混合结缔组织病与肺动脉高压(附1例尸检报告)

MIXED CONNECTIVE TISSUE DISEASE AND PULMONARY HYPERTENSION——A CASE OF AUTOPSY

1例混合结缔组织病伴肺动脉高压患者,死于右心衰竭及肺部感染。患者具有顽固的雷诺现象、抗RNP抗体阳性、抗Sm抗体阴性及肺动脉高压,继发右心衰竭。尸检发现:肺细小动脉壁普遍增厚,内膜及平滑肌增生,管腔狭窄等。认为肺血管的病变及雷诺现象可能与免疫异常有关。

Clinical and Autopsy findings of 1 case with Mixed Connective Tissue Disease (MCTD)associated with severe pulmonary hypertension,died of right heart failure and pulmonary infection,was reported. The patient suffered from continuous Raynaud phenomenon,positive anti-RNP antibody and pulmonary hypertension inducing right heart failure. Autopsy showed stenosis and marked wall thickening in small and middle sized pulmonary arteries, due to proliferation of tunical intima and smooth muscle. The pulmonary vasculitis and Raynaud phenomenon might be related to immunologic abnormalities.