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Neurological and endocrinological involvement in neonatal lupus erythematosus: a retrospective study at a tertiary hospital in Eastern China

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Abstract

Introduction

Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that causes transient impairment of multi-organ functions and is mainly caused by maternally transmitted antibodies.

Objective

This study aims to investigate the clinical features of infants with NLE, focusing on the presence of neurological and endocrinological involvement.

Methods

The clinical data of infants diagnosed with NLE at the Children's Hospital of Soochow University from 2011 to 2022 were collected and retrospectively analyzed.

Result

In all, 39 patients with NLE were included, and the most common symptom was rash, followed by hematological, hepatic, cardiac, gastrointestinal, neurological, and endocrine symptoms. Among the 10 patients with neurological impairment, intracranial hemorrhage was the most common, followed by convulsions, hydrocephalus, extracerebral space enlargement, and aseptic meningitis. All patients with neurological impairment were positive for anti-SSA/Ro antibodies. Five of these patients were double positive for anti-SSA/Ro and anti-SSB/La antibodies. All 10 patients had multi-organ system involvement, with hematological involvement being the most common, and three patients had varying degrees of developmental delay at the post-discharge follow-up. Nine patients with endocrine impairment were positive for anti-SSA/Ro antibodies, with pancreatic impairment being the most common. There were four cases of hyperinsulinemia and hypoglycemia, one case of diabetes mellitus with ketoacidosis, two cases of hypothyroidism, one case of hypoadrenocorticism, and one case of lysinuric protein intolerance, all of which were normalized before discharge. All patients with endocrine impairment showed hematological involvement, and some showed feeding intolerance as their first symptom. One patient had abnormal liver function at post-discharge follow-up, and two patients had a rash caused by a severe allergy to milk protein.

Conclusions

At our hospital, no significant gender differences were observed in the occurrence of NLE, and a predominance of skin, blood, liver, and heart involvement was observed. Patients with multiple central nervous system injuries and organ involvement are more likely to have growth retardation. Endocrine disorders are transient in NLE patients, and some showed feeding intolerance as the first manifestation.

Key Points

•A retrospective investigation of the clinical characteristics and prognosis of 39 NLE patients was performed, focusing on the clinical features of patients with neurological and endocrine system involvement to improve clinicians' understanding of this disease.

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Data availability

The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding authors.

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Funding

This study was financially supported by the National Natural Science Foundation of China (No. 81971423, 82271741), Jiangsu Provincial Social Development Key General Project (No. BE2020658), and Jiangsu Provincial Health and Family Planning Commission Medical Research Project (No. ZD2021013).

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Author notes

  1. Wenqiang Sun and Li Ding have contributed equally to this work.

Authors and Affiliations

  1. Department of Neonatology, Children’s Hospital of Soochow University, Suzhou, China

    Wenqiang Sun, Li Ding, Mengzhao Li, Changchang Fu, Zihao Yang & Xueping Zhu

Authors
  1. Wenqiang Sun
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  2. Li Ding
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  3. Mengzhao Li
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  4. Changchang Fu
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  5. Zihao Yang
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  6. Xueping Zhu
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Contributions

WS and LD designed the study and performed the final data analysis. ML, ZY, and CF collected material. XZ supervised the design and execution of the study and contributed to the writing of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Xueping Zhu.

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Ethics approval

This study complies with the ethical standards approved by the Medical Ethics Committee of the Children’s Hospital of Soochow University. The parents of all infants provided written informed consent.

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Sun, W., Ding, L., Li, M. et al. Neurological and endocrinological involvement in neonatal lupus erythematosus: a retrospective study at a tertiary hospital in Eastern China. Clin Rheumatol 42, 2461–2468 (2023). https://doi.org/10.1007/s10067-023-06622-8

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