• Abdulsamet Erden, Antonis Fanouriakis, Levent Kiliç, Alper Sari, Berkan Armağan, Emre Bilgin, Yusuf Ziya Şener, Benazir Hymabaccus, Fatih Gürler, Serdar Ceylan, Sedat Kiraz, Ömer Karadağ, and Dimitrious Boumpas.
• Division of Rheumatology, Department of Internal Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey
• Turk J Med Sci. 2020 Jan 9; 50 (1): 281-290.

Background/AimNeonatal 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 MethodsWe 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.ResultsA total of198 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.ConclusionsPhenotypic 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.This work is licensed under a Creative Commons Attribution 4.0 International License.

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