Journal
JOURNAL OF INTERNAL MEDICINE
Volume 265, Issue 6, Pages 653-662Publisher
WILEY
DOI: 10.1111/j.1365-2796.2009.02100.x
Keywords
anti-Ro; La antibodies; congenital heart block; PR interval
Categories
Funding
- NIAMS NIH HHS [1-R01-AR46265, R01 AR042455, R01 AR42455-01, R01 AR046265, R37 AR042455] Funding Source: Medline
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One of the strongest associations with autoantibodies directed to components of the SSA/Ro-SSB/La ribonucleoprotein complex is the development of congenital heart block (CHB) in an offspring, an alarming prospect facing 2% of primigravid mothers with these reactivities. This risk is 10-fold higher in women who have had a previously affected child with CHB. Anti-Ro/La antibodies are necessary but insufficient to cause disease. In vitro and in vivo experiments suggest that the pathogenesis involves exaggerated apoptosis, macrophage/myfibroblast crosstalk, TGF beta expression and extensive fibrosis in the conducting system and in some cases surrounding myocardium. A disturbing observation is the rapidity of disease progression, with advanced heart block and life-threatening cardiomyopathy observed < 2 weeks from normal sinus rhythm. Once 3rd degree (complete) block is identified, reversal has never been achieved, despite dexamethasone. Current strategies include the evaluation of an early echocardiographic marker of injury, such as a prolonged PR interval and the use of IVIG as a preventative measure for pregnancies of mothers with previously affected children.
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