Rapidly Progressive Respiratory Failure in Mixed Connective Tissue Disease: Report of an Autopsy Case
Rapidly Progressive Respiratory Failure in Mixed Connective Tissue Disease: Report of an Autopsy Case
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DOI：
10.2169/internalmedicine.51.8728
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发表时间：
2012-01-01
影响因子：
1.2
通讯作者：
Kawabata, Yoshinori
中科院分区：
文献类型：
--
作者：
Watanabe, Yasutaka;Koyama, Shinichiro;Kawabata, Yoshinori
一位64岁的女性出现劳力性呼吸困难。由于存在手指肿胀、雷诺现象、肌无力、抗U1 RNP抗体阳性、心包炎和间质性肺炎，该病例被诊断为混合性结缔组织病（MCTD）。尽管经支气管肺活检（TBLB）的组织学显示机化性肺炎，但应患者要求，皮质类固醇治疗推迟了两个月。尽管给予静脉内环磷酰胺联合泼尼松龙治疗，但患者8周后死于急性进行性间质性肺炎。尸检显示渗出性和机化性弥漫性肺泡损伤（DAD）。先前的报告显示DAD是MCTD中极为罕见的肺部并发症。本报告提出一个病例MCTD急性呼吸衰竭。因此，该病例提示应尽快给予这种治疗。
A 64-year-old woman presented with exertional dyspnea. The case was diagnosed as mixed connective tissue disease (MCTD) due to presence of swollen fingers, Raynaud's phenomenon, muscle weakness, positive anti-U1RNP antibody, pericarditis and interstitial pneumonia. Although the histology from a transbronchial lung biopsy (TBLB) indicated organizing pneumonia, corticosteroid therapy was postponed for two months at the patient's request. She died 8 weeks later from acute progressive interstitial pneumonia in spite of the administration of intravenous cyclophosphamide combined with prednisolone. The autopsy revealed exudative and organizing diffuse alveolar damage (DAD). Previous reports have shown that DAD is an extremely rare pulmonary complication in MCTD. This report presents a case of MCTD with acute respiratory failure. This case thus suggests that this therapy should be administered as soon as possible.