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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. 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