Phillips Kristine, Byrne-Dugan Cathryn, Batterson Eric, Seibold James R
University of Michigan, Ann Arbor, MI 48103, USA.
Nat Rev Rheumatol. 2009 Apr;5(4):225-8. doi: 10.1038/nrrheum.2009.30.
A 36-year-old woman developed new-onset Raynaud phenomenon and rapidly progressive dyspnea over a 2-week period. A lung biopsy demonstrated pauci-inflammatory nonspecific pneumonitis, which proved refractory to systemic corticosteroid and intravenous cyclophosphamide therapy. Her preterminal course in an intensive care unit was typified by sequential organ failure. Postmortem examination showed extensive organ fibrosis, including severe diffuse alveolar damage and parenchymal fibrosis, and a notable lack of potentially treatable tissue inflammation.
Chest radiography, physical examination, screening for autoantibodies, measurement of serum creatinine, creatine phosphokinase, and brain natriuretic peptide levels, cardiac examination, pulmonary function tests, electrocardiography, transthoracic Doppler echocardiography, right heart catheterization, high-resolution thoracic CT, pulmonary ventilation/perfusion scan, lung biopsy.
Interstitial lung disease associated with diffuse systemic sclerosis.
Treatment with oxygen, oral and intravenous corticosteroids, mycophenolate mofetil and intravenous cyclophosphamide.
一名36岁女性在2周内出现新发雷诺现象并迅速进展为呼吸困难。肺活检显示少炎性非特异性肺炎，对全身用糖皮质激素和静脉用环磷酰胺治疗无效。她在重症监护病房临终前的病程以序贯性器官衰竭为特征。尸检显示广泛的器官纤维化，包括严重的弥漫性肺泡损伤和实质纤维化，且明显缺乏潜在可治疗的组织炎症。
胸部X线检查、体格检查、自身抗体筛查、血清肌酐、肌酸磷酸激酶和脑钠肽水平测定、心脏检查、肺功能测试、心电图、经胸多普勒超声心动图、右心导管检查、高分辨率胸部CT、肺通气/灌注扫描、肺活检。
与弥漫性系统性硬化症相关的间质性肺疾病。
吸氧、口服和静脉用糖皮质激素、霉酚酸酯和静脉用环磷酰胺治疗。
