多肌炎的电生理研究
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摘要
目的
分析多肌炎(polymyositis, PM)的基本临床特征及其电生理特点。
资料和方法
1.统计描述2006年1月1日-2010年4月1日在我院住院的符合PM临床诊断的93例患者的临床概况。
2.统计描述93例PM患者的电生理特点,采用卡方检验及t检验对比分析不同肌肉肌电图特点及相关指标出现的阳性率。并分析病程对肌电图结果的影响。
3.总结分析PM伴发间质性肺病患者临床资料、肌电图结果,采用卡方检验及t检验分析其与不伴发间质性肺病的PM患者间差异性。
4.采用卡方检验及t检验,分析叠加结缔组织病的PM患者与单纯型PM患者间,及接受过激素治疗的PM患者与未接受过激素治疗的PM患者的肌酶谱、肌电图结果差异性。
5.描述性分析电生理检查结果为非单纯肌源性损害患者的一般临床资料及电生理结果特点。
结果
1.一般资料93例PM患者,男性24例(25.81%),女性69例(74.19%),男：女=1：2.9,平均年龄43.19±15.64岁,总体病程0.3-228月(19.16±33.12月)。伴发间质性肺病患者18例(19.35%),叠加结缔组织病17例(18.28%),其中类风湿性关节炎最多5例(29.41%)比例最高。伴发肿瘤患者1例(1.08%),为非何杰金淋巴瘤。36例患者(38.71%)在肌电图检查前接受过激素治疗。肌酸激酶升高者83.87%,丙氨酸氨基转移酶升高者88.06%,天冬氨酸氨基转移酶升高者85.07%,乳酸脱氢酶升高者97.33%,血沉增快者65%。93例患者共40例(40.01%)行肌肉活检,其中32例(80%)为典型多肌炎表现,7例(17.5%)肌活检结果为非特异性肌纤维坏变,1例(2.5%)肌活检结果正常。
2.本组PM患者常规肌电图检查,包括上、下肢远、近端四块肌肉(肱二头肌、拇短展肌、股四头肌、胫前肌)：插入电位异常出现率为15.88%；自发电位中纤颤电位出现率68.80%；正锐波出现率66.85%；CRDs出现率为6.45%；MUP平均时限缩短超过20%出现率为79.94%；多相波超过20%出现率为14.48%；肌病干扰相出现率为9.74%；MUP平均时限缩短出现异常率在PM患者中相对较高(P=0.000)。MUP平均波幅：肱二头肌：400.39±76.12 umm；拇短展肌452.1±88.4 um；股四头肌：447.39±77.1 um；胫前肌：468.03±214.09 um。应用person卡方分析上、下肢近端肌,上、下肢远端肌,上肢远、近端肌,下肢远、近端肌肌电图结果出现异常率的差异性：肱二头肌与股四头肌、股四头肌与胫前肌各肌电图结果出现异常率,在统计学上无显著差异(P>0.05)。胫前肌(插入电位延长、纤颤正相电位、MUP平均时限缩短、多相波增多、肌病干扰相)出现异常率较拇短展肌明显高(P=0.04、0.001、0.000、0.045、0.018)。肱二头肌(插入电位延长、纤颤正相电位、MUP平均时限缩短、肌病干扰相)出现异常率较拇短展肌明显增高(P=0.003、0.008、0.000、0.002)。
3.根据病程将患者分为两组：A组：病程小于等于3月,B组：病程大于3月,B组患者ALT值相对A组明显增高(P=0.03)。2组肌电图结果B组肱二头肌MUP平均波幅(399.73±81.71)较A组(403.80±36,59)显著低(P=0.025),余结果出现异常率在统计学上无显著差异性(P>0.05)。
4.根据是否伴发间质性肺病将患者分为两组：不伴间质性肺病患者为A组：74例,平均病程21.77±4.21月,平均年龄49.16±15.10岁；伴间质性肺病患者为B组：19例,平均病程9.02±2.85月,平均年龄41.67±15.51岁。A组病程较B组长(P=0.026)；两组年龄分布无显著差异(P=0.495)；A组CK及LDH值明显高于B组,A组肱二头肌及股四头肌测定结果异常率(异常自发电位、MUP平均时限缩短、肌病干扰相)均明显高于B组(P=0.05、0.00、0.05,且胫前肌MUP平均时限缩短出现异常率高于B组(P=0.02)。A组胫前肌MUP平均波幅(440.8±96.59)较B组(573.79±426.13)显著低(P=0.012)。
5.根据是否叠加结缔组织病将患者分为两组：两组肌酶谱、血沉值及肌电图检查结果无统计学差异性(P>0.05)。根据肌电图检查前3月内是否接受过激素治疗将患者分为两组：两组患者肌酶谱、血沉值及肌电图检查结果无统计学差异性(P>0.05)。
6.本研究93例PM患者的电生理检查结果中,86例患者为肌源性损害,EMG诊断阳性率为(92.47%)；3例(3.23%)为肌源性损害伴神经性受损,其中1例为糖尿病患者,神经传导检查结果中2例上、下肢所检神经MCV、SCV的传导速度减慢、CMAP波幅及SNAP波幅降低；1例正中神经SCV传导速度轻度减慢。因此,本组PM患者肌电图呈肌源性损害的阳性率为95.7%(89/93)。
4例未见肯定肌源性损害患者中,2例(2.15%)结果为部分所检肌肉MUP平均时限稍缩短,1例(1.58%)左胫前肌可见异常自发电位,1例(1.58%)结果为正常。此4例患者中,1例肌活检提示存在炎性肌病改变,余3例患者均为接受激素治疗,临床症状改善后行肌电图检查。
