皮肌炎或多发性肌炎并发肺间质病变的临床分析

目的分析皮肌炎（DM）或多发性肌炎（PM）并发肺间质病变（ILD）的临床特点,探讨DM或PM发生ILD的危险因素。方法收集住院的77例DM或PM患者的临床资料,回顾性分析ILD的发生率、ILD的特点以及ILD的危险因素。结果77例DM或PM中有ILD48例、发生率62.3%,ILD主要表现为干咳、气喘。单因素Lo-gistic回归分析显示干咳、气喘、发热、关节炎、血沉、血清肌酶比值（AST/CK）与ILD的发病呈正相关（P〈0.05）,多因素Logistic回归分析显示干咳、血沉与ILD的发病呈正相关（P〈0.05）。结论ILD是DM或PM的常见并发症,干咳、气喘、发热、关节炎及高水平的血沉、AST/CK是DM或PM发生ILD的危险因素。     

多发性肌炎/皮肌炎合并间质性肺疾病的相关因素及预后不良因素分析

目的 探讨多发性肌炎/皮肌炎(PM/DM)患者发生间质性肺疾病(ILD)的相关因素及影响预后的不良因素.方法 以上海第二军医大学长海医院1997年1月至2006年11月收住的PM/DM患者87例为研究对象,分为ILD组40例(男13例,女27例),平均年龄(54±13)岁;非ILD组47例(男25例,女22例),平均年龄(45±18)岁.对ILD的发生率、临床特征和预后进行分析.正态分布的计量资料采用t检验,偏态分布的计量资料采用秩和检验,计数资料两组率的比较采用x2检验,PM/DM伴发ILD的预测因素和预后不良因素采用logistic回归分析和Kaplan-Meier生存曲线.结果 PM/DM中ILD的发生率为46％(40/87),病死率为40％(16/40).ILD组的平均年龄[(54±13)岁]明显大于非ILD组[(45±18)岁];ILD组出现发热(21/40)、吞咽困难(16/40)、关节痛(26/40)、Gottron皮疹(14/40)和心脏损害(26/40)的百分率明显高于非ILD组(分别为7/47、8/47、9/47、2/47和14/47);ILD组的血清乳酸脱氢酶[(472±285)IU]和ESR[(44 ±24)mm/1 h]明显高于非ILD)组[(310±238)IU和(26±24)mm/1 h];ILD组的IgG[(18±9)g/L]明显高于非ILD组[(14±5)g/L].经多因素非条件logistic回归分析,筛选出4个与ILD相关的预测因子:Gottron皮疹、关节痛、发热和年龄≥40岁,其相对危险度分别为12.048、7.812、6.329和5.236;生存分析结果显示,Gottron皮疹、心脏损害和肺间质病变是影响ILD预后的不良因素.结论 PM/DM患者年龄≥40岁,出现Gottron皮疹、关节痛和发热与ILD的发生密切相关,Gottron皮疹、心脏损害和肺间质病变是影响ILD预后的不良因素.     

血清KL-6检测在特发性炎性肌病伴肺间质病变诊断中的价值

目的探讨血清KL-6检测在特发性炎性肌病(IIM)合并肺间质病变(ILD)诊断中的作用。方法多发性肌炎(PM)和皮肌炎(DM)患者53例,其中合并有ILD的22例,正常对照者50名及肺部感染患者22例。酶联免疫吸附试验(ELISA)检测血清KL-6浓度。分析PM/DM患者临床表现及预后与血清KL-6水平的相关性。结果血清KL-6浓度在合并ILD的PM/DM组、未合并ILD的PM/DM组、肺部感染组和正常对照组的平均值分别是(1543±761)、(429±106)、(336±196)和(289±105)U/ml。合并ILD的PM/DM组血清KL-6水平较未合并ILD的PM/DM组、肺部感染组和正常对照组均显著升高(P＜0．01)。而未合并ILD的PM/DM组、肺部感染组和正常对照组间差异无统计学意义(P＞0．05)。血清KL-6的升高与肺间质病变呈显著正相关(P＜0．01),KL-6诊断PM/DM合并ILD的敏感性为90．9%,特异性为80．6%。随访分析表明,6例死亡的PM/DM患者血清KL-6水平明显高于其他伴发ILD的PM/DM患者(P＜0．01)。结论血清KL-6浓度是PM/DM合并ILD特异和敏感的血清学指标,它可用来鉴别肺部感染和肺间质病变。高水平的血清KL-6浓度可能提示预后差。     

