[Pulmonary hypertension associated with connective tissue diseases].
OBJECTIVE
Pulmonary hypertension is a rare but well-known life-threatening complication of connective tissue diseases. The aim of this article is to analyse the available literature and to report the experience of a pulmonary vascular diseases centre about this complication.


BACKGROUND
Scleroderma and its limited variant, the CREST syndrome (calcification, Raynaud phenomenon, esophageal dysmotility, sclerodactily, telangiectasia), is the most common connective tissue disease affected by pulmonary hypertension. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent exam to detect pulmonary hypertension. However, right heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Pulmonary hypertension is less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significantly lower proportion of patients presents an acute vasodilator response, suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. So, we observed dramatic improvement in rare cases after immunosuppressive therapy.


CONCLUSIONS
New treatments with oral, subcutaneous or inhaled stable prostacyclin analogs or with an endothelin receptor antagonist are currently being evaluated. The role of immunosuppressive therapy has to be defined.
Sanchez
O
O
Service de pneumologie et réanimation respiratoire, UPRES EA 2705 Maladies vasculaires pulmonaires, hôpital Antoine-Béclère, 157, rue de la Porte de Trivaux, 92141 Clamart, France.
Humbert
M
M
Sitbon
O
O
Nunes
H
H
Garcia
G
G
Simonneau
G
G
D000959:Antihypertensive Agents / Q000627:therapeutic use*; D003240:Connective Tissue Diseases / Q000150:complications*; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000209:etiology; D004452:Echocardiography; D011464:Epoprostenol / Q000627:therapeutic use*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011379:Prognosis; D016019:Survival Analysis
D000959:Antihypertensive Agents; D007166:Immunosuppressive Agents; D011464:Epoprostenol