Download Maternal SLE with ANA, anti-Ro/ SSA and anti-La

Maternal SLE with ANA, anti-Ro/ SSA and anti-La/ SSB antibodies and fetal congenital heart block
1,2
Madhusudan Dey1, R Agarwal2 , Vijayalakshmi N3
Reader, 3 Resident : Armed Forces Medical College, Pune, India
Introduction :
• Pregnancy among the anti-SSA/Ro-SSB/La positive women is commonly
associated with different kinds of complications such as renal involvement,
proteinuria and preterm delivery (1).
• Congenital autoimmune atrioventricular (AV) block is usually seen in association
with autoimmune antibodies in mother that cross the placenta and damage the AV
node of fetus. The incidence of CHB is 2% in cases of maternal anti-Ro/ SSA
antibody positivity, 3% when both anti Ro/SSA and anti-La-SSB are positive. The
risk of recurrence is 9 times higher in the subsequent pregnancies (2).
• Complete congenital fetal heart block related to maternal anti-Ro/SSA
autoantibodies typically develops between 20 and 24 weeks of gestation. CHB
with a structurally normal heart is frequently associated with maternal
autoantibodies to Ro/SSA and La/SSB.
Aim and objectives :
• To know the incidence of fetal CHB in patients of SLE who had ANA, anti-Ro/ SSA
and anti-La/ SSB positivity.
TABLE 1: Maternal characteristics of SLE +ve patients
Patient No Age
ANA
Anti Ro/
SSA
Anti La/
SSB
1
24
+
+
2
27
+
3
22
4
Discussion :
GA at
delivery
Mode of
delivery
+
CHB
diagnosed
at POG
34w
35w6d
VD
+
-
Nil
36w3d
LSCS
+
-
-
Nil
38w4d
LSCS
23
+
+
+
24w
35w3d
LSCS
5
21
-
-
-
Nil
37w5d
VD
6
7
8
9
10
11
12
24
28
31
32
26
24
26
+
+
+
+
+
+
+
+
+
+
+
+
+
Nil
Nil
Nil
Nil
nil
Nil
nil
38w
39w1d
36w2d
37w1d
38w6d
36w
37w5d
LSCS
LSCS
VD
LSCS
LSCS
VD
VD
13
25
+
-
-
Nil
38w
VD
Materials & methods :
TABLE 2: Fetal-Newborn features of SLE +ve mothers
• Prospective study with data collection for 33 months from Jan 2012 to Sep 2014.
• Pregnant women previously diagnosed as a case of SLE or diagnosed during the
present pregnancy were included in the study and were followed up till 6 months post
delivery. After diagnosis of SLE, all the mothers underwent blood test for ANA (if not
done earlier), anti Ro/ SSA and anti Ro/ SSB.
• All the fetuses underwent first or second trimester aneuploidy screening, anomaly
scan at 18-20 weeks period of gestation and fetal echocardiography at 18-22 weeks
period of gestation. Pregnant mother was treated during antenatal period with
dexamethasone (4 mg/ day) after detection of fetal congenital heart block and all her
previous medications (if any) were continued throughout during pregnancy.
• Data have been collected regarding the following aspects: Socio-demographic data;
Maternal characteristics in current pregnancy; Indication of delivery; Mode of
delivery; Apgar score and NICU admissions.
Results :
•Total number of deliveries were 9115 during the 33 months study period and makes
the incidence of SLE was 0.14%. 12 out of 13 SLE patients had ANA positive status.
06 patients had Anti Ro/ SSA and 05 patients had Anti La/ SSB positivity.
•Among the Anti Ro/ SSA and Anti La/ SSB positive patients only 02 had fetal
congenital heart block ( Table 2) and both the fetus had complete heart block and
one of them required permanent pacemaker placement at 5 months of age.
Patient
No
Sex
GA at
delivery
Birth
weight
Apgar
score
F
CHB
Lower
diagnose fetal HR
d at POG
34w
76/ min
35w6d
2.3
8,9
Age at
pacemaker
required
5 months
1
2
3
4
M
M
F
Nil
Nil
24w
52/ min
36w3d
38w4d
35w3d
2.6
2.7
2.1
4,6
9,9
7,9
-
5
6
7
8
9
10
11
12
13
F
M
M
M
F
F
M
M
F
Nil
Nil
Nil
Nil
Nil
nil
Nil
nil
Nil
-
37w5d
38w
39w1d
36w2d
37w1d
38w6d
36w
37w5d
38w
3.2
2.75
3.5
2.45
2.1
2.4
2.6
2.9
3.6
7,9
7,9
7,9
7,9
7,9
7,9
7,9
7,9
7,9
-
Features
of block
Complete
heart block
Complete
heart block
-
• Total number of deliveries were 9115 during the 33 months study period and
makes the incidence of SLE was 0.14%. 12 out of 13 SLE patients had ANA positive
status. 06 patients had Anti Ro/ SSA and 05 patients had Anti La/ SSB positivity.
• Among the Anti Ro/ SSA and Anti La/ SSB positive patients only 02 had fetal
congenital heart block and both the fetus had complete heart block and one of them
required permanent pacemaker placement at 5 months of age.
• The frequency of CHB in a primigravida with positive antibodies is 1-7.5%;
however, the recurrence rate in subsequent pregnancies is about 2-3 times higher,
i.e., around 20% (3). Risk factors for recurrence other than a previous child affected
with CHB are positive anti-SSA/Ro and/ or anti-SSB/La antibodies (3), and the
presence of human leukocyte antigen-DR3 in the mother (4).
• Fetal congenital heart block was diagnosed in two fetuses at 34 w and 24 w period
of gestation respectively. The fetus which was diagnosed CHB at 24 weeks of POG,
received antenatal corticosteroids in the form of tab dexamethasone 4mg/ day. Both
the fetus had complete congenital heart block as detected by fetal
echocardiography which was confirmed by neonatal echocardiography after
delivery.
• Regular and close monitoring for heart block and transplacental therapy with
fluorinated steroids (dexamethasone) have shown satisfactory results at first
evidence of heart block and it is beneficial in first and second degree heart block
but once fetal third-degree block is detected, it is irreversible regardless of
treatment (2).
• For treatment of CHB early pacemaker insertion may be required in some
newborns and permanent pacemaker placement is eventually needed in most
children with congenital heart block.
Conclusion :
• Most of the fetal congenital heart blocks are associated with maternal anti-Ro/ SSA
and anti-La/ SSB antibodies.
•Treatment by steroids may improve the outcome in early stages of fetal CHB and
delivery should be planned in a tertiary care centre where pacemaker placement
facility is available.
References :
1. Alshohaib S. Outcome of pregnancy in patients with inactive systemic lupus erythematosus and minimal proteinuria. Saudi J Kidney
Dis Transpl. 2009;20(5):802-5.
2. Carolis SD, Salvi S, Botta A, Santucci S, Martino C, Garofalo S et al. Which Intrauterine Treatment for Autoimmune Congenital Heart
Block? The Open Autoimmunity Journal, 2010, 2, 1-10
3. Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M, et al. Autoimmune-associated congenital heart block: Mortality,
morbidity, and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol. 1998; 31:1658–1666.
4. Olah KS, Gee H. Fetal heart block associated with maternal anti-Ro (SS-A) antibody--current management. A review. Br J Obstet
Gynaecol 1991;98:751-5.