shortly after birth.
- Subsequently, both children developed features of connective tissue diseases.
Findings:
- This report adds to the limited literature describing the association between maternal SLE, CHB, and the later development of connective tissue diseases in offspring.
- The findings suggest a potential transplacental transfer of autoantibodies from mother to fetus, leading to cardiac and systemic autoimmune manifestations.
- The progression from CHB to broader connective tissue disease highlights a potential spectrum of neonatal lupus complications.
Implications:
- These cases underscore the importance of cardiac monitoring for infants born to mothers with SLE.
- Further research is warranted to understand the immunological mechanisms linking maternal SLE, CHB, and childhood connective tissue diseases.
- Early recognition and management may improve outcomes for affected children.
Abstract:
Two children of mothers with systemic lupus erythematosus had congenital heart block and subsequently developed connective tissue diseases. This association has only been described in 2 individuals previously.