结论
1.EMG为诊断PM的重要方法,EMG参数中MUP平均时限的改变为最有意义的指标,上肢肌肉肌源性损害阳性率肱二头肌明显高于拇短展肌、远端肌肉肌源性损害阳性率胫前肌明显高于拇短展肌,选择适当的肌肉及关注重点指标可提高PM患者EMG检查呈肌源性损害的阳性率。
2.病程对肱二头肌MUP平均波幅的影响可能较大。不伴有间质性肺病患者较伴有间质性肺病患者病程长,肌电图检查结果及肌酶谱水平提示前者肌肉损害可能较后者更重。
3.肌电图呈混合性损害者,可能的原因为同时伴发了周围神经病。
4.本组PM患者中EMG检查未发现单纯神经源性损害的病例。
Objectives
This study was aimed to investigate the clinical and electrophysiological characteristics of patients with polymyositis.
Materials and methods
1. The clinical data of 93 consecutive patients with PM admitted to Chinese PLA General Hospital between 2006 and 2010 were summarized.
2. The parameters of EMG from 93 PM patients were analyzed. The positive rates of EMG myopathic pattern in different muscles were compared. The EMG patterns in patients with different disease course were also analyzed.
3. The clinical and EMG features of patients with isolated PM were compared with those of patients with interstitial lung disease (ILD) and patients associated with a connective-tissue disorder.
4. The characteristics of EMG and serum levels of CK, LDH, ALT and AST in patients with prior corticosteroids treatment were compared with those of patients without prior specific treatment.
5 The clinical and electrophysiological features were decribed in patients with mixed electrophysiological changes.
Results
1. The mean age of 93 PM patients was 43.19±15.64 years.25.81% of patients were male and 74.19% of them were female. The male/female ratio was 1:2.9. The mean course of disease was 19.16±33.123 (0.3-228) months.18 patients (19.35%) had ILD.17 patients (18.28%) were associated with a connective-tissue disorder, in which,5 patients (29.41%) had rheumatoid arthritis. One patient (1.08%) had non-Hodgkin's lymphoma.36 PM patients (38.71%) were taking corticosteroids at the time of initial EMG examination. Serum CK, LDH, AST, ALT and ESR were elevated in 83.87%,97.33%,85.07%,88.06% and 65% patients respectively. Muscle biopsy was performed in 40 cases,32 (80%) of them showed pathological features consistent with myositis,7 of them (17.5%) showed non-specific muscle fiber necrosis but without inflammatory cell infiltration,1 (2.5%) case was normal histologically.
