Un CREST syndrome révélé par une hypertension artérielle pulmonaire : à propos d’un cas

Abstract

A CREST syndrome revealed by a pulmonary arterial
hypertension : case report.
Introduction : The high pulmonary arterial blood
pressure is a grave complication, potentially mortal of the
CREST syndrome.
Observation : A 57-year-old woman, suffering from
hypertension and gout, was admitted for a dyspnea class
III of the NYHA and a right heart failure.
The physical examination found a cyanosis of the
extremities with an unsticking nails, crepitant rales at the
the bases of lungs. The immunological search for antibody
circulating anticoagulants of lupique type was negative.
On the other hand that of antibodies antibeta 2
glycoprotein 1 and anti-centromeres antibodies was
positive. The échocardiography found a severe pulmonary
arterial hypertension of 132 mmHg and a pericardial
effusion. Under treatment with calcium inhibitors,
diuretics, D-Penicillamin, the inhospital outcome was
favourable firstly. Secondary, she was admitted in
dermatology three months later for a Raynaud syndrome
and toe necrosis. She died during this hospitalization.
Conclusion : The pulmonary arterial hypertension is
associated more frequently with CREST syndrome as well
as with the presence of serologic antibody anticentromères. It is a severe complication which constitutes
an unfavourable bend in the evolution of the disease
because of the disappointing current therapeutic
measures.