Perinatal management and long-term follow-up of a primipara with severe pulmonary arterial hypertension associated with systemic lupus erythematosus

Author(s):  
Wentao Song ◽  
Shujuan Chu ◽  
Kai Yang ◽  
Xilong Sun ◽  
Haifa Xia ◽  
...  
2007 ◽  
Vol 66 (9) ◽  
pp. 1259-1262 ◽  
Author(s):  
K. P Ng ◽  
G. Cambridge ◽  
M. J Leandro ◽  
J. C W Edwards ◽  
M. Ehrenstein ◽  
...  

2019 ◽  
Vol 22 (1) ◽  
pp. 111-118 ◽  
Author(s):  
Xiaoxiao Guo ◽  
Jinzhi Lai ◽  
Hui Wang ◽  
Zhuang Tian ◽  
Qian Wang ◽  
...  

Abstract Aims Pulmonary arterial hypertension (PAH) is a serious and devastating complication of systemic lupus erythematosus (SLE), especially when the right ventricle (RV) fails. Whether the ratio between tricuspid annular plane systolic excursion (TAPSE) and pulmonary artery systolic pressure (PASP) measured by echocardiography as a simple surrogate of RV to pulmonary circulation (PC) coupling predicts the outcome of SLE-associated PAH has not been investigated. Methods and results Between February 2010 and August 2015, 112 consecutive patients with a diagnosis of SLE-associated PAH confirmed by right heart catheterization were enrolled prospectively. The endpoint was a composite of all-cause mortality and clinical worsening. Baseline clinical characteristics and echocardiographic assessment were analysed. Among all the patients, 47 (42%) patients experienced the endpoint (mean follow-up period 18.1 ± 12.0 months), including 20 patients who died during a median follow-up period of 48.5 months. Multivariable Cox regression analysis showed that TAPSE/PASP ratio [hazard ratio (HR) 0.004, P = 0.017] and 6-min walk distance (6MWD) (HR 0.997, P = 0.036) were the independent predictors for the endpoint. A three-group prediction risk was created based on combined assessment of the TAPSE/PASP ratio and 6MWD relative to their cut-off values. The patients with the worse RV-PC coupling (TAPSE/PASP <0.184 mm/mmHg) and the lower 6MWD (<395 m) had the highest risk (HR 4.62, confidence interval 2.27–9.41, P < 0.001) of experiencing the endpoint. Conclusion The TAPSE/PASP ratio, combined with 6MWD, provides clinical and prognostic insights into patients with SLE-associated PAH. A low TAPSE/PASP and low 6MWD identifies the subgroup of patients with high risk of poor prognosis.


Rheumatology ◽  
2009 ◽  
Vol 48 (11) ◽  
pp. 1451-1454 ◽  
Author(s):  
M. Aringer ◽  
F. Houssiau ◽  
C. Gordon ◽  
W. B. Graninger ◽  
R. E. Voll ◽  
...  

Lupus ◽  
2016 ◽  
Vol 26 (6) ◽  
pp. 616-622 ◽  
Author(s):  
A Bortoluzzi ◽  
F Furini ◽  
F Campanaro ◽  
M Govoni

Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999–2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.


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