Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood

Tadej Avcin; Shirley M L Tse; Rayfel Schneider; Bo Ngan; Earl D Silverman

doi:10.1016/j.jpeds.2005.12.070

Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood

J Pediatr. 2006 May;148(5):683-6. doi: 10.1016/j.jpeds.2005.12.070.

Authors

Tadej Avcin¹, Shirley M L Tse, Rayfel Schneider, Bo Ngan, Earl D Silverman

Affiliation

¹ Division of Rheumatology and Department of Pathology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

PMID: 16737887
DOI: 10.1016/j.jpeds.2005.12.070

Abstract

We describe 3 patients who presented with features of macrophage activation syndrome (MAS) at the time of presentation of systemic lupus erythematosus (SLE), systemic juvenile idiopathic arthritis, and Kawasaki disease. Immunohistochemical studies in the patient with SLE demonstrated extensive expression of CD163 on hemophagocytic macrophages, suggesting a possible role as a marker of MAS.

Publication types

Case Reports

MeSH terms

Adolescent
Antigens, CD / metabolism
Antigens, Differentiation, Myelomonocytic / metabolism
Arthritis, Juvenile / complications*
Arthritis, Juvenile / immunology
Arthritis, Juvenile / metabolism
Female
Humans
Infant
Lupus Erythematosus, Systemic / complications*
Lupus Erythematosus, Systemic / immunology
Lupus Erythematosus, Systemic / metabolism
Macrophage Activation*
Male
Mucocutaneous Lymph Node Syndrome / complications*
Mucocutaneous Lymph Node Syndrome / immunology
Mucocutaneous Lymph Node Syndrome / metabolism
Receptors, Cell Surface / metabolism
Syndrome

Substances

Antigens, CD
Antigens, Differentiation, Myelomonocytic
CD163 antigen
Receptors, Cell Surface