[Pulmonary arterial hypertension and systemic sclerosis].
Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc) and a leading cause of death in patients with it. Recent publications suggest that a prevalence of 10-15% is likely. The prognosis remains poor compared to that of idiopathic PAH. WHO recommends annual echocardiography for PAH screening of patients with SSc. Right heart catheterization is necessary to confirm the diagnosis. Nevertheless, more than half of all SSc patients have symptoms classified as WHO functional class III or IV at diagnosis. Prostacyclin therapy, delivered via continuous intravenous infusion (epoprostenol), has been demonstrated to be effective in patients with severe PAH (both idiopathic and scleroderma-related). Prostacyclin analogs (such as treprostinil and iloprost) are other options. Bosentan is the first endothelin receptor antagonist approved in the EU for the treatment of PAH, both idiopathic and related to connective tissue diseases such as scleroderma, in patients in WHO functional class III. Sildenafil by its selective inhibition of phosphodiesterase type 5 is also effective against both types of PAH. It too is now approved in the EU for this purpose in patients in WHO functional class III, but we do not yet have any information about its long-term effects in scleroderma.
Launay
David
D
Service de Médecine Interne, Centre National de Référence des Atteintes Vasculaires de la Sclérodermie, Hôpital Claude Huriez, CHRU, Lille.
Humbert
Marc
M
Hachulla
Eric
E
D000465:Algorithms; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000627:therapeutic use; D005062:European Union; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D016285:Iloprost / Q000008:administration & dosage / Q000627:therapeutic use; D007262:Infusions, Intravenous; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use; D015995:Prevalence; D011379:Prognosis; D011687:Purines; D016032:Randomized Controlled Trials as Topic; D012595:Scleroderma, Systemic / Q000150:complications*; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use; D013450:Sulfones; D013997:Time Factors; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use; D014944:World Health Organization
D000959:Antihypertensive Agents; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D016285:Iloprost; D000077300:Bosentan; C427248:treprostinil