无肌病皮肌炎相关机化性肺炎临床特点分析
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摘要
目的分析无肌病皮肌炎相关机化性肺炎(ADM-OP)患者临床特点和治疗反应。方法回顾2014年6月至2018年6月于我院住院的8例ADM-OP临床资料,并与同期收治的9例隐源性机化性肺炎(COP)进行比较。结果 ADM-OP出现皮肤改变、Gottron征、技工手及抗合成酶抗体阳性分别为87.5%、75.0%、87.5%及62.5%。与COP相比,ADM-OP在性别、吸烟、呼吸系统症状体征、动脉血氧分压、动脉血二氧化碳分压及治疗方案无差异,发病年龄和入院胸部CT纤维化评分存在差异。治疗3个月胸部CT纤维化评分和变化率、用力肺活量存在差异,治疗6个月胸部CT纤维化评分和变化率、用力肺活量、肺一氧化碳弥散量存在差异。结论ADM-OP患者以皮肤改变、Gottron征、技工手及抗合成酶抗体阳性表现多见,对治疗反应好,影像和肺功能改善速度较COP患者缓慢。
Objective To analyze the clinical characteristics of patients with amyopathic dermatomyositis with organizing pneumonia(ADM-OP). Methods The clinical data of 8 patients hospitalized with ADM-OP from June 2014 to June 2018 were retrospectively reviewed and simultaneously compared with those of 8 patients of cryptogenic organizing pneumonia(COP). Results The incidence of skin lesion, Gottron's sign, mechanic's hand and positive anti-synthase antibodies in the ADM-OP patients were 87.5%, 87.5% 75.0% and 87.5% respectively. Gender, smoking,respiratory symptoms and signs, arterial partial pressure of oxygen, arterial partial pressure of carbon dioxide and treatment strategy were no statistical difference between ADM-OP and COP patients, but the onset age and Chest CT fibrosis scores(CTFS) on admission existed differences. After treatment for 3 months, CTFS, rate of change and forced vital capacity(FVC) existed differences. After treatment for 6 months, CTFS, rate of change, FVC and diffusing capacity of the lung for carbon monoxide existed differences. Conclusions Skin lesion, Gottron's sign, mechanic's hand and positive anti-synthase antibodies are more common in ADM-OP patients. Their response to treatment is good but the improvement rates in CTFS and pulmonary function are slower than those of COP patients.
Objective To analyze the clinical characteristics of patients with amyopathic dermatomyositis with organizing pneumonia(ADM-OP). Methods The clinical data of 8 patients hospitalized with ADM-OP from June 2014 to June 2018 were retrospectively reviewed and simultaneously compared with those of 8 patients of cryptogenic organizing pneumonia(COP). Results The incidence of skin lesion, Gottron's sign, mechanic's hand and positive anti-synthase antibodies in the ADM-OP patients were 87.5%, 87.5% 75.0% and 87.5% respectively. Gender, smoking,respiratory symptoms and signs, arterial partial pressure of oxygen, arterial partial pressure of carbon dioxide and treatment strategy were no statistical difference between ADM-OP and COP patients, but the onset age and Chest CT fibrosis scores(CTFS) on admission existed differences. After treatment for 3 months, CTFS, rate of change and forced vital capacity(FVC) existed differences. After treatment for 6 months, CTFS, rate of change, FVC and diffusing capacity of the lung for carbon monoxide existed differences. Conclusions Skin lesion, Gottron's sign, mechanic's hand and positive anti-synthase antibodies are more common in ADM-OP patients. Their response to treatment is good but the improvement rates in CTFS and pulmonary function are slower than those of COP patients.
引文
1 Sontheimer RD. Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis. Curr Opin Rheumatol, 1999, 11(6):475-482.
2韩磊,吕良敬.无肌病性皮肌炎的研究进展.中华风湿病学杂志,2011,15(11):795-797.
3 Sun Y, Liu Y, Yan B, et al. Interstitial lung disease in clinically amyopathic dermatomyositis(CADM)patients:a retrospective study of 41 Chinese Han patients. Rheumatol Int, 2013, 33(5):1295-1302.
4 Li XR, Peng SC, Wei LQ. Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. Int J Clin Exp Pathol, 2015, 8(9):11230-11235.
5中华医学会风湿病学分会.多发性肌炎和皮肌炎诊断及治疗指南.中华风湿病学杂志,2010, 14(12):828-831.
6 Bradley B, Branley HM, Egan JJ, et al. Interstitial Lung Disease Guideline:the British Thoracic Society in Collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax, 2008,63(Suppl 5):vl-v58.
7 Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society Statement:Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med,2013,188(6):733-748.
8 Lynch DA, Godwin JD, Safrin S, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis:diagnosis and prognosis. Am J Respir Crit Care Med, 2005, 172(4):488-493.
9徐凌,沈策.机化性肺炎的研究进展.国际呼吸杂志,2007, 27(24):1903-1906.
10董馨,郑毅,王丽,等.结缔组织病相关性机化性肺炎的临床特点.中华风湿病学杂志,2015, 19(6):374-379.
11沈敏,龚瑜林,曾小峰,等.以间质性肺疾病起病的皮肌炎临床特点分析.中华医学杂志,2014, 94(43):3402-3406.
12 Hayashi S, Tanaka M, Kobayashi H, et al. High-resolution computed tomography characterization of interstitial lung diseases in polymyositis/dermatomyositis. J Rheumatol, 2008, 35(2):260-269.
13 Tsuchiya Y, Fischer A, Solomon JJ, et al. Connective tissue diseaserelated thoracic disease. Clin Chest Med, 2015, 36(2):283-297.
2韩磊,吕良敬.无肌病性皮肌炎的研究进展.中华风湿病学杂志,2011,15(11):795-797.
3 Sun Y, Liu Y, Yan B, et al. Interstitial lung disease in clinically amyopathic dermatomyositis(CADM)patients:a retrospective study of 41 Chinese Han patients. Rheumatol Int, 2013, 33(5):1295-1302.
4 Li XR, Peng SC, Wei LQ. Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. Int J Clin Exp Pathol, 2015, 8(9):11230-11235.
5中华医学会风湿病学分会.多发性肌炎和皮肌炎诊断及治疗指南.中华风湿病学杂志,2010, 14(12):828-831.
6 Bradley B, Branley HM, Egan JJ, et al. Interstitial Lung Disease Guideline:the British Thoracic Society in Collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax, 2008,63(Suppl 5):vl-v58.
7 Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society Statement:Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med,2013,188(6):733-748.
8 Lynch DA, Godwin JD, Safrin S, et al. High-resolution computed tomography in idiopathic pulmonary fibrosis:diagnosis and prognosis. Am J Respir Crit Care Med, 2005, 172(4):488-493.
9徐凌,沈策.机化性肺炎的研究进展.国际呼吸杂志,2007, 27(24):1903-1906.
10董馨,郑毅,王丽,等.结缔组织病相关性机化性肺炎的临床特点.中华风湿病学杂志,2015, 19(6):374-379.
11沈敏,龚瑜林,曾小峰,等.以间质性肺疾病起病的皮肌炎临床特点分析.中华医学杂志,2014, 94(43):3402-3406.
12 Hayashi S, Tanaka M, Kobayashi H, et al. High-resolution computed tomography characterization of interstitial lung diseases in polymyositis/dermatomyositis. J Rheumatol, 2008, 35(2):260-269.
13 Tsuchiya Y, Fischer A, Solomon JJ, et al. Connective tissue diseaserelated thoracic disease. Clin Chest Med, 2015, 36(2):283-297.