Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment
Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Baldo, Francesco [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2020 |
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| Schlagwörter: |
Macrophage activation syndrome Hemophagocytic lymphohistiocytosis |
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| Anmerkung: |
© Springer Nature Switzerland AG 2020 |
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| Übergeordnetes Werk: |
Enthalten in: Current treatment options in rheumatology - Berlin [u.a.] : Springer, 2015, 6(2020), 3 vom: 07. Aug., Seite 245-259 |
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| Übergeordnetes Werk: |
volume:6 ; year:2020 ; number:3 ; day:07 ; month:08 ; pages:245-259 |
| Links: |
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| DOI / URN: |
10.1007/s40674-020-00153-y |
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| Katalog-ID: |
SPR040857220 |
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| 520 | |a Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. | ||
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10.1007/s40674-020-00153-y doi (DE-627)SPR040857220 (SPR)s40674-020-00153-y-e DE-627 ger DE-627 rakwb eng Baldo, Francesco verfasserin aut Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Nature Switzerland AG 2020 Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 Beretta, Gisella aut Tibaldi, Jessica aut Caorsi, Roberta aut Minoia, Francesca aut Enthalten in Current treatment options in rheumatology Berlin [u.a.] : Springer, 2015 6(2020), 3 vom: 07. Aug., Seite 245-259 (DE-627)815913990 (DE-600)2806597-9 2198-6002 nnns volume:6 year:2020 number:3 day:07 month:08 pages:245-259 https://dx.doi.org/10.1007/s40674-020-00153-y lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 6 2020 3 07 08 245-259 |
| spelling |
10.1007/s40674-020-00153-y doi (DE-627)SPR040857220 (SPR)s40674-020-00153-y-e DE-627 ger DE-627 rakwb eng Baldo, Francesco verfasserin aut Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Nature Switzerland AG 2020 Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 Beretta, Gisella aut Tibaldi, Jessica aut Caorsi, Roberta aut Minoia, Francesca aut Enthalten in Current treatment options in rheumatology Berlin [u.a.] : Springer, 2015 6(2020), 3 vom: 07. Aug., Seite 245-259 (DE-627)815913990 (DE-600)2806597-9 2198-6002 nnns volume:6 year:2020 number:3 day:07 month:08 pages:245-259 https://dx.doi.org/10.1007/s40674-020-00153-y lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 6 2020 3 07 08 245-259 |
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10.1007/s40674-020-00153-y doi (DE-627)SPR040857220 (SPR)s40674-020-00153-y-e DE-627 ger DE-627 rakwb eng Baldo, Francesco verfasserin aut Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Nature Switzerland AG 2020 Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 Beretta, Gisella aut Tibaldi, Jessica aut Caorsi, Roberta aut Minoia, Francesca aut Enthalten in Current treatment options in rheumatology Berlin [u.a.] : Springer, 2015 6(2020), 3 vom: 07. Aug., Seite 245-259 (DE-627)815913990 (DE-600)2806597-9 2198-6002 nnns volume:6 year:2020 number:3 day:07 month:08 pages:245-259 https://dx.doi.org/10.1007/s40674-020-00153-y lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 6 2020 3 07 08 245-259 |
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10.1007/s40674-020-00153-y doi (DE-627)SPR040857220 (SPR)s40674-020-00153-y-e DE-627 ger DE-627 rakwb eng Baldo, Francesco verfasserin aut Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Nature Switzerland AG 2020 Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 Beretta, Gisella aut Tibaldi, Jessica aut Caorsi, Roberta aut Minoia, Francesca aut Enthalten in Current treatment options in rheumatology Berlin [u.a.] : Springer, 2015 6(2020), 3 vom: 07. Aug., Seite 245-259 (DE-627)815913990 (DE-600)2806597-9 2198-6002 nnns volume:6 year:2020 number:3 day:07 month:08 pages:245-259 https://dx.doi.org/10.1007/s40674-020-00153-y lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 6 2020 3 07 08 245-259 |
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10.1007/s40674-020-00153-y doi (DE-627)SPR040857220 (SPR)s40674-020-00153-y-e DE-627 ger DE-627 rakwb eng Baldo, Francesco verfasserin aut Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © Springer Nature Switzerland AG 2020 Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 Beretta, Gisella aut Tibaldi, Jessica aut Caorsi, Roberta aut Minoia, Francesca aut Enthalten in Current treatment options in rheumatology Berlin [u.a.] : Springer, 2015 6(2020), 3 vom: 07. Aug., Seite 245-259 (DE-627)815913990 (DE-600)2806597-9 2198-6002 nnns volume:6 year:2020 number:3 day:07 month:08 pages:245-259 https://dx.doi.org/10.1007/s40674-020-00153-y lizenzpflichtig Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 6 2020 3 07 08 245-259 |
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Enthalten in Current treatment options in rheumatology 6(2020), 3 vom: 07. Aug., Seite 245-259 volume:6 year:2020 number:3 day:07 month:08 pages:245-259 |
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Macrophage activation syndrome Hemophagocytic lymphohistiocytosis Hemophagocytic syndromes Systemic juvenile idiopathic arthritis Still’s disease Cytokine storm syndromes |
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Baldo, Francesco @@aut@@ Beretta, Gisella @@aut@@ Tibaldi, Jessica @@aut@@ Caorsi, Roberta @@aut@@ Minoia, Francesca @@aut@@ |
