Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants

Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms...

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Main Authors: Ji Yeon Song, Su Eun Park, Joung-Hee Byun, Narae Lee, Young Mi Han, Shin Yun Byun, Seong Heon Kim
Format: Article
Language:English
Published: MDPI AG 2021-07-01
Series:Journal of Clinical Medicine
Subjects:
Online Access:https://www.mdpi.com/2077-0383/10/14/3195
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author Ji Yeon Song
Su Eun Park
Joung-Hee Byun
Narae Lee
Young Mi Han
Shin Yun Byun
Seong Heon Kim
author_facet Ji Yeon Song
Su Eun Park
Joung-Hee Byun
Narae Lee
Young Mi Han
Shin Yun Byun
Seong Heon Kim
author_sort Ji Yeon Song
collection DOAJ
description Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan National University Children’s Hospital between January 2009 and December 2020 and their mothers. Twenty-seven patients with NLE comprising 13 male patients (48.1%) and 14 female patients (51.9%) were included. The most common symptom was rash (40.7%), followed by fever (25.9%), arrhythmia (14.8%), splenomegaly (11.1%), and intrauterine growth retardation (7.4%). Seven patients with fever had various organ system manifestations, including cutaneous (100%), hematological (71.4%), hepatobiliary (57.1%), and central nervous system (CNS; 28.6%) manifestations. Two of the febrile patients had aseptic meningitis. Cutaneous, cardiac, hematological, hepatobiliary, and CNS involvement were noted in 44.4%, 18.5%, 51.9%, 40.7%, and 22.2% of the patients, respectively. Systemic lupus erythematosus (SLE) was the most common maternal disease (14/27, 51.9%). Ten mothers (37.0%) had not been diagnosed with any autoimmune disease until their babies were diagnosed. Among them, three were subsequently diagnosed with SLE, five were diagnosed with the Sjögren’s syndrome, and two of them still had no known diagnosis of any autoimmune disorder. Fever is a common symptom of NLE; thus, when there is no clear focus of fever in infants, NLE needs to be considered, especially in cases with skin rashes.
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spelling doaj.art-63c3e0526eaa487a9890864b9d4b0dc92023-11-22T04:08:11ZengMDPI AGJournal of Clinical Medicine2077-03832021-07-011014319510.3390/jcm10143195Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young InfantsJi Yeon Song0Su Eun Park1Joung-Hee Byun2Narae Lee3Young Mi Han4Shin Yun Byun5Seong Heon Kim6Department of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Pusan National University Children’s Hospital, Yangsan 50612, KoreaDepartment of Pediatrics, Seoul National University Children’s Hospital & College of Medicine, Seoul 03080, KoreaNeonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan National University Children’s Hospital between January 2009 and December 2020 and their mothers. Twenty-seven patients with NLE comprising 13 male patients (48.1%) and 14 female patients (51.9%) were included. The most common symptom was rash (40.7%), followed by fever (25.9%), arrhythmia (14.8%), splenomegaly (11.1%), and intrauterine growth retardation (7.4%). Seven patients with fever had various organ system manifestations, including cutaneous (100%), hematological (71.4%), hepatobiliary (57.1%), and central nervous system (CNS; 28.6%) manifestations. Two of the febrile patients had aseptic meningitis. Cutaneous, cardiac, hematological, hepatobiliary, and CNS involvement were noted in 44.4%, 18.5%, 51.9%, 40.7%, and 22.2% of the patients, respectively. Systemic lupus erythematosus (SLE) was the most common maternal disease (14/27, 51.9%). Ten mothers (37.0%) had not been diagnosed with any autoimmune disease until their babies were diagnosed. Among them, three were subsequently diagnosed with SLE, five were diagnosed with the Sjögren’s syndrome, and two of them still had no known diagnosis of any autoimmune disorder. Fever is a common symptom of NLE; thus, when there is no clear focus of fever in infants, NLE needs to be considered, especially in cases with skin rashes.https://www.mdpi.com/2077-0383/10/14/3195neonatal lupus erythematosusfeverinfantanti-SSA/Roanti-SSB/LaSLE
spellingShingle Ji Yeon Song
Su Eun Park
Joung-Hee Byun
Narae Lee
Young Mi Han
Shin Yun Byun
Seong Heon Kim
Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
Journal of Clinical Medicine
neonatal lupus erythematosus
fever
infant
anti-SSA/Ro
anti-SSB/La
SLE
title Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
title_full Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
title_fullStr Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
title_full_unstemmed Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
title_short Neonatal Lupus Erythematosus as a Rare Cause of Fever in Young Infants
title_sort neonatal lupus erythematosus as a rare cause of fever in young infants
topic neonatal lupus erythematosus
fever
infant
anti-SSA/Ro
anti-SSB/La
SLE
url https://www.mdpi.com/2077-0383/10/14/3195
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