Skip to main content

Advertisement

Macrophage activation syndrome (MAS) in juvenile systemic lupus erythematosus (JSLE): an underrecognized complication?

Article metrics

  • 1891 Accesses

Objective

To define the characteristics of MAS complicating JSLE.

Methods

Patients with JSLE and MAS were collected from: 1) Gaslini Institute of Genoa, Italy; 2) PRINTO and PRCSG investigators; 3) literature. Control groups of JSLE without MAS included 33 patients with active lupus seen at Gaslini Institute (SLE-GI) and 387 patients from a multinational study of damage in JSLE (SLE-MS). Clinical and laboratory features of MAS with (BM+) or without (BM-) bone marrow demonstration of haemophagocytosis were contrasted each other and with those of lupus without MAS.

Results

20 BM+ and 18 BM-patients with JSLE-associated MAS were identified. Comparison of percentage frequency of the main clinical and laboratory features of MAS in patient groups is shown in table 1.

Table 1 Comparison of percentage frequency of the main clinical and laboratory features of MAS in partient groups. (NA: not available)

Conclusion

Features of MAS in patients with or without BM haemophagocytosis were comparable, except for a greater frequency of leukopenia in BM+ patients. This suggests that this complication is more common than previously realized. All features but leukopenia and fever discriminated well between MAS and active lupus without MAS.

Author information

Correspondence to A Parodi.

Rights and permissions

Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Reprints and Permissions

About this article

Cite this article

Parodi, A., Davì, S., Pringe, A. et al. Macrophage activation syndrome (MAS) in juvenile systemic lupus erythematosus (JSLE): an underrecognized complication?. Pediatr Rheumatol 6, P236 (2008) doi:10.1186/1546-0096-6-S1-P236

Download citation

Keywords

  • Public Health
  • Bone Marrow
  • Systemic Lupus Erythematosus
  • Patient Group
  • Leukopenia