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<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">SPR040857220</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20230519121126.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">201007s2020 xx |||||o 00| ||eng c</controlfield><datafield tag="024" ind1="7" ind2=" "><subfield code="a">10.1007/s40674-020-00153-y</subfield><subfield code="2">doi</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)SPR040857220</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(SPR)s40674-020-00153-y-e</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="100" ind1="1" ind2=" "><subfield code="a">Baldo, Francesco</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">2020</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">Text</subfield><subfield code="b">txt</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">Computermedien</subfield><subfield code="b">c</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">Online-Ressource</subfield><subfield code="b">cr</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="500" ind1=" " ind2=" "><subfield code="a">© Springer Nature Switzerland AG 2020</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. 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Baldo, Francesco |
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Baldo, Francesco misc Macrophage activation syndrome misc Hemophagocytic lymphohistiocytosis misc Hemophagocytic syndromes misc Systemic juvenile idiopathic arthritis misc Still’s disease misc Cytokine storm syndromes Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment |
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Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment Macrophage activation syndrome (dpeaa)DE-He213 Hemophagocytic lymphohistiocytosis (dpeaa)DE-He213 Hemophagocytic syndromes (dpeaa)DE-He213 Systemic juvenile idiopathic arthritis (dpeaa)DE-He213 Still’s disease (dpeaa)DE-He213 Cytokine storm syndromes (dpeaa)DE-He213 |
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misc Macrophage activation syndrome misc Hemophagocytic lymphohistiocytosis misc Hemophagocytic syndromes misc Systemic juvenile idiopathic arthritis misc Still’s disease misc Cytokine storm syndromes |
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misc Macrophage activation syndrome misc Hemophagocytic lymphohistiocytosis misc Hemophagocytic syndromes misc Systemic juvenile idiopathic arthritis misc Still’s disease misc Cytokine storm syndromes |
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misc Macrophage activation syndrome misc Hemophagocytic lymphohistiocytosis misc Hemophagocytic syndromes misc Systemic juvenile idiopathic arthritis misc Still’s disease misc Cytokine storm syndromes |
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Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment |
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Baldo, Francesco |
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Baldo, Francesco Beretta, Gisella Tibaldi, Jessica Caorsi, Roberta Minoia, Francesca |
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Baldo, Francesco |
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macrophage activation syndrome in childhood inflammatory disorders: diagnosis, genetics, pathophysiology, and treatment |
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Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment |
| abstract |
Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. © Springer Nature Switzerland AG 2020 |
| abstractGer |
Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. © Springer Nature Switzerland AG 2020 |
| abstract_unstemmed |
Purpose of review Macrophage activation syndrome (MAS) is a life-threatening inflammatory condition, belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), which may severely complicate the course of rheumatologic diseases, mainly systemic juvenile idiopathic arthritis. Although the pathophysiology of MAS is still not completely understood, it is characterized by an abnormal proliferation of T lymphocytes and macrophages, responsible for cytokine overproduction and hemophagocytosis. Because MAS may be rapidly fatal, its prompt recognition is imperative in order to immediately start adequate treatment. This review will overview the main features of MAS occurring in the context of childhood inflammatory conditions, discussing the recent updates in diagnosis, pathophysiology, and management. Recent findings Recently, several diagnostic tools have been developed to recognize MAS in the context of pediatric inflammatory disorders. These are largely based on routinely available clinical and laboratory features and may aid physician in clinical practice. Progress in understanding the pathophysiology of MAS led to a significant improvement in identifying novel therapies targeting pro-inflammatory cytokines, a less toxic approach for children with MAS. Summary In the future, tailored therapy based on the recognition of genetic predisposition, underlying disorder, triggers, and biomarkers will likely improve the outcome of pediatric patients with MAS/sHLH. © Springer Nature Switzerland AG 2020 |
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Macrophage Activation Syndrome in Childhood Inflammatory Disorders: Diagnosis, Genetics, Pathophysiology, and Treatment |
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Beretta, Gisella Tibaldi, Jessica Caorsi, Roberta Minoia, Francesca |
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2024-07-03T18:42:22.714Z |
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| score |
7.4003057 |