2. Electrophysiological data (biceps brachii, abductor pollicis brevis, quadriceps femoris, tibialis anterior) of 93 PM patients:abnormal insertional potential was observed in 15.88%, fibrillations was observed in 68.80%, positive sharp waves was observed in 66.85%, CRDs was observed in 6.45% of the muscles tested. The short duration MUPs were observed in 79.94% of the muscles tested. Over 20% increment of polyphasic MUPs was observed in 14.48% of the muscles tested. The myopathic interference pattern (IP) was observed in 9.74% of the muscles tested. The short duration MUPs were the most sensitive electrophysiological parameter in PM patients. The mean amplitude of MUPs (biceps brachii, abductor pollicis brevis, quadriceps femons, tibialis anterior) were 400.39±76.12μV,452.1±88.4μV,447.39±77.1μV and 468.03±214.09μV respectively. The difference of abnormal EMG findings between biceps brachii and quadriceps femoris, quadriceps femoris and tiabialis anterior were not statitically significant (P>0.05). The abnormal EMG findings were more frequent in tibialis anterior than in abductor pollicis brevis (abnormal insertional potential, fibrillations and positive sharp waves, short duration MUPs, over 20% increment of polyphasic MUPs, myopathic IP) (P=0.04、0.001、0.000、0.045、0.018). The abnormal EMG findings were more frequent in biceps brachii than in abductor pollicis brevis (abnormal insertional potential, fibrillations and positive sharp waves, short duration MUPs, over 20% increment of polyphasic MUPs, myopathic IP) (P=0.003、0.008、0.000、0.002).
3. The ALT level was higher and the mean amplitude of MUPs (biceps brachii) was lower in patients with a history longer than 3 months than those with a history shorter than 3 months (P<0.05).
4. All PM cases were divided into two groups. Group A:without ILD,74 cases, mean course:21.77±4.21 months, mean age:49.16±15.10 years. Group B: with ILD,19 cases, mean course:9.02±2.85 months, mean age:41.67±15.51 years. The course was longer in patients in Group A than that of in Group B (P<0.05). The mean age was not statistically different between the two groups. The serum level of CK and LDH in Group A were significantly higher than those in group B. The abnormal EMG findings in biceps brachii and quadriceps femoris in Group A (abnormal spontaneous activity, short duration MUPs, myopathic IP) were significantly more frequent than those in group B (P=0.05、0.00、0.05). Short duration MUPs in tiabialis anterior in group A was more frequent than that in group B (P=0.02). The decrement of mean amplitude of MUPs in tiabialis anterior in group A (440.8±96.59μV) was greater than that in group B (573.79±426.13μV) (P=0.012).
5. The abnormal EMG findings, serum level of CK, LDH, ALT and AST, ESR were not statistically different between isolated PM patients and PM patients associated with connective tissue disease, PM patients without corticosteroids treatment 3 months prior to EMG examination and those with corticosteroids treatment.
6. The isolated myopathic EMG pattern was detected in 86 of 93 PM patients (92.47%). Three cases were myopathic pattern combined with neurogenic pattern (3.23%), one patient had diabetes mellitus. Nerve conduction study revealed MCV and SCV decrement with reduction of CMAP and SNAP amplitude in nerves in two cases. SCV decrement was detected in median nerve in one case. Thus, myopathic EMG pattern was detected in 89 of 93 PM patients (95.7%). The other 4 cases din't show definite myopathic EMG pattern, but slight decrement of mean duration of MUPs was seen in some muscles in two cases and abnormal spontaneous potensials were detected in tibialis anterior in one case. Among the four cases, one was pathologically proved to be inflammatory myopathy, and the other three cases had prior corticosteroids treatment with clinical improvement before EMG examination.
