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

• Published in: Clinical rheumatology 2023-09, Vol.42 (9), p.2461-2468
• Main Authors: Sun, Wenqiang, Ding, Li, Li, Mengzhao, Fu, Changchang, Yang, Zihao, Zhu, Xueping
• Format: Article
• Language: English
• Subjects: Aftercare; Antibodies; Antibodies, Antinuclear; Aseptic meningitis; Autoimmune diseases; blood; Central nervous system; Child; China; Convulsions; Diabetes mellitus; Endocrine disorders; Endocrine System; Exanthema; gastrointestinal system; gender; Growth rate; growth retardation; heart; Hematology; Hemorrhage; hospitals; Humans; Hydrocephalus; Hyperinsulinemia; hypersensitivity; hypoadrenocorticism; Hypoglycemia; Hypothyroidism; Infant; Infant, Newborn; Infants; Intolerance; Ketoacidosis; ketosis; Liver; liver function; Lupus; lupus erythematosus; Lupus Erythematosus, Cutaneous - diagnosis; Lupus Erythematosus, Systemic - diagnosis; Medicine; Medicine & Public Health; Meningitis; milk proteins; Neonates; Neurological complications; Original Article; Patient Discharge; Patients; prognosis; Retrospective Studies; Rheumatology; Sex differences; Systemic lupus erythematosus; Tertiary Care Centers
• ISSN: 0770-3198; 1434-9949; 1434-9949
• DOI: 10.1007/s10067-023-06622-8

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