皮肌炎并发肺间质疾病的临床特点

目的探索皮肌炎(dermatomyositis,DM)患者并发肺部间质病变(interstitial lung disease,ILD)的临床特点和危险因素。方法收集天津医科大学总医院2008年10月至2011年10月DM并行肺部CT患者,比较并发ILD与未并发ILD患者临床资料和实验室指标,行统计学分析。结果共纳入45例DM患者,29例并发ILD,16例为非ILD。DM并发ILD组关节炎和雷诺现象发生率分别为44.8%、20.7%;非ILD组发生率分别为6.3%、0,2组性别差异有统计学意义(P0.05)。两组患者发热、面部皮疹、皮肤红斑性皮疹、Gottron征、吞咽困难中、四肢无力、白细胞、血红蛋白、血小板、红细胞沉降率、肌酸激酶、肌酸激酶同功酶MB、丙氨酸转氨酶、天冬氨酸、乳酸脱氢酶、白蛋白、C反应蛋白、抗核抗体、抗Jo-1抗体比较,差异均无统计学意义(P0.05)。结论 DM并发雷诺现象及关节炎时,ILD发病率高相关。     

婴幼儿肺炎并心力衰竭血清心肌酶谱检测的临床意义

目的 探讨血清心肌酶谱在婴幼儿肺炎并心力心衰中的变化及临床意义.方法 将收治的41例肺炎合并心力衰竭患儿为观察组,以同期住院的32例支气管肺炎患儿为对照组,对两组心肌酶谱进行回顾性分析.结果 观察组心肌酶谱中肌酸激酶(CK)、肌酸激酶同功酶(CK-MB)、乳酸脱氢酶(LDH)、α-羟丁酸酶(α-HBDH)、谷草转氨酶(AST)均明显增高,与对照组比较有统计学显著意义(P<0.05或P<0.01);观察组恢复期AST较急性期显著降低(P<0.05),余心肌酶谱各指标呈下降趋势,但与急性期比较无统计学意义(P>0.05).结论 心肌酶谱可以反映婴幼儿肺炎合并心力衰竭时心肌受损的严重程度.CK-MB增高是判断心肌损害的关键指标,及时检查、积极治疗可以减少并发症的发生,提高抢救成功率,降低死亡率.     

降糖速度与T2DM患者心肌酶学变化的关系

目的 探讨降糖速度对2型糖尿病(T2DM)患者心肌酶学水平变化的影响.方法 选取T2 DM患者202例.将降糖速率≤6mmol·L-1.d-1的患者纳入T2DM1组105例,降糖速率＞6 mmol·L-·d-1的患者纳入T2 DM2组97例.监测并记录两组患者降糖前、降糖后、随访期间3个时间段肌酸激酶同工酶(CK-MB)、肌红蛋白、肌钙蛋白(cTnI)的变化情况.结果 T2DM1组降糖后(CK-MB)、肌红蛋白、cTnI水平较降糖前显著下降(P＜0.05).T2DM1组随访期间心肌酶学水平较降糖前亦明显下降(P＜0.05).T2 DM2组降糖后CK-MB、肌红蛋白、cTnI水平较降糖前上升(P＜0.05).而T2 DM2组随访期间心肌酶学水平较降糖前明显下降(P＜0.05).另外,T2 DM2组降糖前后心肌酶学差值明显高于T2 DM1组(P＜0.05).结论 长期良好的控制血糖可以有效降低心肌酶学水平,但当降糖速度过快时反而会加重心肌细胞损伤,导致心肌酶学水平上升,所以应当合理控制降糖速度.     

多发性肌炎/皮肌炎合并间质性肺疾病的临床分析

目的探讨多发性肌炎/皮肌炎(PM/DM)合并间质性肺疾病(ILD)患者的临床表现、实验室检查、胸部影像学及肺功能的变化及意义。方法对住院的PM/DM并ILD患者7例(男性5例,女性2例,平均年龄55.7岁;5例为DM,2例为PM)均进行血清酶学和自身抗体测定、高分辨率CT(HRCT)及肺功能检查和皮肤肌肉活检。结果 6例患者出现酶学增高,以肌酸激酶(CK)和乳酸脱氢酶(LDH)增高明显;4例患者抗核抗体(ANA)(+),2例抗Jo-1抗体(+);皮肤肌肉活检病理诊断符合肌炎、皮肌炎改变。HRCT提示4例肺出现网点影及斑片阴影,3例出现双肺磨玻璃样病变和实变阴影。7例患者均出现限制性通气功能障碍,一氧化碳弥散量(DLCO)明显降低。6例患者使用糖皮质激素和免疫抑制剂治疗。5例患者治疗后病情稳定,1例患者死亡。结论 HRCT可以及早发现PM/DM肺部病变。CK明显增高和抗Jo-1抗体阳性是诊断DM/PM并ILD的重要指标。患者可出现严重的弥散功能低下,应用激素合并免疫抑制剂治疗可取得较好疗效。     

