THU0595 SUCCESSFUL COMBINED IMMUNE-MODULATING THERAPY OF IN UTERO MYOCARDITIS AS THE MANIFESTATION OF NEONATAL LUPUS IN A RO/SS-A ANTIBODY EXPOSED NEWBORN
Authors: E. Kiss, Á. Szappanos
Journal: Annals of the Rheumatic Diseases (2020)

Background: Congenital heart block (CHB) occurs in 2-5% of pregnancies due to maternal Ro/SSA and/or anti-La/SSB antibodies, and has a recurrence rate of 12-20% in the following pregnancy. The highest risk for CHB of the foetus is in the gestational age between 16- and 28-weeks. Without immunotherapy, this autoantibody mediated disorder comes with substantial mortality with a rate of 15-30%. Ro/SSA and/or anti-La/SSB antibodies attack the atrioventricular node with the subsequent inflammation and fibrosis. In addition, other cardiac manifestations are known such as endocardial fibroelastosis, dilated cardiomyopathy, and valvular insufficiency. The early clinical diagnosis in utero is essential to be specified due to myocardial tissue damages can be reversible. In the prevention and in the confirmed cardiac involvement the first line therapies are chloroquine, dexamethasone and intravenous immunoglobulins, and also a regular foetal echocardiography is of essential importance. Objectives: Main objective of this report is description of successful treatment of an anti-SS-A antibody exposed foetus with cardiac manifestation. Methods: Case report of a 25-year-old pregnant woman and her baby. The mother was diagnosed with Sjogren’s syndrome in 2013. In previous case history there were two late foetal deaths at the 23rd and 33rd gestational age in 2016 and 2017, respectively as a consequence of foetal bradycardia. During her 2nd pregnancy the mother received chloroquine and azathioprine. At present she was admitted to our Institute in October 2019 at 23rd weeks of gestation without any compliant and any abnormality of pregnancy. Foetal development was normal. Mother received azathioprine and chloroquine from the beginning of pregnancy and dexamethasone from the 16th weeks of gestation. Foetal echocardiography was performed at the 16th gestational week, and every week thereafter. Reflective areas, reflecting oedema and inflammation, appeared at 24th gestational week, localised to the left atrium. Results: The case was referred, and the combo therapy was completed with 1 mg/maternal kg intravenous immune globulin, dexamethasone dose was increased to 4 mg for a week, then decreased to 2 mg. Intratect was given every 2nd week. Progression was stopped as according to control foetal echocardiography after the 2nd infusion. After 4th IVIG the involved area of myocarditis decreased significantly, localised to anterior wall of left atrium and the atrial primum septum. However, at 32nd g. week pericardial fluid was visualised in maximum 9 mm width without signs of pericardial tamponade. At the end of last December, the baby was born at the 35th gestational week with 50 cm and 2570 g and no signs of any congenital anomaly. Pericardial fluid was 4 mm maximum. Her development is normal. Conclusion: Neonatal lupus with various cardiac manifestations may develop in anti-SS-A antibody exposed babies. Therefore, these pregnancies require stringent gynaecologic and cardiologic controls. Although congenital heart block is the most frequent complication developing between the 18-28 th gestational weeks, other manifestations also may occur. High dose intravenous immune globulin therapy can be effective even after failure to combined traditional immune suppressant therapy. References: [1]Congenital heart block: Pace earlier (Childhood) than later (Adulthood). Manolis AA, Manolis TA, Melita H, Manolis AS. Trends Cardiovasc Med. 2019 Jun 20. [2]Autoimmune-mediated congenital heart block. Wainwright B, Bhan R, Trad C, Cohen R, Saxena A, Buyon J, Izmirly P. Best Pract Res Clin Obstet Gynaecol. 2019 Oct 8. Disclosure of Interests: Emese Kiss Consultant of: EK has received consultancy fees from Egis., Ágnes Szappanos: None declared