ABSTRACT

Neonatal lupus erythematosus is an acquired autoimmune disease which is characterised by transplacental transition of anti- SS-A/Ro and anti-SS-B/La antibodies and binding of these antibodies to fetal tissues. Cutaneous, cardiac, hepatic and hematological manifestations can be seen. Most important and serious complication is congenital heart block and this situation is a major cause of mortality approximately in 20-30% of all patients. Effectivity of current theurepatic approaches are still a questionmark. Aim of this study is to review current literature systematically to compare different treatment options.

Pubmed Medline database was literature review in August 2015. Relevant publications were seeked using the MeSH terms "neonatal lupus" and "congenital heart block"; results were restricted to studies in humans and articles in the English language. After recruiting all eligable publications, when available, treatment protocols of with congenital heart block were noted case-by-case.

Treatment protocols of 243 (49.3%) over 492 patients were noted. Major drug classes and their percentage of use are as follow: Steroids in 106 (43.6%) patients, intravenous immunoglobulin in 14 (5.7%) patients and hydroxychloroquine in 5 (2%) patients. 134 (55.1%) patients had no treatment. Type of steroid was known of 92 (86.7%) patients and it was dexamethasone, prednisolon and betamethasone in 54 (58.6%), 27 (29.3%), 11 (11.9%) patients, respectively.

There is no pre-defined, accepted treatment regimen of congenital heart block. There is a large spectrum of treatment options and dosage of these options. Another question is that approxiamately half of the patients do not take any medication related to congenital heart block. Also another important point is that even prednisolon can not across the placental membrane, prednisolon was found to be used very common.