Complete heart block in a 40-year-old man with anti-SSA/Ro autoantibodies

Abstract

Atrio-ventricular dissociation (AVD), including complete heart block (CHB), are far more common in the elderly. We report a rare case of CHB in a 40-year-old man, who tested positive for anti-Ro autoantibodies without systemic features. He had been suffering for giddiness over the previous two months. On arrival, his electrocardiogram revealed high-degree AVD. Upon further history, he mentioned that his 68-year-old mother with systemic lupus erythematosus (SLE) had suffered from similar episodes, requiring a permanent pacemaker implantation. On further investigation, he tested positive for antinuclear antibodies (ANA), anti-SSA/Ro and anti-RNP antibodies. However, from history and clinical examination, he had not manifested any articular, extra-articular or extra-glandular features suspicious of rheumatological conditions. Following a failed trial of intravenous hydrocortisone, he subsequently had a permanent pacemaker implanted himself. Although difficult to ascertain whether our patient suffered from a congenital form of anti-SSA/Ro-related CHB, there is evidence to suggest delayed presentation of CHB in those with anti-SSA/Ro and neonatal lupus syndrome. Anti-SSA/Ro antibodies without systemic features can be present in 3% of the population, although this occurs more commonly in the presence of a confirm diagnosis of SLE, Sjögren’s syndrome or poly- and dermatomyositis. Despite the scarcity of evidence, a trial of steroid-based treatment was attempted prior to subjecting the young patient to a permanent pacemaker and its associated complications. To our knowledge, this is only the second case of isolated anti-SSA/Ro syndrome presenting with CHB reported in the literature.