Macrophage Activation Syndrome in Children with Systemic-Onset Juvenile Chronic Arthritis

Vol. 113, No. 2, 2005

Free Abstract Article (Fulltext) Article (PDF 154 KB)

Original Paper

Macrophage Activation Syndrome in Children with Systemic-Onset Juvenile Chronic Arthritis
Shinji Kounami, Megumi Yoshiyama, Keiko Nakayama, Masumi Okuda, Shuji Okuda, Noriyuki Aoyagi, Norishige Yoshikawa

Departmentof Pediatrics, Wakayama Medical University, Wakayama City, Japan

Address of Corresponding Author

Acta Haematol 2005;113:124-129 (DOI: 10.1159/000083450)

Key Words

Cyclosporin A
Hemophagocytic lymphohistiocytosis
Macrophage activation syndrome
Urine 2-microglobulin

Abstract

Macrophage activation syndrome (MAS) is a life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile chronic arthritis (SOJCA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study assessed the clinical features of nine MAS events in five children with SOJCA. Nonremitting fever and decreased platelet and white blood cell counts led to a diagnosis of MAS. The urinary beta 2-microglobulin ( beta 2MG) level was a sensitive indicator of MAS. Serum levels of beta 2MG and soluble interleukin-2 receptor were also elevated. These biologic markers reflecting hyperactivated cellular immunity are useful indicators of MAS. Four children treated with cyclosporin A (CSP) achieved rapid and complete recovery, but one patient without CSP died due to rapidly progressive respiratory failure. All children treated with CSP responded quickly, and fever abated within 36 h of initiation of treatment. CSP should be added to first-line therapy of MAS.

Author Contacts

Shinji Kounami
Department of Pediatrics, Wakayama Medical University
811-1 Kimiidera
Wakayama City, 641-0012 (Japan)
Tel. +81 73 441 0633, Fax +81 73 444 9055, E-Mail nami@mail.wakayama-med.ac.jp

Article Information

Received: April 2, 2004
Accepted: May 26, 2004
Number of Print Pages : 6
Number of Figures : 1, Number of Tables : 5, Number of References : 18

Medline Abstract (ID 15802891)

Download Citation

Cited In

This journal is part of the fourth subject package of the Karger

Journal Archive Collection

Information on packages (PDF)
Free sample issues

For non-native English speakers and international authors who would like assistance with their writing before submission, we suggest American Journal Experts for their scientific editing service.