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FRI0258 Long-term prognosisofpatients with systemic lupus erythematosus-associated pulmonary arterial hypertension: cstar-pah cohort study
  1. J Qian1,
  2. M Li2,
  3. X Zhang3,
  4. Q Wang2,
  5. J Zhao2,
  6. Z Tian4,
  7. X Zeng2,
  8. on behalf of CSTAR group
  1. 1Internal Medicine
  2. 2Rheumatology, Peking Union Medical College Hospital, Beijing
  3. 3Rheumatology, The People's Hospital of Guangdong Province, Guangzhou
  4. 4Cardiology, Peking Union Medical College Hospital, Beijing, China


Background SLE-associated pulmonary arterial hypertension (PAH) is common in Asian countries, and the clinical outcome of patients with SLE-associated PAH is dramatically impaired.

Objectives This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with SLE-associated PAH confirmed by right heart catheterization (RHC).

Methods A multicenter cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary endpoint was death from any cause. The secondary experimental end point was treatment goal achievement (TGA), defined as an integrated outcome.

Results Among the 310 patients enrolled from 14 PAH centers, the median follow-up was 24.0 months. The 1-, 3- and 5-year survival rates were 92.1%, 84.8% and 72.9%, respectively. The 1-, 3- and 5-year TGA rates were 31.5%, 53.6% and 62.7%, respectively.Serositis, 6MWD >380 m and CI ≥2.5 L/min×m2 were identified as independent prognostic factors of TGA. TGA within 5 years was identified as a factor associated with survival in patients with SLE-associated PAH (Figure 1).

Table 1.

Baseline characteristics of patients with SLE-associated PAH

Conclusions TGA was associated with the long-term survival, which supports and provides evidence to the treat-to-target strategy in SLE-associated PAH. Early diagnosis, intervention and heart function preservation are priorities for better long-term outcomes.


  1. Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376–87.

  2. Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138:1383–94.

  3. Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179:151–7.


Disclosure of Interest None declared

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