Pregnant women with previous child with cardiac neonatal lupus
Conditions
Interventions
Drug: Hydroxychloroquine Those who meet eligibility criteria will receive 400mg/d of HCQ (two 200mg pills) Mothers already on HCQ will remain on 400mg/d, or escalate to 400mg/d if taking less than
Sponsors
Tokyo Metropolitan Tama Medical Center
Eligibility
Sex/Gender
Female
Inclusion criteria
Inclusion criteria: 1. Mothers must have anti-SSA/Ro and/or anti-SSB/La Ab documented. 2. Mothers must have a previous child with cardiac NL, defined herein as: the presence of heart block (1st, 2nd, or 3rd degree) documented by electrocardiogram (EKG), echocardiogram, pacemaker, or statement in the medical record, and/or; presence of cardiac injury, which specifically includes autopsy evidence of a mononuclear infiltrate in the endocardium, myocardium, and pericardium and/or endocardial fibroelastosis (EFE) on echocardiogram always associated with cardiac dysfunction. 3. Pregnancy <=10 weeks. 4. Mother may be taking <=20 mg prednisone 5. Mother may be asymptomatic, or have a rheumatic disease such as SLE or SS. 6. Mother may or may not already be taking HCQ.
Exclusion criteria
Exclusion criteria: 1. Mother does not have Ab to SSA/Ro or SSB/La. 2. Identification of any of the following structural lesions considered causal for CHB, i.e., L-transposition of the great arteries, polysplenia, atrioventricular septal defects, etc) 3. Mother is taking fluorinated steroids (dexamethasone, betamethasone) 4. Contraindication of HCQ: history of hypersensitivity to HCQ, retinopathy (except SLE retinopathy)or maculopathy 5. lack of cooperation of local rheumatologists, obstetricians, and pediatric cardiologists 6. difficulty in visiting hospital or home-visit
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Advanced heart block (type II or III) [screening fetal echocardiography: biweekly from 18 weeks to 26 weeks] | — |
Secondary
| Measure | Time frame |
|---|---|
| 1)Prolonged PR interval (>150msec) [screening fetal echocardiography: biweekly from 18 weeks to 26 weeks, EKG at birth, and at 6 months and one year follow up] 2) Any sign of myocardial injury, without change in cardiac rate or rhythm [fetal echocardiography: biweekly from 18 weeks to 26 weeks] a) shortening fraction <28% = 2 SD below normal mean or qualitatively reduced systolic function; b) cardio-thoracic ratio >0.33; c) hydropic changes; d) moderate/severe tricuspid regurgitation. 3) Echocardiographic densities consistent with EFE confirmed postnatally [screening fetal echocardiography: biweekly from 18 weeks to 26 weeks] 4) Fetal death not related to cardiac dysfunction An autopsy with full evaluation of the heart will be encouraged but cannot be mandated. If AV block or evidence of a cardiomyopathy can be "proven," then these will provide the basis for final categorization. If not possible, the death will not be considered a recurrence rate but will be reported. 5) NL rash [at birth, at 6 months and 1 year follow up] 6) Prematurity [At birth] (gestational age <37 weeks at birth) 7) Birth weight <10% in the context of gestational age [At birth] 8) Abnormal fluid collection [At birth] | — |
Countries
Japan
Contacts
Public ContactNaoto Yokogawa, MD
Tokyo Metropolitan Tama Medical Center Department of Rheumatic Diseases
Outcome results
None listed