PMID- 24851680
OWN - NLM
STAT- MEDLINE
DCOM- 20150205
LR  - 20140523
IS  - 0376-2491 (Print)
IS  - 0376-2491 (Linking)
VI  - 94
IP  - 13
DP  - 2014 Apr 8
TI  - [Systemic lupus erythematosus associated pulmonary arterial hypertension: 
      clinical analysis of 91 cases].
PG  - 969-72
AB  - OBJECTIVE: To explore the clinical characteristics and therapeutic efficacy of 
      systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension 
      (PAH). METHODS: A total of 91 cases of SLE-PAH from 2007 to 2011 were reviewed 
      and followed up. They were divided into 2 groups: group A: New York Heart 
      Association (NYHA) functional class 1 and 2; group B: NYHA functional class 3 and 
      4. RESULTS: There were 2 males and 89 females with a mean age of 37 +/- 11 years. 
      The mean duration of SLE disease process was 7 +/- 6 years. PAH was the primary 
      symptom of SLE onset in 10 cases. Pulmonary arterial systolic pressure (PASP) as 
      measured by ultrasonic cardiography (UCG) were between 40 to 128 mmHg. Eighteen 
      cases (19.78%) underwent right heart catheterization (RHC). There were good 
      parallels of PASP value between RHC and UCG. The main characteristics included 
      Raynaud's phenomenon (53.8%), pericardial effusion (51.6%) and a high titer of 
      anti-RNP antibody (57.1%). PASP was positively associated with SLE disease 
      activity in mild and moderate cases. Among 27 mortality cases, there were 4 in 
      group A (14.8%) and 23 in group B (85.2%) . And the causes of immortality were 
      mostly non-cardiac in group A and right heart failure in group B. 
      Cyclophosphamide was effective in mild and moderate cases. Forty-four cases 
      received PAH target treatment and it could decrease the PASP in mild and moderate 
      cases and significantly prolong the survival for severe cases. CONCLUSION: The 
      major clinical characteristics of SLE-PAH patients include Raynaud's phenomenon, 
      pericardial effusion and positivity of anti-RNP antibody. PASP is positively 
      associated with SLE disease activity in mild and moderate cases for whom 
      intravenous pulse cyclophosphamide therapy may be effective. For severe cases, 
      concomitant PAH target therapy may significantly improve the prognosis.
FAU - Teng, Jialin
AU  - Teng J
AD  - Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of 
      Medicine, Shanghai 200001, China.
FAU - Zhang, Wei
AU  - Zhang W
AD  - Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of 
      Medicine, Shanghai 200001, China. Email: eyysb@126.com.
LA  - chi
PT  - English Abstract
PT  - Journal Article
PL  - China
TA  - Zhonghua Yi Xue Za Zhi
JT  - Zhonghua yi xue za zhi
JID - 7511141
SB  - IM
MH  - Adult
MH  - Familial Primary Pulmonary Hypertension/complications/*diagnosis/*therapy
MH  - Female
MH  - Humans
MH  - Lupus Erythematosus, Systemic/complications/*diagnosis/*therapy
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Treatment Outcome
EDAT- 2014/05/24 06:00
MHDA- 2015/02/06 06:00
CRDT- 2014/05/24 06:00
PHST- 2014/05/24 06:00 [entrez]
PHST- 2014/05/24 06:00 [pubmed]
PHST- 2015/02/06 06:00 [medline]
PST - ppublish
SO  - Zhonghua Yi Xue Za Zhi. 2014 Apr 8;94(13):969-72.