Pulmonary hypertension systemic lupus erythematodes

Authors: D. Tegzová
Authors‘ workplace: Revmatologický ústav, Praha
Published in: Čes. Revmatol., , 2003, No. 2, p. 92-98.


Pulmonary hypertension (PH) represents rare and serious complication of various systemic connectivetissue diseases. It occurs most frequently in scleroderma, mixed connective tissue disease,systemic lupus erythematosus and in antiphospholipid syndrome. PH in systemic diseases is newlyclassified as the arterial PH. Its development is potentiated by several epigenetic mechanismsincluding vasculitis, vasculopathy, thromboembolia, vascular endothelial impairment, and by theimbalance between vasoconstriction and vasodilatation mechanisms. Suspection of PH can beestablished on the basis of echocardiography and the diagnosis is verified by right-side catheterisation.The degree of reversibility of vasoconstriction of pulmonary vessels and therefore the mostsuitable therapy can be estimated using vasodilatation test. PH treatment has recently undergonea rapid development. Formerly, calcium channel blockers were used and since the end of eightiespatients have been treated with prostacycline and epoprostenole respectively, administered incontinuous intravenous infusion. In recent few years some other drugs were tested, namely theepoprostenole derivatives. These drugs need not be administered only intravenously; they can begiven per inhalation (iloprost), subcutaneously (treprostinol) or perorally (beraprost). Another newdrug is the phosphodiesterase inhibitor (sildenafil), endothelial receptor blocker (bosentan), nitrogenoxide or L-arginine and also therapy using adenosine and serine is being tested. Some combinationsof drugs are also examined. The above given possibilities of treatment, however, are notroutinly available, they can be tested only within the framework of therapeutical research studies.PH treatment is life lasting and comparatively costly. The therapy of the systemic connective tissuedisease has no effects on the already developed PH, but it is necessary for stabilization of the primarydisease.

Key words:
pulmonary hypertension, systemic lupus erythematosus, antiphospholipid antibodies,heart failure, vasodilatation therapy

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Dermatology & STDs Paediatric rheumatology Rheumatology
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