Pulmonary hypertension
Other forms of PAH are far more common. In scleroderma the incidence has been estimated to be 6 to 60% of all patients, in rheumatoid arthritis up to 21%, in systemic lupus erythematosus 4 to 14%, in portal hypertension between 2 and 5%, in HIV about 0.5%, and in sickle cell disease ranging from 20 to 40%.