Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 243.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26634252
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Pediatr+Rheumatol+Online+J
2015 ; 13
(ä): 55
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Evidence-based diagnosis and treatment of macrophage activation syndrome in
systemic juvenile idiopathic arthritis
#MMPMID26634252
Boom V
; Anton J
; Lahdenne P
; Quartier P
; Ravelli A
; Wulffraat NM
; Vastert SJ
Pediatr Rheumatol Online J
2015[Dec]; 13
(ä): 55
PMID26634252
show ga
BACKGROUND: Macrophage activation syndrome (MAS) is a severe and potentially
lethal complication of several inflammatory diseases but seems particularly
linked to systemic juvenile idiopathic arthritis (sJIA). Standardized diagnostic
and treatment guidelines for MAS in sJIA are currently lacking. The aim of this
systematic literature review was to evaluate currently available literature on
diagnostic criteria for MAS in sJIA and provide an overview of possible
biomarkers for diagnosis, disease activity and treatment response and recent
advances in treatment. METHODS: A systematic literature search was performed in
MEDLINE, EMBASE and Cochrane. 495 papers were identified. Potentially relevant
papers were selected by 3 authors after which full text screening was performed.
All selected papers were evaluated by at least two independent experts for
validity and level of evidence according to EULAR guidelines. RESULTS: 27 papers
were included: 7 on diagnosis, 9 on biomarkers and 11 on treatment. Systematic
review of the literature confirmed that there are no validated diagnostic
criteria for MAS in sJIA. The preliminary Ravelli criteria, with the addition of
ferritin, performed well in a large retrospective case-control study. Recently,
an international consortium lead by PRINTO proposed a new set of diagnostic
criteria able to distinguish MAS from active sJIA and/or infection with superior
performance. Other promising diagnostic biomarkers potentially distinguish MAS
complicating sJIA from primary and virus-associated hemophagocytic
lymphohistiocytosis. The highest level of evidence for treatment comes from
case-series. High dose corticosteroids with or without cyclosporine A were
frequently reported as first-line therapy. From the newer treatment modalities,
promising responses have been reported with anakinra. CONCLUSION: MAS in sJIA
seems to be diagnosed best by the recently proposed PRINTO criteria, although
prospective validation is needed. Novel promising biomarkers for sJIA related MAS
are in need of prospective validation as well, and are not widely available yet.
Currently, treatment of MAS in sJIA relies more on experience than evidence based
medicine. Taking into account the severity of MAS and the scarcity of evidence,
early expert consultation is recommended as soon as MAS is suspected.
|Adrenal Cortex Hormones/administration & dosage/therapeutic use
[MESH]
|Arthritis, Juvenile/*complications
[MESH]
|Biomarkers/analysis
[MESH]
|Cyclosporine/administration & dosage/therapeutic use
[MESH]
|Drug Therapy, Combination
[MESH]
|Humans
[MESH]
|Immunosuppressive Agents/administration & dosage/therapeutic use
[MESH]