皮肌炎多发性肌炎患者间质性肺病病理资料推断性分析

目的 分析皮肌炎(DM)、多发件肌炎(PM)合并间质性肺病(ILD)患者的胸部X线、胸部高分辨CT(HRCT)及肺功能等肺部表现临床特点及其预后.方法 回顾性分析上海长海医院风湿免疫科1999年1月至2007年1月期间收住院的33例DM/PM合并ILD除外感染患者的临床资料.结果 33例ILD患者经胸部X线及HRCT证实.根据临床-影像学特点并结合血气分析、肺功能测定,参照美国胸科学会和欧洲呼吸协会问质性肺炎的分类诊断标准,推断分析病理类型主要为非特异性间质性肺炎(NSIP)(57%)和寻常性间质性肺炎(UIP)(25%). UIP类型预后差、病死率高(70%).结论 结合患者的影像学资料、肺功能、血气分析推断病理类型主要为NSIP和UIP,其中UIP病死率高、预后差.     

甲型H1N1流感患者血清心肌酶学的临床研究

目的 探讨甲型H1N1流感患者血清心肌酶学水平与其疾病严重程度的关系.方法 对2009年9月至12月承德市集中收治的206例甲型H1N1流感患者的心肌酶水平进行对比分析.结果 危、重症组血清肌酸激酶(CK)、肌酸激酶同工酶(CK-MB)、门冬氨酸氨基转移酶(AST)、乳酸脱氢酶(LDH)高于轻症组(P＜0.05或P＜0.01),甲型H1N1流感的严重程度与血清CK、CK-MB、AST、LDH均呈正相关(r=0.160、0.396、0.181、0.392,P=0.021、0.001、0.009、0.001).结论 监测血清CK、CK-MB、AST、LDH等酶学水平,对判断甲型H1N1流感患者病情的危重程度及预后具有实际意义.     

血清骨桥蛋白水平与类风湿关节炎活动性的关系及在伴肺间质病变中的意义

目的 检测类风湿关节炎(RA)患者血清骨桥蛋白(OPN)水平,分析血清OPN水平与RA疾病活动的相关性,初步探讨OPN在RA合并肺间质病变(ILD)发病机制中的作用.方法 收集临床确诊的65例RA患者血清.其中活动期RA患者43例,缓解期RA患者22例;其中合并ILD者24例,未合并ILD者41例.以20名健康体检者血清为健康对照组.采用酶联免疫吸附法(ELISA)检测血清OPN水平,并与RA患者的临床及实验室指标进行相关性分析.结果 ①RA组血清OPN水平(中位数18.0 ng/ml),高于健康对照组(中位数14.0 ng/ml,P<0.01);活动期RA组血清OPN水平(中位数20.0 ng/ml),高于缓解期RA组(中位数1.5 ng/ml,P<0.01).②RA组血清OPN水平与病程、关节压痛数、红细胞沉降率(ESR)、C反应蛋白(CRP)等呈显著正相关,与关节肿胀数无相关性.③RA合并ILD组血清OPN水平(中位数20.0 ng/ml),高于RA未合并ILD组(中位数17.0 ng/ml,P<0.05);且与动脉氧分压(PaO2)呈负相关;与肺功能(肺活量、一氧化碳弥散吸收率)无相关性.④RA合并ILD组关节压痛数,关节肿胀数、ESR、CRP等与RA未合并ILD组相比差异具有统计学意义(P均<0.01);RA合并ILD组血清类风湿因子(RF)(IgM)高于RA未合并ILD组(P<0.05).结论 OPN在RA发病机制中具有一定意义且与RA活动性有关,可作为评价疾病活动度的炎性指标;OPN参与了RA合并ILD的发生及进展,并与肺损害严重程度有关.     

Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis

OBJECTIVE: To assess the difference in clinical features and prognosis of patients with interstitial lung disease (ILD) comparing polymyositis (PM) and dermatomyositis (DM). METHODS: Medical records of 28 ILD patients with PM/DM (16 PM-ILD, 12 DM-ILD) were reviewed retrospectively. RESULTS: Serum CPK concentrations were significantly higher in PM-ILD than in DM-ILD. Bronchoalveolar lavage analysis showed that the percentages of lymphocytes and eosinophils were significantly higher in DM-ILD than in PM-ILD. Ten patients (5 PM-ILD, 5 DM-ILD) underwent surgical lung biopsy, and 3 (3 DM-ILD) had an autopsy. Nonspecific interstitial pneumonia (NSIP) was found in 7 (4 PM-ILD, 3 DM-ILD) and usual interstitial pneumonia (UIP) in 3 (1 PM-ILD, 2 DM-ILD). Interestingly, diffuse alveolar damage (DAD) was found in 3 patients with DM-ILD, who all died of deterioration of ILD; but no one with PM-ILD had DAD. Corticosteroid treatment alone achieved a favorable response in 6 patients (37.5%) with PM-ILD, but in only one (8.3%) with DM-ILD. Administration of cyclosporine in the early phase of onset benefited 4 corticosteroid-resistant patients with DM-ILD. Conclusively, survival in DM-ILD was significantly worse than that in PM-ILD. CONCLUSION: DM-ILD is more refractory to corticosteroid therapy, resulting in poorer prognosis compared with PM-ILD. These data indicate that intensive therapy, including cyclosporine, should be considered for DM-ILD.     

Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study

The aim of the study was to investigate the characteristics of adult clinically amyopathic dermatomyositis (CADM) with rapid progressive interstitial lung disease (ILD). Hospitalized patients with dermatomyositis (DM) and polymyositis (PM) between 1998 and 2005 in the Shanghai Renji Hospital were retrospectively studied. One hundred and forty-five patients were classified into CADM, classic DM or PM according to the modified Sontheimer's definition or Bohan-Peter's classification criteria. They were further stratified based on the presence or absence of clinical ILD. The Kaplan-Meier survival analysis and COX regression were performed. The predictive factors for ILD and other clinical properties of CADM-ILD were explored. The presence of clinical ILD was a significant risk factor for the poor outcome of DM/PM (OR = 4.237, CI 95%: 1.239-14.49, p = 0.021). Other risk factors are the presence of rashes and elevated urea nitrogen. Patients with DM/PM complicated by ILD had different clinical courses. Patients with CADM-ILD showed a rapidly progressive pattern with 6-month survival rate of 40.8%. The DM-ILD manifested a progressive pattern with a 5-year survival rate of 54%, while PM-ILD was chronic with 5- and 10-year survival rate of 72.4% and 60.3%, respectively. Better preserved muscle strength, elevated erythrocyte sedimentation rate, and hypoalbuminemia may herald ILD in DM/PM. Patients with CADM-ILD who later died had lower PO(2), higher lactate dehydrogenase, and prominent arthritis/arthralgia compared with those who survived. The presence of antinuclear antibody seems to be protective. Rapid progressive CADM-ILD is refractory to conventional treatment. ILD is a common complication in over 40% of our hospitalized DM/PM cohort and is also a prominent prognostic indicator. CADM is a special phenotype of DM/PM. CADM-ILD, which is usually rapidly progressive and fatal, requires further investigation.     

Risk factors for the recurrence of interstitial lung disease in patients with polymyositis and dermatomyositis: a retrospective cohort study

Clinical Rheumatology - To identify risk factors for the recurrence of interstitial lung disease (ILD) in patients with polymyositis (PM)/dermatomyositis (DM). Forty-four PM/DM-ILD patients who had...     

多发性肌炎和皮肌炎分期分型与实验室诊断指标相关性分析

目的探讨多发性肌炎(PM)和皮肌炎(DM)患者临床分期与实验室诊断指标的关系。方法观察2003-01~2005-01河北医科大学附属第二医院收治的58例PM和DM患者,82·9%活动期患者肌酸激酶(CK)、肌酸激酶同工酶(CK-MB)、天冬氨酸氨基转移酶(AST)、乳酸脱氢酶(LDH)、羟丁酸脱氢酶(HBDH)较稳定期明显增高,尤其以CK变化最明显;活动期自发电位和多相波的发生率(75·9%、48·3%)明显高于稳定期(46·7%、20·0%);PM大多有肌纤维萎缩和淋巴细胞为主的炎症细胞浸润,DM表现为肌束周围不同程度的萎缩。结果按病情的控制情况分期;同时进行血清酶、肌电图和病理检查。结论CK可以作为监测疾病活动性的一个指标;活动期患者肌电图自发电位和多相波的出现量大;PM的病理表现为肌纤维膜有炎细胞浸润,且有特异性的退行性表现;DM特征性的病理表现为肌束周围萎缩和微小血管改变。     

Interstitial lung disease in polymyositis and dermatomyositis

OBJECTIVES: To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM. METHODS: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. RESULTS: Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent. CONCLUSION: Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.     

