engleski

Pulmonary hypertension associated with connective tissue disease

Pulmonary hypertension (PH) significantly impairs outcome and quality of life of patients with connective tissue diseases. Frequency of HP depends upon the type of underlying disease, and varies from 12 % in progressive systemic sclerosis to less than 1% in rheumatoid arthritis. Pathogenesis of PH includes pulmonary arteriopathy, abnormal vasoreactivity and vascular damage, left ventricular diastolic dysfunction resulting in pulmonary venous hypertension, loss of vascular bed as a sequel of concomitant interstitial lung disease and chronic thromboembolic disease associated with hypercoagulability. Early recognition of PH is important due to numerous novel therapeutic opportunities. Patients with connective tissue diseases and respiratory involvement should undergo screening tests that include pulmonary function tests, transthoracic echocardiography (TTE), ventilation and perfusion (V/Q) lung scans, chest X ray and high resolution computed tomography (HRCT) imaging. In addition, in certain circumstances, the right heart catheterization with vasodilatator testing is recommended. During the last decade therapeutic approach to patients with PH has been changed and thus the survival and quality of life have been significantly improved. New insights in the pathogenesis of PH allowed development and usage of several vasodilatator agents with different mechanism of action: calcium channel blockers, prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors. The combination therapy with drugs from more than one class has become an important option proposed for patients with unsatisfactory response to monotherapy. As an example of that synergism we report a patient with mixed connective tissue disease and severe pulmonary hypertension with documented improvement on combined treatment.

Pulmonary hypertension, connective tissue disease

nije evidentirano