Scandinavian journal of rheumatology
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Isolated congenital heart block (CHB) detected in utero is strongly associated with autoantibodies reactive with the intracellular soluble ribonucleoproteins 48 kD SSB/La, 52 kD SSA/Ro, and 60 kD SSA/Ro. An erythematous skin rash with a predilection for the scalp and periorbital area, most often apparent in the first few postnatal months, is also highly associated with these maternal antibodies. The permanent cardiac disease and transient cutaneous disease are the most common manifestations of the neonatal lupus syndromes. ⋯ Numerous anecdotal cases support the use of dexamethasone for treatment of effusions and hydrops and possibly incomplete block. To further efforts both at the bench and bedside, national registries have been established in the U. S. and Canada.