Short‐term and long‐term outcomes of interstitial lung disease in polymyositis and dermatomyositis: A series of 107 patients

Objective

This study was undertaken to assess the characteristics and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/DM) and to determine variables predictive of ILD deterioration in PM/DM.

Methods

Among 348 consecutive patients with PM/DM, 107 patients with ILD were identified by medical records search in 4 medical centers. All patients underwent pulmonary function tests (PFTs) and pulmonary high‐resolution computed tomography (HRCT) scan.

Results

ILD onset preceded PM/DM clinical manifestations in 20 patients, was identified concurrently with PM/DM in 69 patients, and occurred after PM/DM onset in 18 patients. Patients with ILD could be divided into 3 groups according to their presenting lung manifestations: patients with acute lung disease (n = 20), patients with progressive‐course lung signs (n = 55), and asymptomatic patients with abnormalities consistent with ILD evident on PFTs and HRCT scan (n = 32). We observed that 32.7% of the patients had resolution of pulmonary disorders, whereas 15.9% experienced ILD deterioration. Factors that predicted a poor ILD prognosis were older age, symptomatic ILD, lower values of vital capacity and diffusing capacity for carbon monoxide, a pattern of usual interstitial pneumonia on HRCT scan and lung biopsy, and steroid‐refractory ILD. The mortality rate was higher in patients with ILD deterioration than in those without ILD deterioration (47.1% versus 3.3%).

Conclusion

Our findings indicate that ILD results in high morbidity in PM/DM. Our findings also suggest that more aggressive therapy may be required in PM/DM patients presenting with factors predictive of poor ILD outcome.

     

Interstitial lung disease in polymyositis and dermatomyositis: clinical course and response to treatment

OBJECTIVES: To assess the prevalence, clinical characteristics, and treatment options of patients with interstitial lung disease (ILD) in polymyositis and dermatomyositis (PM/DM). Patients and Methods: Sixty-three consecutive patients with PM/DM underwent standardized screening. Patients with ILD were monitored prospectively, and graded immunosuppression was administered according to the rate of clinical progression. RESULTS: ILD was diagnosed in 20 of 63 patients (32%). Generally, the clinical and serologic findings of the anti-Jo1 syndrome were present. Follow-up evaluation disclosed either a progressive or a nonprogressive course. The 10 patients with progressive ILD were distinguished from the nonprogressive group by extensive ground-glass opacities on high-resolution computed tomography (HRCT) and by bronchoalveolar lavage (BAL) neutrophilia. Intravenous pulse cyclophosphamide prevented further progression in all 10 patients and led to some functional improvement. In the 10 patients without rapidly progressive lung disease, immunosuppression of moderate intensity stabilized pulmonary findings during a median 35 months of follow-up. CONCLUSIONS: The prevalence of ILD in our patients with PM/DM was 32%; this emphasizes the need for pulmonary screening in all PM/DM patients. Progressive disease, featuring ground-glass opacities on HRCT and an inflammatory BAL cell profile, is amenable to intensive immunosuppression. Conversely, patients who do not have these HRCT and BAL features appear to have a low risk of pulmonary deterioration. RELEVANCE: Because the treatment for ILD seems to depend on the rate of clinical progression, future therapeutic trials of lung disease in PM/DM should stratify patients accordingly.     