Conclusions
1. EMG was essential for the diagnosis of PM. Short duration MUPs was the most sensitive and significant parameter. The difference of myopathic EMG pattern between proximal and distal muscles was detected in the upper extremities, but not in the lower extremities.
2. The mean amplitude of MUPs in biceps brachii might be lower in patients with longer course. The course of PM patients without ILD was longer than that of PM patients with ILD. And, the abnormal EMG findingds and elevated serum levels of CK and LDH indicated that muscle damage might be severe in PM patients without ILD.
3. The neurogenic EMG pattern in PM may be caused by complicated neuropathy.
4. Isolated neurogenic EMG pattern was not found in this series of PM cases.
Keywords
polymyositis; electromyogram; retrospective analysis; interstitial lung disease; creatine kinase
分析多肌炎(polymyositis, PM)的基本临床特征及其电生理特点。
资料和方法
1.统计描述2006年1月1日-2010年4月1日在我院住院的符合PM临床诊断的93例患者的临床概况。
2.统计描述93例PM患者的电生理特点,采用卡方检验及t检验对比分析不同肌肉肌电图特点及相关指标出现的阳性率。并分析病程对肌电图结果的影响。
3.总结分析PM伴发间质性肺病患者临床资料、肌电图结果,采用卡方检验及t检验分析其与不伴发间质性肺病的PM患者间差异性。
4.采用卡方检验及t检验,分析叠加结缔组织病的PM患者与单纯型PM患者间,及接受过激素治疗的PM患者与未接受过激素治疗的PM患者的肌酶谱、肌电图结果差异性。
5.描述性分析电生理检查结果为非单纯肌源性损害患者的一般临床资料及电生理结果特点。
结果
1.一般资料93例PM患者,男性24例(25.81%),女性69例(74.19%),男：女=1：2.9,平均年龄43.19±15.64岁,总体病程0.3-228月(19.16±33.12月)。伴发间质性肺病患者18例(19.35%),叠加结缔组织病17例(18.28%),其中类风湿性关节炎最多5例(29.41%)比例最高。伴发肿瘤患者1例(1.08%),为非何杰金淋巴瘤。36例患者(38.71%)在肌电图检查前接受过激素治疗。肌酸激酶升高者83.87%,丙氨酸氨基转移酶升高者88.06%,天冬氨酸氨基转移酶升高者85.07%,乳酸脱氢酶升高者97.33%,血沉增快者65%。93例患者共40例(40.01%)行肌肉活检,其中32例(80%)为典型多肌炎表现,7例(17.5%)肌活检结果为非特异性肌纤维坏变,1例(2.5%)肌活检结果正常。
