Predicting Macrophage Activation Syndrome in Childhood-onset Systemic Lupus Erythematosus Patients at Diagnosis
© 2021 by the Journal of Rheumatology..
OBJECTIVE: Macrophage activation syndrome (MAS), a life-threatening inflammatory complication, is increasingly recognized in childhood-onset systemic lupus erythematosus (cSLE). It can be a challenge to differentiate active cSLE from MAS. We generated decision rules for discriminating MAS from active cSLE in newly diagnosed patients.
METHODS: We conducted a retrospective cohort study of consecutive, newly diagnosed, active cSLE patients with fever, requiring hospital admission to The Hospital for Sick Children from January 2003 to December 2007 (cohort 1) and January 2008 to December 2013 (cohort 2). All patients met ≥ 4 American College of Rheumatology or Systemic Lupus International Collaborating Clinics criteria, and were steroid-naïve and infection-free. MAS was diagnosed based on expert opinion. Recursive partitioning was applied to each cohort to derive a decision rule based on clinical and laboratory features, distinguishing MAS from non-MAS cSLE. Each decision rule was applied to the alternate, independent cohort. Sensitivity and specificity of these decision rules were compared to existing criteria.
RESULTS: Cohort 1 (n = 34) and cohort 2 (n = 41) each had 10 patients with MAS. Recursive partitioning in cohort 1 identified ferritin ≥ 699 μg/L as the sole best discriminator between MAS and non-MAS patients (R2 = 0.48), and in cohort 2, ferritin ≥ 1107 μg/L was the best discriminator for MAS, followed by lymphocytes < 0.72 × 103/mm3 (R2 = 0.52). Cross-validation of our decision rules maintained 90-100% sensitivity and 65-85% specificity.
CONCLUSION: Our decision rule demonstrated improved performance compared to preliminary guidelines for MAS in cSLE from the Lupus Working Group of the Paediatric Rheumatology European Society and familial hemophagocytic lymphohistiocytosis diagnostic criteria. Validation in independent cohorts is required.
| Errataetall: |
CommentIn: J Rheumatol. 2021 Sep;48(9):1355-1357. - PMID 34329185 |
|---|---|
| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2021 |
|---|---|
| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
|---|---|
| Enthalten in: |
The Journal of rheumatology - 48(2021), 9 vom: 01. Sept., Seite 1450-1457 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Gerstein, Maya [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Journal Article |
|---|
| Anmerkungen: |
Date Completed 21.10.2021 Date Revised 21.10.2021 published: Print-Electronic CommentIn: J Rheumatol. 2021 Sep;48(9):1355-1357. - PMID 34329185 Citation Status MEDLINE |
|---|
| doi: |
10.3899/jrheum.200292 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM31828524X |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM31828524X | ||
| 003 | DE-627 | ||
| 005 | 20231225165031.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231225s2021 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.3899/jrheum.200292 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1060.xml |
| 035 | |a (DE-627)NLM31828524X | ||
| 035 | |a (NLM)33262295 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Gerstein, Maya |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Predicting Macrophage Activation Syndrome in Childhood-onset Systemic Lupus Erythematosus Patients at Diagnosis |
| 264 | 1 | |c 2021 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 21.10.2021 | ||
| 500 | |a Date Revised 21.10.2021 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a CommentIn: J Rheumatol. 2021 Sep;48(9):1355-1357. - PMID 34329185 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2021 by the Journal of Rheumatology. | ||
| 520 | |a OBJECTIVE: Macrophage activation syndrome (MAS), a life-threatening inflammatory complication, is increasingly recognized in childhood-onset systemic lupus erythematosus (cSLE). It can be a challenge to differentiate active cSLE from MAS. We generated decision rules for discriminating MAS from active cSLE in newly diagnosed patients | ||
| 520 | |a METHODS: We conducted a retrospective cohort study of consecutive, newly diagnosed, active cSLE patients with fever, requiring hospital admission to The Hospital for Sick Children from January 2003 to December 2007 (cohort 1) and January 2008 to December 2013 (cohort 2). All patients met ≥ 4 American College of Rheumatology or Systemic Lupus International Collaborating Clinics criteria, and were steroid-naïve and infection-free. MAS was diagnosed based on expert opinion. Recursive partitioning was applied to each cohort to derive a decision rule based on clinical and laboratory features, distinguishing MAS from non-MAS cSLE. Each decision rule was applied to the alternate, independent cohort. Sensitivity and specificity of these decision rules were compared to existing criteria | ||
| 520 | |a RESULTS: Cohort 1 (n = 34) and cohort 2 (n = 41) each had 10 patients with MAS. Recursive partitioning in cohort 1 identified ferritin ≥ 699 μg/L as the sole best discriminator between MAS and non-MAS patients (R2 = 0.48), and in cohort 2, ferritin ≥ 1107 μg/L was the best discriminator for MAS, followed by lymphocytes < 0.72 × 103/mm3 (R2 = 0.52). Cross-validation of our decision rules maintained 90-100% sensitivity and 65-85% specificity | ||
| 520 | |a CONCLUSION: Our decision rule demonstrated improved performance compared to preliminary guidelines for MAS in cSLE from the Lupus Working Group of the Paediatric Rheumatology European Society and familial hemophagocytic lymphohistiocytosis diagnostic criteria. Validation in independent cohorts is required | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a macrophage activation syndrome | |
| 650 | 4 | |a pediatric systemic lupus erythematosus | |
| 650 | 4 | |a systemic lupus erythematosus | |
| 700 | 1 | |a Borgia, R Ezequiel |e verfasserin |4 aut | |
| 700 | 1 | |a Dominguez, Daniela |e verfasserin |4 aut | |
| 700 | 1 | |a Feldman, Brian M |e verfasserin |4 aut | |
| 700 | 1 | |a Liao, Fangming |e verfasserin |4 aut | |
| 700 | 1 | |a Levy, Deborah M |e verfasserin |4 aut | |
| 700 | 1 | |a Ng, Lawrence |e verfasserin |4 aut | |
| 700 | 1 | |a Abdelhaleem, Mohamed |e verfasserin |4 aut | |
| 700 | 1 | |a Silverman, Earl D |e verfasserin |4 aut | |
| 700 | 1 | |a Hiraki, Linda T |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t The Journal of rheumatology |d 1983 |g 48(2021), 9 vom: 01. Sept., Seite 1450-1457 |w (DE-627)NLM000019593 |x 1499-2752 |7 nnns |
| 773 | 1 | 8 | |g volume:48 |g year:2021 |g number:9 |g day:01 |g month:09 |g pages:1450-1457 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.3899/jrheum.200292 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 48 |j 2021 |e 9 |b 01 |c 09 |h 1450-1457 | ||