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SAT0567 Demographic, Clinic and Laboratory Features of 755 Patients with Neonatal Lupus Erythematosus: Systematic Review
  1. A. Erden1,
  2. L. Kilic1,
  3. E. Bilgin2,
  4. S. Ceylan2,
  5. B. Hymabaccus2,
  6. Y.Z. Sener2,
  7. F. Gürler2,
  8. A. Sari1,
  9. B. Armagan1,
  10. O. Karadag1,
  11. S. Kiraz1,
  12. U. Kalyoncu1
  1. 1Department of Rheumatology
  2. 2Department of Internal Medicine, Hacettepe University Faculty of Medicine, Ankara, Turkey

Abstract

Background Neonatal lupus erythematosus (NLE) which is caused by trans placental transmission of maternal anti SS-A/Ro and anti SS-B/La antibodies. Cutaneous, cardiac, hepatic and hematologic manifestations can be seen.

Objectives The objective of this systematic review was to analyze demographic data, clinical manifestations, autoantibodies, treatment modalities and mortality status of NLE patients.

Methods Systematic review has been done at August 2015 on Pubmed Medline. Publications are determined by using MeSH term: (“neonatal lupus, congenital hearth block” (MeSH)) and restricted to “in humans” and “in English”. Out of study design, abstracts of all publications has been assessed. 1377 article has been assessed in abstract format, after that; 277 original article/case report has been evaluated in full-text format. Finally, 199 article/ case report has been included to study after full-text evaluation. Totally, 755 patients enrolled. Demographic, clinical, laboratory features were assessed both maternal and infant level.

Results Maternal features: Mean maternal age was 29.7 (4.8) years. Median gestational week was 23 (21–40) weeks. Accompanying diseases were known in 547 patients (SLE 176 (32.2%), Sjogren syndrome 99 (18.1%), undifferentiated connective tissue disease 31 (5.7%), rheumatoid arthritis 7, other autoimmune disease 53 (9.6%), no disease 179 (32.7%)). ANA results were known in 349 mothers with 287 (82.2%) positivity. ENA were known in 601 mothers (SS-A 506 (84.2%), SS-B 313 (52.1%), anti-RNP 31 (5.2%), negative 57 (9.1%)). In 327 mothers, diagnosis time of collagen tissue disease was known (212 (64.8%) mothers before pregnancy, 18 (5.5%) during pregnancy and 97 (29.7%) postpartum period). Maternal treatments before pregnancy were known in 137 of mothers (glucocorticoid 38 (27.7%), hydroxychloroquine 13 (9.5%), azathioprine 4 (2.9%), IVIG 2 (1.4%), and no treatment 94 (68.6%)).

Infants' features: Totally, 272/477 (57.0%) was female. Mean birth weight was 2680 (364–4370) gram, median delivery week was 37 (19–42) and 126 of 295 (42.7%) NLE was premature. Autoantibodies were known in 272 infants (225 (82.7%) SS-A, 126 SS-B (46.3%), 25 (9.2%) anti-RNP, all negative 20 (7.4%)). Mortality data were available for 485 infants with 412 (84.9%) were alive (85 with pacemaker), 37 (7.6%) were intrauterine exitus, and 36 (7.4%) were post-partum exitus.

NLE features: Most common manifestations of NLE was; cardiac 467 (61.8%), cutaneous 249 (33.1%), hematologic 117 (15.5%), and hepatic 78 (10.5%). Cardiac involvement types were known in 284 infants (AV complete block 219 (77.1%), 2. block 27 (9.5%), 1. block 19 (6.7%), and sinus bradycardia 19 (6.7%). Totally, 62 of 195 (31.2%) AV complete block detected post-partum period.

Conclusions NLE was cause of premature (almost 43%) delivery and had high mortality ratio (almost 15% of infants). SLE and Sjogren's syndrome were leading maternal diagnosis. Although ANA and SS-A are main maternal autoantibodies, SS-B may also be found half of the cases. Congenital heart block, particularly AV block, is life-treating involvement, and unfortunately one of three infants have diagnosed at post-partum period.

Disclosure of Interest None declared

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