[2009] [THU0293] CLINICAL PECULIARITY OF PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS
A. Volkov1, E. Nasonov2 1Ultrasoud diagnostic; 2Director, Institute of Rheumatology, RAMS, Moscow, Russian Federation
Background: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and visceral organs, in which the lung is frequently (70-90% of patients) and severely involved. Pulmonary hypertension (PH) affects about 13% of patients with SSc and is one of the most important complications adversely influencing their survival.
Objectives: Our objectives were to evaluate the clinical peculiarity of systemic sclerosis in patients who developed pulmonary arterial hypertension (PAH) and clinical heterogeneity PH, associated with systemic sclerosis.
Methods: 31 SSc patients with PH and 83 without PH were enrolled in the study. PH was diagnosed by Doppler echocardiography with Vivid 7 ultrasound system, in several cases was confirmed by right heart catheterization. All the patients were submitted to chest radiographs and high-resolution CT (HRCT), underwent pulmonary function testing and 6-MWT, and the presence of autoantibodies was determined. More detailed investigation of internal organ involvement was directed by clinical symptoms and signs, as part of normal clinical practice.
Results: The age SSc-PH patients and duration of the diseases were higher then SSc patients without PH (54 y and 144 months vs 43 y and 114 months, p < 0,01). The SSc patients who detected PH were more frequently presented limited skin involvement (80%) but skin score were not differ significantly. Sjögren syndrome were more frequent in patients with PH, however severity of Raynaud's phenomenon were identical expressed. Internal organ involvement in SSc-PH patients were detected earlier (59,6 vs 69,4 months, p< 0,05), accordingly Systemic Sclerosis Damage Index was higher (6,5±1.8 vs 5,3±2.0, p = 0,004). Anticentromere antibody also more frequent in SSc-PH patients. Right ventricular systolic pressure were higher in SSc-PH patients undoubtedly (55,15±23,69 mm.Hg) and correlated with age (r = 0,3 p = 0,001), DLCO (r = -0,23 p = 0,046), uric acid level (r = 0,24 p = 0,03), Systemic Sclerosis Damage Index (r = 0,25 p = 0,017), 6-MWT (r = - 0,33 p <0,001), SpO2 after exercise (r = - 0, 32 p = 0,0017).
After PH detection, clinical class identification and evaluation patients have been divided into 4 groups accepted in classification, depending on the received results.
Pulmonary arterial hypertension has been confirmed more than at half: at 15 - isolated, at 4 - in a combination with interstitial lung disease and at 1 patient in a combination with primary biliary cirrhosis. In other 11 cases PH were associated with hypoxemia (3 pts), Left Heart Disease (7 pts) and in one case patients had chronic post thromboembolic PH.
Conclusion: The results confirm that there are clinical differences between SSc and SSc-PH patients. The presence of pulmonary hypertension demands more careful analysis of the clinical picture and complex therapy.
Disclosure of Interest: The authors declare that they have no competing interests.
Ann Rheum Dis 2009;68(Suppl3):269
Scleroderma, myositis and related syndromes
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