Cure pulmonary arterial hypertension associated with HIV (PAH-HIV)? ... Toward an answer

PAH-HIV is a severe condition associated with HIV infection. Guidelines propose to associate a specific PAH treatment with a highly active anti-retroviral therapy (HAART). Recent studies report some excellent response to Bosentan treatment, with hemodynamic normalisation and long term benefit of this treatment. But, there is no data upon specific treatment discontinuation We report here two patients with persistent remission after Bosentan cessation. Patients were women, IV drugs users, one of African origin 36-years old, the other Caucasian of 46-years old. Diagnosis of PH was performed respectively one year and twenty years after diagnosis of HIV infection. Both patients received HAART and Bosentan. Because of persistent normalization of hemodynamic and functional parameters, bosentan was withdrawn respectively 5 and 1 years after beginning. Both patients remain asymptomatic with normal hemodynamic results respectively at 42 and 12 months after bosentan discontinuation. Data are illustrated on figure for both patients. Conclusion: We propose herein to go one step further in PAH treatment with these two cases of cured PAH-HIV, despite more than three years of Bosentan discontinuation. But we recommended not stopping treatment without complete clinical, hemodynamic and immunological persistent normalisation during at least one year.