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

Abstract

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.

Manifestações clínicas recorrentes do Lúpus Eritematoso Sistêmico (LES) conforme o período de desenvolvimento inicial da patologia

“…A LESj: febre, ulceração oral, perda de peso, artralgia, frequente envolvimento renal, neuropsiquiátrico (psicose, dores de cabeça, disfunção cognitiva, doença cerebrovascular), hematológico (anemia hemolítica, linfopenia, trombocitopenia) e doença mucocutânea que inclui erupção malar, ulceração malar, fotossensibilidade e lesões de vasculite. SMITH et al, 2019;MASSIAS et al, 2020;SONG et al, 2021). Além disso, outro aspecto possível de se destacar é a diferença apresentada no curso da doença e do prognóstico do LES em decorrência do período em que suas apresentações clínicas se iniciam, alguns trabalhos discorrem sobre a atividade mais grave da patologia nos casos de Lúpus Eritematoso Sistêmico Juvenil (LESj) e com início na infância (LESc) em comparação com o Lúpus Eritematoso Sistêmico com início na idade adulta (LESa) (HEDRICH; SMITH; BERESFORD, 2018;LIVINGTON;BONNER apud KIM, 2018;SMITH, 2019).…”

“…Inicialmente, dois estudos trouxeram como o grupo em análise pacientes com Lúpus Eritematoso Neonatal. Ambos os trabalhos evidenciaram que as principais apresentações clínicas são sintomas cutâneos (DERDULSKA et al, 2021;SONG et al, 2021). De modo mais específico, as manifestações na pele se deram em forma de erupções cutâneasexantema e eritema anulare vasculite cutânea (SONG et al, 2021).…”

“…Ambos os trabalhos evidenciaram que as principais apresentações clínicas são sintomas cutâneos (DERDULSKA et al, 2021;SONG et al, 2021). De modo mais específico, as manifestações na pele se deram em forma de erupções cutâneasexantema e eritema anulare vasculite cutânea (SONG et al, 2021). Ademais, nos pacientes neonatos foi possível notar manifestações hepatobiliares, hematológicas, como anemia, trombocitopenia e neutropenia, e achados neurológicos, particularmente, hidrocefalia, macrocefalia e acidente vascular encefálico (AVE) (DERDULSKA et al, 2021;SONG et al, 2021).…”

Neonatal lupus presenting as aseptic meningitis: A diagnostic pitfall

“…Indeed, NLE has not been frequently reported as a cause of CSF pleocytosis in the neonatal population. A case series of 27 NLE patients from South Korea described only 2 patients with aseptic meningitis 5 …”

“…A case series of 27 NLE patients from South Korea described only 2 patients with aseptic meningitis. 5 The pathophysiology of NLE causing aseptic meningitis remains uncertain. Multiple studies postulate that the manifestations of NLE are a result of maternal autoantibodies being deposited and bound to various tissues.…”