PMID- 31848493
OWN - NLM
STAT- MEDLINE
DCOM- 20191226
LR  - 20200917
IS  - 1671-167X (Print)
IS  - 1671-167X (Linking)
VI  - 51
IP  - 6
DP  - 2019 Dec 18
TI  - [Clinical characteristics and treatment outcomes of macrophage activation 
      syndrome in adults: A case series of 67 patients].
PG  - 996-1002
AB  - OBJECTIVE: To described the clinical and laboratory features and outcome of 67 
      macrophage activation syndrome (MAS). METHODS: A total of 67 MAS patients from 
      three centers from January 2007 to December 2017 were enrolled. Clinical and 
      laboratory features, and response to therapy were analyzed. Predictive factors 
      for remission and survival were explored. RESULTS: We identified a mean age of 
      (36.1+/-16.3) years at diagnosis of MAS and a median connective tissue disease 
      (CTD) duration of 8 months prior to MAS development. Among 67 MAS patients 
      identified, underlying diseases included adult-onset Still's disease (AOSD) in 
      56.7% and systemic lupus erythematosus (SLE) in 30.0%. Fever and splenomegaly 
      were found in 100.0% and 82.1% of the patients, respectively. Ferritinemia and 
      elevation of serum soluble interleukin-2 receptor was seen in 100.0% and 93.2% of 
      the patients. Serum levels of alanine aminotransferase, D-dimer, ferritin and C 
      reactive protein were significantly higher in MAS associated with the AOSD 
      patients than in MAS associated with the SLE patients. A significant decrease of 
      erythrocyte sedimentation rate was found in MAS associated with AOSD, as compared 
      with MAS associated with SLE. The most commonly used therapy was corticosteroids, 
      which were initially administered in 100.0% of the patients. Intravenous 
      immunoglobulin (IVIG) was administered in 91.0%, cyclosporine A in 64.2%, and 
      etoposide in 46.3% of the patients, respectively. The induction therapy yielded a 
      complete remission (CR) at the end of week 8 in 47.8% of the MAS patients. The 
      overall mortality rate at the end of week 16 was 22.4%. The median serum levels 
      of gamma-glutamyltransferase, alkaline phosphatase, total bilirubin and direct 
      bilirubin were significantly lower in the patients who achieved complete 
      remission at the end of week 8 than in those who did not, and splenomegaly was 
      significantly less frequent (71.9% vs.91.4%, P=0.037). Both the mean age at 
      diagnosis of MAS and the mean age at diagnosis of underlying CTD of the deceased 
      patients were elder than those of the survived population (P=0.014 and P=0.017, 
      respectively). The platelet count was significantly less in the deceased 
      population as compared with the living population (P=0.018). No addition of 
      cyclosporine A (P=0.004) was identified as risk factors associated with death in 
      Logistic regression analysis. CONCLUSION: MAS secondary to connective tissue 
      disease is most common with AOSD and SLE. In terms of laboratory findings, there 
      were considerable differences between the patients with underlying SLE and those 
      with AOSD. Advanced age and low platelet counts are significant predictive 
      factors for death, while treatment with cyclosporine may reduce the risk.
FAU - Yao, H H
AU  - Yao HH
AD  - Department of Rheumatology and Immunology, Peking University People's Hospital, 
      Beijing 100044, China.
FAU - Wang, Y N
AU  - Wang YN
AD  - Department of Hematology, Beijing Friendship Hospital, Capital Medical 
      University, Beijing 100050, China.
FAU - Zhang, X
AU  - Zhang X
AD  - Department of Rheumatology and Immunology, Peking University People's Hospital, 
      Beijing 100044, China.
FAU - Zhao, J X
AU  - Zhao JX
AD  - Department of Rheumatology and Immunology, Peking University Third Hospital, 
      Beijing 100191, China.
FAU - Jia, Y
AU  - Jia Y
AD  - Department of Rheumatology and Immunology, Peking University People's Hospital, 
      Beijing 100044, China.
FAU - Wang, Z
AU  - Wang Z
AD  - Department of Hematology, Beijing Friendship Hospital, Capital Medical 
      University, Beijing 100050, China.
FAU - Li, Z G
AU  - Li ZG
AD  - Department of Rheumatology and Immunology, Peking University People's Hospital, 
      Beijing 100044, China.
LA  - chi
PT  - Journal Article
PL  - China
TA  - Beijing Da Xue Xue Bao Yi Xue Ban
JT  - Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health 
      sciences
JID - 101125284
RN  - 83HN0GTJ6D (Cyclosporine)
SB  - IM
MH  - Adult
MH  - Cyclosporine
MH  - Humans
MH  - *Macrophage Activation Syndrome
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Still's Disease, Adult-Onset
MH  - Treatment Outcome
MH  - Young Adult
PMC - PMC7433608
EDAT- 2019/12/19 06:00
MHDA- 2019/12/27 06:00
PMCR- 2019/12/18
CRDT- 2019/12/19 06:00
PHST- 2019/12/19 06:00 [entrez]
PHST- 2019/12/19 06:00 [pubmed]
PHST- 2019/12/27 06:00 [medline]
PHST- 2019/12/18 00:00 [pmc-release]
AID - bjdxxbyxb-51-6-996 [pii]
AID - 10.19723/j.issn.1671-167X.2019.06.003 [doi]
PST - ppublish
SO  - Beijing Da Xue Xue Bao Yi Xue Ban. 2019 Dec 18;51(6):996-1002. doi: 
      10.19723/j.issn.1671-167X.2019.06.003.