2.本组PM患者常规肌电图检查,包括上、下肢远、近端四块肌肉(肱二头肌、拇短展肌、股四头肌、胫前肌)：插入电位异常出现率为15.88%；自发电位中纤颤电位出现率68.80%；正锐波出现率66.85%；CRDs出现率为6.45%；MUP平均时限缩短超过20%出现率为79.94%；多相波超过20%出现率为14.48%；肌病干扰相出现率为9.74%；MUP平均时限缩短出现异常率在PM患者中相对较高(P=0.000)。MUP平均波幅：肱二头肌：400.39±76.12 umm；拇短展肌452.1±88.4 um；股四头肌：447.39±77.1 um；胫前肌：468.03±214.09 um。应用person卡方分析上、下肢近端肌,上、下肢远端肌,上肢远、近端肌,下肢远、近端肌肌电图结果出现异常率的差异性：肱二头肌与股四头肌、股四头肌与胫前肌各肌电图结果出现异常率,在统计学上无显著差异(P>0.05)。胫前肌(插入电位延长、纤颤正相电位、MUP平均时限缩短、多相波增多、肌病干扰相)出现异常率较拇短展肌明显高(P=0.04、0.001、0.000、0.045、0.018)。肱二头肌(插入电位延长、纤颤正相电位、MUP平均时限缩短、肌病干扰相)出现异常率较拇短展肌明显增高(P=0.003、0.008、0.000、0.002)。
3.根据病程将患者分为两组：A组：病程小于等于3月,B组：病程大于3月,B组患者ALT值相对A组明显增高(P=0.03)。2组肌电图结果B组肱二头肌MUP平均波幅(399.73±81.71)较A组(403.80±36,59)显著低(P=0.025),余结果出现异常率在统计学上无显著差异性(P>0.05)。
4.根据是否伴发间质性肺病将患者分为两组：不伴间质性肺病患者为A组：74例,平均病程21.77±4.21月,平均年龄49.16±15.10岁；伴间质性肺病患者为B组：19例,平均病程9.02±2.85月,平均年龄41.67±15.51岁。A组病程较B组长(P=0.026)；两组年龄分布无显著差异(P=0.495)；A组CK及LDH值明显高于B组,A组肱二头肌及股四头肌测定结果异常率(异常自发电位、MUP平均时限缩短、肌病干扰相)均明显高于B组(P=0.05、0.00、0.05,且胫前肌MUP平均时限缩短出现异常率高于B组(P=0.02)。A组胫前肌MUP平均波幅(440.8±96.59)较B组(573.79±426.13)显著低(P=0.012)。
5.根据是否叠加结缔组织病将患者分为两组：两组肌酶谱、血沉值及肌电图检查结果无统计学差异性(P>0.05)。根据肌电图检查前3月内是否接受过激素治疗将患者分为两组：两组患者肌酶谱、血沉值及肌电图检查结果无统计学差异性(P>0.05)。
6.本研究93例PM患者的电生理检查结果中,86例患者为肌源性损害,EMG诊断阳性率为(92.47%)；3例(3.23%)为肌源性损害伴神经性受损,其中1例为糖尿病患者,神经传导检查结果中2例上、下肢所检神经MCV、SCV的传导速度减慢、CMAP波幅及SNAP波幅降低；1例正中神经SCV传导速度轻度减慢。因此,本组PM患者肌电图呈肌源性损害的阳性率为95.7%(89/93)。
4例未见肯定肌源性损害患者中,2例(2.15%)结果为部分所检肌肉MUP平均时限稍缩短,1例(1.58%)左胫前肌可见异常自发电位,1例(1.58%)结果为正常。此4例患者中,1例肌活检提示存在炎性肌病改变,余3例患者均为接受激素治疗,临床症状改善后行肌电图检查。
结论
1.EMG为诊断PM的重要方法,EMG参数中MUP平均时限的改变为最有意义的指标,上肢肌肉肌源性损害阳性率肱二头肌明显高于拇短展肌、远端肌肉肌源性损害阳性率胫前肌明显高于拇短展肌,选择适当的肌肉及关注重点指标可提高PM患者EMG检查呈肌源性损害的阳性率。
2.病程对肱二头肌MUP平均波幅的影响可能较大。不伴有间质性肺病患者较伴有间质性肺病患者病程长,肌电图检查结果及肌酶谱水平提示前者肌肉损害可能较后者更重。
3.肌电图呈混合性损害者,可能的原因为同时伴发了周围神经病。
4.本组PM患者中EMG检查未发现单纯神经源性损害的病例。
Objectives
This study was aimed to investigate the clinical and electrophysiological characteristics of patients with polymyositis.
Materials and methods
1. The clinical data of 93 consecutive patients with PM admitted to Chinese PLA General Hospital between 2006 and 2010 were summarized.
