School of Medicine Publications
Document Type
Article
Publication Date
8-16-2026
Abstract
Congenital complete atrioventricular block (CCAVB) is a well-recognized manifestation of neonatal lupus erythematosus (NLE), caused by transplacental transfer of maternal anti-Ro/Sjögren's Syndrome Type A (SSA) and anti-La/Sjögren's Syndrome Type B (SSB) antibodies. Although non-cardiac manifestations of NLE typically resolve within the first year of life, the long-term autoimmune risk in affected offspring remains unclear. We report a 24-year-old woman with CCAVB secondary to maternal Sjögren's disease who presented with acute heart failure, nephrotic-range proteinuria, bicytopenia, pericardial effusion, and findings concerning for Libman-Sacks endocarditis. Despite negative conventional lupus serologies, renal biopsy demonstrated Class IV/V lupus-like immune-complex glomerulonephritis with full-house immune complex deposition, leading the multidisciplinary team to favor seronegative lupus nephritis. This case demonstrates the diagnostic limitations of serology-based approaches in atypical lupus presentations and underscores the critical role of tissue biopsy. The case also raises questions regarding long-term autoimmune risk after autoimmune congenital heart block, an area for which prospective data and evidence-based surveillance strategies remain limited.
Recommended Citation
Dacak, T. M., Rodriguez, A., Gomez, L. F., Haines, C. N., & Asif, N. (2026). Maternal Sjögren's Disease and Its Long Shadow: Adult-Onset Seronegative Lupus Nephritis Following Congenital Heart Block. Cureus, 18(8), e114594. https://doi.org/10.7759/cureus.114594
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Publication Title
Cureus
DOI
10.7759/cureus.114594
Academic Level
faculty
Mentor/PI Department
Internal Medicine
Comments
Copyright © 2026, Dacak et al.
This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.