[Pulmonary artery hypertension associated with connective tissue diseases].
MOST IMPORTANT: Pulmonary hypertension (PH) is a severe, potentially life-threatening complication of connective tissue diseases, among which scleroderma is first line. The aim of this paper was to review the literature and report our experience with this particular complication of connective tissue diseases. In our centre of pulmonary vascular diseases, connective tissue diseases represent the third cause of PH.


RESULTS
Scleroderma and particularly its limited cutaneous variant, the CREST syndrome, is the most common connective tissue disease affected by pulmonary hypertension. It can be related to a specific lung parenchymal involvement (hypoxic PH), to an isolated pulmonary vascular involvement or to a cardiac dysfunction secondary to specific myocardial lesions.


METHODS
Echocardiography is an excellent examination to detect pulmonary hypertension. However, right heart catheterisation is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide (NO).
Oral calcium channel blockers are indicated in patients who are responders to acute NO tests. Treatment with continuous intravenous prostacylin is obviously an improvement, at least functionally, although it appears less effective than in primary PH. With the new subcutaneous, oral and inhaled vasodilatators (prostaglandin and endothelin receptor antagonists), a few cases of improvement of PH with intensive immunosuppressive therapy were observed, essentially during systemic lupus erythematosus and Sharp syndrome.


CONCLUSIONS
PH is a severe complication of connective tissue diseases. Early detection of this complication should allow an earlier and more aggressive therapeutic approach in these patients, before irreversible vascular lesions occur.
Sanchez
O
O
Centre des maladies vasculaires pulmonaires (UPRES EA 2705), Service de pneumologie et réanimation respiratoire, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92140 Clamart.
Sitbon
O
O
Garcia
G
G
Jaïs
X
X
Simonneau
G
G
Humbert
M
M
D000328:Adult; D017675:CREST Syndrome / Q000150:complications; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D006328:Cardiac Catheterization; D015984:Causality; D003240:Connective Tissue Diseases / Q000150:complications*; D004452:Echocardiography; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D009569:Nitric Oxide; D011652:Pulmonary Circulation; D012595:Scleroderma, Systemic / Q000150:complications; D013997:Time Factors; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D009569:Nitric Oxide; D011464:Epoprostenol