2. The parameters of EMG from 93 PM patients were analyzed. The positive rates of EMG myopathic pattern in different muscles were compared. The EMG patterns in patients with different disease course were also analyzed.
3. The clinical and EMG features of patients with isolated PM were compared with those of patients with interstitial lung disease (ILD) and patients associated with a connective-tissue disorder.
4. The characteristics of EMG and serum levels of CK, LDH, ALT and AST in patients with prior corticosteroids treatment were compared with those of patients without prior specific treatment.
5 The clinical and electrophysiological features were decribed in patients with mixed electrophysiological changes.
Results
1. The mean age of 93 PM patients was 43.19±15.64 years.25.81% of patients were male and 74.19% of them were female. The male/female ratio was 1:2.9. The mean course of disease was 19.16±33.123 (0.3-228) months.18 patients (19.35%) had ILD.17 patients (18.28%) were associated with a connective-tissue disorder, in which,5 patients (29.41%) had rheumatoid arthritis. One patient (1.08%) had non-Hodgkin's lymphoma.36 PM patients (38.71%) were taking corticosteroids at the time of initial EMG examination. Serum CK, LDH, AST, ALT and ESR were elevated in 83.87%,97.33%,85.07%,88.06% and 65% patients respectively. Muscle biopsy was performed in 40 cases,32 (80%) of them showed pathological features consistent with myositis,7 of them (17.5%) showed non-specific muscle fiber necrosis but without inflammatory cell infiltration,1 (2.5%) case was normal histologically.
2. Electrophysiological data (biceps brachii, abductor pollicis brevis, quadriceps femoris, tibialis anterior) of 93 PM patients:abnormal insertional potential was observed in 15.88%, fibrillations was observed in 68.80%, positive sharp waves was observed in 66.85%, CRDs was observed in 6.45% of the muscles tested. The short duration MUPs were observed in 79.94% of the muscles tested. Over 20% increment of polyphasic MUPs was observed in 14.48% of the muscles tested. The myopathic interference pattern (IP) was observed in 9.74% of the muscles tested. The short duration MUPs were the most sensitive electrophysiological parameter in PM patients. The mean amplitude of MUPs (biceps brachii, abductor pollicis brevis, quadriceps femons, tibialis anterior) were 400.39±76.12μV,452.1±88.4μV,447.39±77.1μV and 468.03±214.09μV respectively. The difference of abnormal EMG findings between biceps brachii and quadriceps femoris, quadriceps femoris and tiabialis anterior were not statitically significant (P>0.05). The abnormal EMG findings were more frequent in tibialis anterior than in abductor pollicis brevis (abnormal insertional potential, fibrillations and positive sharp waves, short duration MUPs, over 20% increment of polyphasic MUPs, myopathic IP) (P=0.04、0.001、0.000、0.045、0.018). The abnormal EMG findings were more frequent in biceps brachii than in abductor pollicis brevis (abnormal insertional potential, fibrillations and positive sharp waves, short duration MUPs, over 20% increment of polyphasic MUPs, myopathic IP) (P=0.003、0.008、0.000、0.002).
3. The ALT level was higher and the mean amplitude of MUPs (biceps brachii) was lower in patients with a history longer than 3 months than those with a history shorter than 3 months (P<0.05).
4. All PM cases were divided into two groups. Group A:without ILD,74 cases, mean course:21.77±4.21 months, mean age:49.16±15.10 years. Group B: with ILD,19 cases, mean course:9.02±2.85 months, mean age:41.67±15.51 years. The course was longer in patients in Group A than that of in Group B (P<0.05). The mean age was not statistically different between the two groups. The serum level of CK and LDH in Group A were significantly higher than those in group B. The abnormal EMG findings in biceps brachii and quadriceps femoris in Group A (abnormal spontaneous activity, short duration MUPs, myopathic IP) were significantly more frequent than those in group B (P=0.05、0.00、0.05). Short duration MUPs in tiabialis anterior in group A was more frequent than that in group B (P=0.02). The decrement of mean amplitude of MUPs in tiabialis anterior in group A (440.8±96.59μV) was greater than that in group B (573.79±426.13μV) (P=0.012).
