Macrophage activation syndrome in a newborn infant born to a mother with autoimmune disease

Abstract

Macrophage activation syndrome (MAS) is a complication of rheumatic disorders characterized by cytopenia, multiple organ dysfunction and coagulopathy associated with an inappropriate activation of macrophage. In neonatal lupus erythematosus, MAS is rare but fatal, requiring early diagnosis and treatment for optimal outcome. We report a case of MAS in a neonate born to a mother with autoimmune disease, improved by treatment with steroid, intravenous immunoglobulin and cyclosporine.

A case of neonatal lupus presenting with severe systemic hypertension and multiorgan failure in absence of congenital heart block (CHB): clinical management and recent literature review of neonatal cardiac lupus

“…Plasma amino acids, urine organic acids, urine reducing substances and acylcarnitine and carnitine pro le values were all within normal limits ruling out the common inborn errors of metabolism. The initial thrombocytopenia and anemia associated with increased AST and ferritin raised concern for macrophage activation syndrome (MAS) in the context of maternal alloimmune antibodies, but the quick resolution of the thrombocytopenia and the clinical course did not t with MAS diagnostic criteria 4 . Other rare disorders like Gestational autoimmune liver disease (GALD) and Hemophagocytic lymphohistiocytosis (HLH) were also considered and ruled out given the improvement in the hepatic function after vigorous hemodynamic support by increasing circulating volume.…”

Neonatal Liver Failure With Hemophagocytosis and Skin Rash

“…The diagnosis is challenging in absence of maternal history for connective tissue diseases and other features of lupus may not be present. Most neonates will recover with conservative management; however; immunoglobulin therapy, steroids, and cyclosporine may be required in severe cases associated with macrophage activation syndrome (3,4).…”

A fatal case of hemophagocytic lymphohistiocytosis due to neonatal lupus erythematosus

“…Other complications of NLE, including hemophagocytic lymphohistiocytosis (HLH), are rare. [3][4][5][6][7][8] The prognosis of infants with NLE depends on the organ systems involved. 9 Cardiac involvement is associated with a 17.5% mortality rate, with approximately one-third of patients dying in utero.…”

“…Characteristic clinical manifestations of NLE include a photosensitive periorbital rash, heart block, elevated liver enzymes, and cytopenias. Other complications of NLE, including hemophagocytic lymphohistiocytosis (HLH), are rare 3–8 …”