Abstract
Severe pulmonary hypertension as the first sign of systemic lupus erythematosus (SLE) is uncommon, but has a considerable influence on prognosis compared to other published cases of SLE. However, with the development of new therapeutic alternatives, patient mortality has decreased significantly in recent years. This case, involving a 36-year-old man whose initial presentation of SLE was severe PAH, illustrates the importance of early detection in these individuals to achieve better outcomes.
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