Macrophage activation syndrome associated with neonatal lupus erythematosus

Area of Science:

• Neonatal immunology
• Autoimmune diseases
• Rheumatology

Background:

• Neonatal lupus erythematosus (NLE) is a multisystem inflammatory condition caused by maternal autoantibodies.
• NLE presents with skin, cardiac, hepatic, and hematologic manifestations, and can be a cause of neonatal fever.
• Macrophage activation syndrome (MAS) is a rare but serious complication of NLE, with unknown pathogenesis.

Purpose of the Study:

• To report a case of NLE-associated MAS.
• To analyze the serum cytokine profile in a patient with NLE-associated MAS.
• To explore the potential pathophysiology of MAS in the context of NLE.

Main Methods:

• Case report of a neonate diagnosed with NLE-associated MAS.
• Serum cytokine analysis including IL-6, sTNF-RII, and IFN-α at different disease stages.
• Comparison of cytokine profiles with known patterns in MAS secondary to systemic lupus erythematosus.

Main Results:

• The patient presented with NLE and subsequently developed MAS.
• Elevated serum IL-6 and sTNF-RII levels were observed at disease onset.
• During the MAS phase, serum sTNF-RII levels were markedly increased, and serum IFN-α remained persistently elevated.

Conclusions:

• MAS can complicate NLE, presenting with a distinct cytokine signature.
• The cytokine profile in NLE-associated MAS, particularly elevated sTNF-RII and IFN-α, suggests shared pathophysiological mechanisms with MAS secondary to systemic lupus erythematosus.
• Further research is warranted to elucidate the precise mechanisms driving MAS in NLE.