Prevalence of interstitial lung disease in polymyositis and dermatomyositis: A meta-analysis from 2000 to 2020

ObjectiveInterstitial lung disease (ILD) is the most important prognostic factor for mortality in patients with polymyositis (PM) and dermatomyositis (DM), but the prevalence of ILD in PM/DM may vary between countries. The aim of this study was to determine the overall prevalence of ILD in global patients with PM/DM.MethodsWe performed a systematic literature review of studies published from Jan 1, 2000 to April 30, 2020 on ILD and PM/DM. We extracted data and pooled the prevalence by using a random-effect model due to high heterogeneity. Heterogeneity was assessed by subgroup analysis and sensitivity analysis.ResultsA total of 34 studies with 10,130 patients were included in our meta-analysis. Pooled data demonstrated that the global prevalence of ILD in patients with PM/DM was 0.41 (95% confidence interval [CI] 0.35–0.48). However, this prevalence varied with geographical locations and time trends. The prevalence of ILD in PM/DM was 0.5 (95% CI 0.42–0.57) in Asia, 0.23 (95% CI 0.15–0.31) in America, and 0.26 (95% CI 0.18–0.34) in Europe. A higher prevalence of ILD was reported in studies published in 2011–2015 (0.43, 95% CI 0.34–0.52) and 2016–2020 (0.45, 95% CI 0.35–0.54), compared with those published in 2000–2010 (0.27, 95% CI 0.16–0.39). The pooled prevalence of ILD in patients with DM, PM, and clinically amyopathic dermatomyositis subtype was 0.42 (95% CI 0.35–0.49), 0.35 (95% CI 0.27–0.42), and 0.53 (95% CI 0.32–0.74), respectively. Patients with anti-Jo-1 and anti-melanoma differentiation-associated gene 5 antibodies were more likely to develop ILD than other myositis-specific autoantibodies.ConclusionThe global prevalence of ILD in patients with PM/DM was approximately 41% and the condition was predominant in Asians. This highlights potential genetic and environmental differences in the pathogenesis of ILD in patients with PM/DM. More studies are required to elucidate the specific associations.     

Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis

OBJECTIVE: To assess the prevalence, characteristics and prognostic factors of interstitial lung disease (ILD) in Korean patients with polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM). METHODS: We reviewed the medical records of 72 consecutive PM and DM patients, including six patients with ADM, who were seen at the Rheumatology Clinic of Seoul National University Hospital between 1984 and 2003. RESULTS: Twenty-nine PM/DM patients (40.3%) developed ILD. Anti-Jo-1 antibody and arthralgia were associated with the presence of ILD (P = 0.022 and P = 0.041, respectively), whereas dysphagia was more frequently found in patients without ILD (P = 0.041). Lung biopsies revealed diffuse alveolar damage (DAD) (n = 2), usual interstitial pneumonia (UIP) with DAD (n = 2), UIP (n = 1), and non-specific interstitial pneumonia (n = 2). Of the 29 patients, 11 (37.9%) died. The mean survival time in ILD patients was significantly shorter than in those without ILD (13.8+/-1.8 vs 19.2+/-0.9 yr, P = 0.017). Poor survival in ILD patients was associated with a Hamman-Rich-like presentation (P = 0.0000), ADM features (P = 0.0001) and an initial forced vital capacity (FVC) < or =60% (P = 0.024). CONCLUSIONS: ILD was observed in 40.3% of Korean PM/DM patients and was associated with poor survival. A Hamman-Rich-like presentation, ADM features and an initial FVC < or =60% were associated with poor survival in ILD.     

High-resolution computed tomography characterization of interstitial lung diseases in polymyositis/dermatomyositis

OBJECTIVE: Interstitial lung disease (ILD) associated with polymyositis (PM) and dermatomyositis (DM) sometimes progresses rapidly and is resistant to therapy. Clinical features that forecast the prognosis of the disease remain to be elucidated. Our aim was to assess if selected clinical features and high-resolution computed tomography (HRCT) findings can assist in predicting the clinical course of ILD in PM/DM. METHODS: We examined HRCT findings retrospectively for ILD identified in 17 patients with PM and 16 with DM. Radiological patterns and clinical features are analyzed in comparison with clinical course. RESULTS: Mortality rates were 12% and 44% for ILD associated with PM and DM, respectively. Most patients with DM died of rapidly progressive lung deterioration. No patient in the PM group died of respiratory failure. In the DM group, all patients with fatal ILD had ground-glass attenuation and reticular opacity as the principal radiological findings. Consolidation was recognized frequently as the principal pattern in nonfatal cases. Radiological patterns were categorized into 3 groups; A: consolidation dominant, B: ground-glass attenuation/reticular opacity dominant without chronic fibrosing process, and C: ground-glass attenuation/reticular opacity dominant with chronic fibrosing process. Occurrences of fatal disease were 0%, 83%, and 20%, in groups A, B, and C. CONCLUSION: The prognosis of ILD associated with DM differs from that with PM. The former can be classified into 3 subgroups on the basis of radiological findings, which are closely associated with clinical course.