Systemic lupus erythematosus accounts for approximately 15% of connective tissue disease-associated pulmonary hypertension in international registries.
This review highlights the importance of early echocardiographic screening and prompt treatment with PAH therapies and immunosuppression to improve survival in patients with SLE-associated PAH.
Pulmonary hypertension (PH) can occur at any time during the course of systemic lupus erythematosus (SLE), and can be independent of lupus disease activity in other systems. The pathogenesis of PH in SLE can be multifactorial, but pulmonary arterial hypertension (PAH) is the commonest cause of PH in SLE. The international PH registries have published that approximately 15% of connective tissue disease-associated PH is lupus related in their cohorts. As the symptoms of PH in SLE can be mild and non-specific in early stages, an increasing awareness of this devastating complication is essential for early diagnosis. Echocardiographic evaluation of several right heart variables in addition to systolic pulmonary artery pressure estimation reduces false positive rates for PH detection. Antiphospholipid antibodies may predict SLE-PAH. Prompt treatment of PAH with newer PAH therapy as well as immunosuppression can reduce morbidity and prolong survival. The survival in SLE-associated PAH is better compared with systemic sclerosis-associated PH but worse than idiopathic PAH. Pregnancy in SLE-PAH can result in a fatal outcome, especially in severe and poorly controlled PH at onset.
Prabu et al. (2013) conducted a review in Systemic lupus erythematosus-associated pulmonary arterial hypertension. Systemic lupus erythematosus accounts for approximately 15% of connective tissue disease-associated pulmonary hypertension in international registries.