Pulmonary hypertension associated with systemic lupus erythematosus

Pulmonary hypertension (PH) is a severe form of cardiopulmonary complication that occasionally occurs in patients with systemic lupus erythematosus (SLE). Symptoms are nonspecific and in-depth clinical suspicion besides advanced imaging studies are needed to confirm the diagnosis. Several potential causes of PH are found in SLE patients including thromboembolism and interstitial lung disease, as well as a type of PH indistinguishable from that found in idiopathic pulmonary arterial hypertension. Several auto-antibodies have been associated with the presence of PH in SLE, highlighting anti-Sm and anti-Lal SSB specificities and those related with the antiphospholipid syndrome. In Latin American (Mexico) patients, PH is a late manifestation of SLE and it is correlated with active renal involvement, disease activity index and serum C-reactive protein concentration. Treatment includes standard PH treatment besides corticosteroids, conventional and novel immunosuppressive agents, prostaglandins as well as phosphodiesterase and endothelin-1 inhibitors. Early identification and establishment of therapy may modify the natural history of this life-threatening complication of SLE.