5. The abnormal EMG findings, serum level of CK, LDH, ALT and AST, ESR were not statistically different between isolated PM patients and PM patients associated with connective tissue disease, PM patients without corticosteroids treatment 3 months prior to EMG examination and those with corticosteroids treatment.
6. The isolated myopathic EMG pattern was detected in 86 of 93 PM patients (92.47%). Three cases were myopathic pattern combined with neurogenic pattern (3.23%), one patient had diabetes mellitus. Nerve conduction study revealed MCV and SCV decrement with reduction of CMAP and SNAP amplitude in nerves in two cases. SCV decrement was detected in median nerve in one case. Thus, myopathic EMG pattern was detected in 89 of 93 PM patients (95.7%). The other 4 cases din't show definite myopathic EMG pattern, but slight decrement of mean duration of MUPs was seen in some muscles in two cases and abnormal spontaneous potensials were detected in tibialis anterior in one case. Among the four cases, one was pathologically proved to be inflammatory myopathy, and the other three cases had prior corticosteroids treatment with clinical improvement before EMG examination.
Conclusions
1. EMG was essential for the diagnosis of PM. Short duration MUPs was the most sensitive and significant parameter. The difference of myopathic EMG pattern between proximal and distal muscles was detected in the upper extremities, but not in the lower extremities.
2. The mean amplitude of MUPs in biceps brachii might be lower in patients with longer course. The course of PM patients without ILD was longer than that of PM patients with ILD. And, the abnormal EMG findingds and elevated serum levels of CK and LDH indicated that muscle damage might be severe in PM patients without ILD.
3. The neurogenic EMG pattern in PM may be caused by complicated neuropathy.
4. Isolated neurogenic EMG pattern was not found in this series of PM cases.
Keywords
polymyositis; electromyogram; retrospective analysis; interstitial lung disease; creatine kinase
引文
[1]Dalakas Mc. Autoimmune muscular pathologies. Neurol Sci.2005,26 (Suppl 1):S72-81.
[2]Mastaglia FL, Garlepp MJ, Phillips BA, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve.2003,27(4):407-425.
[3]Dalakas Mc, Hohlfeldr. Polymyositis and dermatomyositis. Lancet.2003,362 (9388):971-982
[4]Bohan A, Peter JB. Polymyositis and dermatomyositis(second of two parts). N Engl J Med.1975,292(8):403-407.
[5]王保进.多变量分析(统计软件与数据分析).北京大学出版社.2007.
[6]陈超,邹滢SPSS15.0中文版常用功能与应用实例精讲.电子工业出版社.2009.
[7]Sato JO, Sallum AM, Ferriani VP, et al. A Brazilian registry of juvenile dermatomyositis:onset features and classification of 189 cases. Clin Exp Rheumatol.2009,7(6):1031-1038.
[8]Rios G. Retrospective review of the clinical manifestations and outcomes in Puerto Ricans with idiopathic inflammatory myopathies. Clin Rheumatol. 2005,11(3):153-156.
[9]Wilson FC, Ytterberg SR, St Sauver JL, et al. Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota. Rheumatol.2008,35(3):445-447.
[10]Lynn SJ, Sawyers SM, Moller PW, et al. Adult-onset inflammatory myopathy: North Canterbury experience 1989-2001. Intern Med.2005,35(3):170-173.
[11]Prieto S, Grau JM. The geoepidemiology of autoimmune muscle disease. Autoimmun Rev.2010,9(5):A330-334.
[12]Kee SJ, Kim TJ, Lee SJ, et al. Dermatomyositis associated with hepatitis B virus-related hepatocellular carcinoma. Rheumatol Int.2009,29(5):595-599.
