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Báo cáo y học: " From antibody insult to fibrosis in neonatal lupus – the heart of the matter"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: From antibody insult to fibrosis in neonatal lupus – the heart of the matter. | Arthritis Research Therapy Vol 5 No 6 Buyon and Clancy Commentary From antibody insult to fibrosis in neonatal lupus -the heart of the matter Jill P Buyon and Robert M Clancy Department of Rheumatology Hospital for Joint Diseases New York University School of Medicine New York USA Corresponding author Robert M Clancy e-mail bobdclancy@aol.com Received 14 Mar 2003 Accepted 26 Mar 2003 Published 25 Sep 2003 Arthritis Res Ther 2003 5 266-270 DOI 10.1186 ar763 2003 BioMed Central Ltd Print ISSN 1478-6354 Online ISSN 1478-6362 Abstract Few diseases exemplify the integration of research from bench to bedside as well as neonatal lupus often referred to as a model of passively acquired autoimmunity. In essence this disease encompasses two patients both the mother and her child. The signature histologic lesion of autoimmune-associated congenital heart block is fibrosis of the conducting tissue and in some cases the surrounding myocardium. It is astounding how rapid and in most cases irreversible is the fibrotic response to injury. The mechanism by which maternal anti-SSA Ro-SSB La antibodies initiate and perpetuate inflammation and eventuate in scarring of the atrioventricular node is not yet defined. In vitro and in vivo studies suggest that one pathologic cascade leading to scarring may be initiated via apoptosis resulting in translocation of SSA Ro-SSB La antigens and subsequent surface binding by maternal autoantibodies. These opsonized cardiocytes are phagocytosed by macrophages which secrete factors that transdifferentiate fibroblasts into myofibroblasts a scarring phenotype. Dissecting the individual components in this fibrotic pathway should provide insights into the rarity of irreversible injury and should form the basis of rational approaches to prevention and treatment. Keywords anti-SSA Ro and SSB La antibodies congenital heart block myofibroblasts neonatal lupus Introduction Following the seminal observation in the early 1980s that sera from nearly all mothers