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

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Pediatric Rheumatology20086 (Suppl 1) :P236

https://doi.org/10.1186/1546-0096-6-S1-P236

  • Published:

Keywords

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

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)

 

MAS BM+

MAS BM-

SLE-GI

SLE-MS

Fever

95.0

83.3

21.2

64.2

Hepatomegaly

47.4

55.6

12.1

10.4

CNS dysfunction

37.5

28.6

3.0

8.5

Haemorrhages

40.0

33.3

9.1

NA

Leukopenia

90.0

44.4

63.6

NA

Thrombocytopenia

90.0

61.1

18.2

NA

Hypertransaminasemia

80.0

93.8

30.3

NA

Hypertriglyceridemia

75.0

88.2

20.0

NA

Hypofibrinogenemia

37.5

42.9

0

NA

Hyperferiitinemia

92.9

94.4

0

NA

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.

Authors’ Affiliations

(1)
IRCCS G. Gaslini, Genova, Italy
(2)
Hospital General de Ninos Pedro de Elizalde, Buenos Aires, Argentina
(3)
IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy
(4)
Department of Pediatrics, University of Calgary, Calgary, AB, Canada
(5)
Hopital Necker Enfants Malades, Paris, France
(6)
Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina
(7)
Hacettepe University Children's Hospital, Ankara, Turkey
(8)
Children's Hospital Central California, Madera, CA, USA
(9)
Hospital das Clinicas, Faculdade de Medicina de Botucatu, Botucatu, Brazil
(10)
Great Ormond Street Hospital for Children, London, UK
(11)
Jaslok Hospital and Research Centre, Mumbay, India
(12)
Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
(13)
Rheumatic Children's Hospital, Garmisch-Partenkirchen, Germany
(14)
Department of Pediatric Rheumatology, University Hospital of Leuven, Leuven, Belgium
(15)
Hospital de Clinicas Caracas, Caracas, Venezuela
(16)
Universidad Nacional de Colombia, Bogotà, Colombia
(17)
Children's Hospital, Seattle, WA, USA
(18)
Children's Clinical University Hospital, Riga, Latvia
(19)
Ospedale di Acireale, Acireale, Italy
(20)
Ospedale Pediatrico Bambino Gesù, Roma, Italy
(21)
IRCCS G. Gaslini and Università di Genova, Genova, Italy

Copyright

© Parodi et al; licensee BioMed Central Ltd. 2008

This article is published under license to BioMed Central Ltd.

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