LONG-TERM OUTCOMES OF POSTNATAL IMMUNOSUPPRESSIVE THERAPY IN CHILDREN WITH CARDIAC NEONATAL LUPUS ERYTHEMATOSUS
Bibliographic record
Abstract
Objectives Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune condition. The antibody-mediated neonatal heart disease of NLE is associated with a high risk of mortality and adverse health outcomes. Prenatal treatment can slow progression to complete heart block and improve neonatal survival, but the impacts of postnatal immunosuppressant treatment have not been described. This study describes the outcomes of children with antibody-mediated cardiac NLE treated with postnatal therapy. Methods We reviewed patients consented from the LE clinic in a tertiary pediatric center born between January 1, 1997 - June 10, 2024. We identified patients with cardiac manifestations who received postnatal immunosuppressants. The protocol typically included 1 dose of intravenous immunoglobulin (IVIG) 2g/kg, pulse corticosteroid of 30 mg/kg/day for 3 days, followed by 2 mg/kg/day for 4 weeks with tapering guided by troponin levels. We reviewed medical records with long-term outcomes supplemented by patient/parent questionnaires. We report the prevalence of features and outcomes using summary statistics. Results We reviewed 33 patients with cardiac manifestations of NLE and postnatal therapy. Of the total cohort, 28 (85%) mothers received prenatal immunosuppressive therapy with dexamethasone, IVIG and/or a beta agonist. The median duration of follow-up was 6.04 years (IQR: 2.47 - 11.36 years). The majority (70%) of patients presented with heart block at birth (first-degree/second-degree atrioventricular block [n=7], complete heart block [n=16]). The remaining 10 patients had extranodal manifestations of cardiac inflammation, including echogenic endocardium and valvular regurgitation. Of the 7 patients with first or second-degree block at birth, 4 progressed to complete heart block within an average of 11 months. One patient with first-degree block had complete reversal to normal sinus rhythm. Of the 18 (70%) patients that required a permanent pacemaker, 83% were paced within the first year of life. Of all patients that developed complete heart block in their lifetime, 2 never required pacing (age at last follow-up 17.38 and 19.25 years). No patients required a heart transplant. Three patients developed device-associated or sternal wound infections an average of 1.1 months after birth. Mortality in the cohort was 9% with cause of death attributed to circulatory shock due to sepsis and an unrelated genetic syndrome. Conclusion We report on the outcomes of a large series of children with cardiac NLE who received postnatal immunosuppressive therapy. The vast majority of patients had good outcomes. Postnatal immunosuppressive therapy may be responsible for improved cardiovascular outcomes in children with antibody-mediated neonatal heart disease.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".