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关于原发性肺动脉高压伴雷诺氏现象的病例,国内未见报告。现报告临床及病理所见1例。 中年女性,因双指(趾)发凉、麻木、胀痛伴面部发紧、咀嚼费力6年,心悸、气短、下肢浮肿2年,于1982年8月22日入院。入院时面色苍白,皮肤较硬,指(趾)呈青紫色,触之发凉。两肺呼吸音清晰,心界轻度扩大,胸骨左缘Ⅲ～Ⅳ肋间及心尖部闻及Ⅱ级收缩期回流性杂音,肺动脉第二音增强并分裂。肝大,下肢浮肿。血沉快,抗核抗体及抗DNA抗体滴度明显增高,肾功正常。心电图示右室肥厚,超声心动图、胸部X线检查及右室造影均符合肺心病及肺动脉高压表现。右心导管检查右室压力96.2/3.9(35.1)mmHg。住院期间患者有不规则发热及难以忍受的关节酸痛,曾用酚(艹卡)明、哌唑嗪、强的松及
On the primary pulmonary hypertension with Raynaud’s phenomenon, no domestic report. Now report the clinical and pathological findings in 1 case. Middle-aged women, two fingers (toes) due to cold, numbness, pain and tenderness with facial hair tight, chewing laborious 6 years, palpitations, shortness of breath, lower extremity edema for 2 years, in 1982 August 22 admission. Pale at admission, hard skin, finger (toe) was purple, touching the cold. Breath sounds clear two lungs, heart slightly expanded, the left sternal border Ⅲ ~ Ⅳ intercostal and apical smell and Ⅱ grade systolic reflux murmur, pulmonary artery second enhanced and split. Liver, lower extremity edema. Rapid ESR, anti-nuclear antibodies and anti-DNA antibody titers increased significantly, normal renal function. Right ventricular hypertrophy, echocardiography, chest X-ray and right ventriculography were consistent with pulmonary heart disease and pulmonary hypertension. Right ventricular catheterization right ventricular pressure 96.2 / 3.9 (35.1) mmHg. Patients during hospitalization have irregular fever and unbearable joint pain, have been used phenols (carbuncle) Ming, prazosin, prednisone and