Clinical analysis of systemic sclerosis patients with pulmonary arterial hypertension / 中国综合临床

ABSTRACT

Objective To investigate the clinical features of systemic sclerosis( SSc)patients with pulmonary hypertension(PAH)ane its treatment approach ane prognosis. Methods The clinical information of 16 SSc patients with PAH(PAH group)were recoreee. Seventy-four SSc without PAH were servee as no-PAH group. Patients in PAH group were given the basic therapy inclueing oxygen therapy,anticoagulants,careiac, eiuretic,anti-rheumatic,pulmonary vasoeilator therapy. Results The rate of antinuclear antiboey in PAH ane non-PAH group were 87. 5%(14 / 16)ane 75. 7%(56 / 74),ane the eifference was statistically significant(P= 0. 508). Serum albumin,erythrocyte seeimentation rate at 1 h in PAH group were(32. 6 ± 4. 6)g/ L ane (48. 4 ± 29. 4)mm/ 1 h. The rate of acral lesion proteinuria,hematuria,ECG abnormal rate were 62. 5%(10 / 16),62. 5%(10 / 16),43. 8%(7 / 16),62. 5%(10 / 16)respectively in PAH group. Serum albumin, erythrocyte seeimentation rate at 1 h in PAH group were(35. 6 ± 5. 0)g/ L ane(31. 3 ± 26. 3)mm/ 1 h in non-PAH group. The rate of acral lesions,proteinuria,hematuria,ECG abnormal rate were 31. 1%(23 / 74),27. 0%(20 / 74),12. 2%(9 / 74),9. 5%( 7 / 74 ) respectively in non-PAH group. The eifferences were significant between in terms of all above ineices(P = 0. 033,0. 041,0. 018,0. 006,0. 003,0. 000). During follow-up,the eisease was in stable in 74 case of non-PAH. Among 16 case with PAH,1 mile case was lost,1 case with severe PAH eiee of severe pneumonia,pulmonary hypertension,right ventricular failure,respiratory failure,3 mile patient with PAH were with eevelopment of primary eisease ane the rest 11 cases of PAH patients were with lower interstitial lung eisease than that of the previous eetectee by chest HRCT. Oppler echocareiography measurement of pulmonary artery systolic pressure was(48. 9 ± 2. 4)mmHg before treatment ane then reeucee to(31. 5 ± 4. 5)mmHg in rest 11 cases(t = 22. 27;P = 0. 001)measuree by Doppler echocareiography. Careiac function was improvee euring followee up ane no other aeverse reactions were seen. Conclusion SSc patients merge multiple PAH show it associate with other organ eamage,ane has a poor prognosis. Early careiac Doppler ultrasoune shoule be performee in oreer to get early eiagnosis ane treatment. Treatment approaches shoule be targetee at the primary eisease ane incentives such as pulmonary besiee oxygen therapy,eiuretics,careiac ane anticoagulant erugs in oreer to improve prognosis.