Macrophage activation syndrome in neonatal lupus presenting with fever and rash

Copyright © 2024 by The Korean College of Rheumatology. All rights reserved..

Neonatal lupus can occur in infants born to mother with autoimmune disorders through transplacental auto-antibodies. Clinical manifestations in neonatal lupus include cutaneous lesions and hematologic or hepatobiliary findings resembling those seen in systemic lupus erythematosus. In autoimmune state, macrophage activation syndrome (MAS) represent a critical and potentially fatal complication that can result in mortality if not immediately identified and managed with the appropriate care. Here we present a 33-day-old girl diagnosed with neonatal lupus and serious MAS. She was delivered by a primipara mother who did not exhibit any autoimmune symptoms. The patient visited the hospital due to fever and pancytopenia. Laboratory data were compatible with MAS, including pancytopenia, high level of ferritin, soluble interleukin-2, and decreased natural killer cell activity. In addition, autoimmune study showed positive results for anti-nuclear antibody (ANA), anti-Sjogren syndrome antigen A (SSA), and SSB, The autoimmune study for mother also showed positive results for ANA, anti-SSA, and SSB. The patient recovered after she received high dose steroid and supportive care. Our case indicates that neonatal lupus should be taken into consideration when fever, erythematous skin rash, and pancytopenia are observed in infants, even if their mothers have no prior history of autoimmune conditions.

Medienart:

E-Artikel

Erscheinungsjahr:

2024

2023

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:31

Enthalten in:

Journal of rheumatic diseases - 31(2023), 1 vom: 01. Jan., Seite 49-53

Sprache:

Englisch

Beteiligte Personen:

Yu, Ji Yoon [VerfasserIn]
Kim, Tae Hwan [VerfasserIn]
Kim, Ye Ji [VerfasserIn]
Kang, Hyun Mi [VerfasserIn]
Yoo, In Hyuk [VerfasserIn]
Rhim, Jung Woo [VerfasserIn]
Lee, Soo Young [VerfasserIn]
Jeong, Dae Chul [VerfasserIn]

Links:

Volltext

Themen:

Case Reports
Macrophage activation syndrome
Neonatal systemic lupus erythematosus

Anmerkungen:

Date Revised 23.12.2023

published: Print-Electronic

Citation Status PubMed-not-MEDLINE

doi:

10.4078/jrd.2023.0039

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM366216120