Abdulsamet ERDEN, Berkan ARMAĞAN, Yusuf Ziya SENER, Sedat KİRAZ, Alper SARI, Ömer KARADAĞ, Serdar CEYLAN, Benazir HYMABACCUS, Levent KILIÇ, Antonis FANOURIAKIS, Fatih GÜRLER, Emre BİLGİN, Dimitrious T. BOUMPAS

Geoepidemiology and clinical characteristics of neonatal lupus erythematosus: a systematic literature review of individual patients’ data

Background/aim: Neonatal lupus erythematosus (NLE) is an autoimmune syndrome caused by transplacental transmission of maternalautoantibodies, often with devastating consequences. The objective of this systematic literature review was to analyze the demographicdata, geoepidemiology, clinical, and serological characteristics associated with NLE.Materials and methods: We performed a systematic literature search of the Pubmed database covering the period from 1976 to August2015, using the MeSH terms “neonatal lupus” or “congenital heart block”. To be included in the study, articles of any type (originalarticles, case series, and case reports) had to report on infants with NLE on an individualized (i.e. patient-by-patient) basis.Results: A total of 198 studies were included in the review, reporting on a total of 755 NLE patients. The most frequently reportedclinical manifestations of NLE were congenital heart block (CHB, 65.2%), cutaneous lupus (33.1%), and cytopenias (15.5%). We founddifferences in NLE characteristics based on study geographical origin, with CHB being much more frequent in patients of European orAmerican descent (49.4% and 35%, respectively), while reports originating from Asia reported a higher prevalence of skin involvement(45.2%). Most CHB cases (72.9%) were diagnosed between the 18th and 26th week of gestation.Conclusions: Phenotypic differences of NLE depending on race and country may reflect true pathophysiologic differences ormethodologic discrepancies. While maternal autoimmune disease is not a prerequisite for the development of NLE, the existence of atruly “immunonegative” CHB is questionable.

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