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Pulmonary arterial hypertension in SLE: what do we know?
11Department of Rheumatology, School of Immunity and Infection, University of Birmingham, Birmingham, UK.
Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is common, often caused by pulmonary arterial hypertension (PAH). Early diagnosis and treatment, including newer therapies and immunosuppression, improve survival for SLE-PAH patients.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Pulmonary hypertension (PH) is a serious complication of systemic lupus erythematosus (SLE).
- Pulmonary arterial hypertension (PAH) is the most frequent cause of PH in SLE patients.
- Approximately 15% of connective tissue disease-associated PH cases are linked to SLE.
Purpose of the Study:
- To highlight the importance of recognizing and diagnosing PH in SLE.
- To discuss the multifactorial pathogenesis and common causes of PH in SLE.
- To review diagnostic approaches and prognostic factors for SLE-associated PAH.
Main Methods:
- Review of international PH registry data.
- Analysis of clinical presentation and diagnostic tools for PH in SLE.
- Evaluation of treatment strategies and survival outcomes in SLE-PAH.
Main Results:
- PH in SLE can be independent of lupus activity in other organs.
- Echocardiography aids in accurate PH detection by assessing right heart variables and systolic pulmonary artery pressure.
- Antiphospholipid antibodies may serve as predictors for SLE-PAH.
Conclusions:
- Increased awareness is crucial for early diagnosis of PH in SLE due to subtle early symptoms.
- Prompt treatment with modern PAH therapies and immunosuppression improves outcomes.
- Survival in SLE-PAH is better than in systemic sclerosis-associated PH but worse than idiopathic PAH; pregnancy poses significant risks.
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