Abdulsamet ERDEN, Antonıs FANOURİAKİS, Levent KILIÇ, Alper SARI, Berkan ARMAĞAN, Emre BİLGİN, Yusuf Ziya ŞENER, Benazir HYMABACCUS, Fatih GÜRLER, Serdar CEYLAN, Sedat KİRAZ, Ömer KARADAĞ, Dımıtrıous 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 maternal autoantibodies, often with devastating consequences. The objective of this systematic literature review was to analyze the demographic data, 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 August 2015, using the MeSH terms "neonatal lupus" or "congenital heart block". To be included in the study, articles of any type original articles, 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 reported clinical manifestations of NLE were congenital heart block CHB, 65.2% , cutaneous lupus 33.1% , and cytopenias 15.5% . We found differences in NLE characteristics based on study geographical origin, with CHB being much more frequent in patients of European or American 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 or methodologic discrepancies. While maternal autoimmune disease is not a prerequisite for the development of NLE, the existence of a truly "immunonegative" CHB is questionable.

Keywords:

neonatal lupus, congenital heart block, geoepidemiology,

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