[13]Rosti G, Pauni Z, Vojvodi D, et al. Systemic lupus erythematosus and dermatomyositis-case report. Srp Arh Celok Lek.2005,133(Suppl 2):137-140.
[14]Voermans NC, Pillen S, de Jong EM, et al. Morphea profunda presenting as a neuromuscular mimic. Clin Neuromuscul Dis.2008,9(4):407-414.
[15]Holmes MV, Ioannou Y, Borysiewicz C, et al. Juvenile dermatomyositis with Sjogren's syndrome. Clin Rheumatol.2008,27 (Suppl 1):S3-5.
[16]Tsuruta Y, Ikezoe K, Nakagaki H, et al. A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis. Clin Rheumatol. 2004,23(2):160-162.
[17]Szabo N, Lukacs S, Kulcsar I, et al. Association of idiopathic inflammatory myopathy and Crohn's disease. Clin Rheumatol.2009,28(1):99-101.
[18]Crinquette C, De Seze J, Maurage CA, et al. [Polymyositis and cranial neuropathy].Rev Neurol (Paris).2007,163(11):1075-1081.
[19]Yoshidome Y, Morimoto S, Tamura N, et al. A case of polymyositis complicated with myasthenic crisis. Clin Rheumatol.2007,26(9):1569-1570.
[20]Ito Y, Kawabata D, Yukawa N, et al. Severe subcutaneous generalized edema in a patient with dermatomyositis. Mod Rheumatol.2007; 17(2):171-173.
[21]Kokotis P, Theodossiadis P, Bouros C, et al. Bilateral ocular myositis as a late complication of dermatomyositis.Rheumatol.2005,32(2)379-381.
[22]Saygi S, Alehan F, Baskin E, et al. Juvenile dermatomyositis presenting with anasarca. Child Neurol.2008,23(11):1353-1356.
[23]Zedan M, El-Ayouty M, Abdel-Hady H, et al. Anasarca:not a nephrotic syndrome but dermatomyositis. Eur J Pediatr.2008,167(7):831-834.
[24]Ota S, Kasahara A, Yamada T, et al. Dermatomyositis with massive ascites. Rheumatol Int.2007,27(9):877-879.
[25]Allen JA, Greenberg SA, Amato AA. Dermatomyositis-like muscle pathology in patients with chronic graft-versus-host disease. Muscle Nerve. 2009,40(4):643-647.
[26]Tahiri L, Guignard S, Pinto P, et al. Antisynthetases syndrome associated with right heart failure. Joint Bone Spine.2009,76(6):715-717.
[27]Shingu A, Abe S, Seo Y, et al. [A case of polymyositis with concomitant diffuse alveolar damage following interstitial pneumonia] Nihon Kokyuki Gakkai Zasshi.2006,44(12):938-943.
[28]Dressler F, Huppertz HI. [Juvenile dermatomyositis]. Z Rheumatol. 2006,65(7):587-590.
[29]Nishikai M, Akiya K. [Clinical significance of magnetic resonance imaging of skeletal muscles in idiopathic inflammatory myopathies of adults]. Ryumachi. 2000,40(6):881-890.
[30]Pautas E, Cherin P, Piette JC, et al. Features of polymyositis and dermatomyositis in the elderly:a case-control study. Clin Exp Rheumatol. 2000,18(2):241-244.
[31]Dieng MT, Diallo M, Dia D, et al. [Dermatomyositis in Senegal. Study of 56 cases]. Dakar Med.2005;50(3):123-127.
[32]Mebazaa A, Boussen H, Nouira R, et al. Dermatomyositis and malignancy in Tunisia:a multicenter national retrospective study of 20 cases. Am Acad Dermatol.2003,48(4):530-534.
[33]Dourmishev LA, Popov JM, Rusinova D. Paraneoplastic dermatomyositis associated with testicular cancer:a case report and literature review. Acta Dermatovenerol Alp Panonica Adriat.2010,19(1):39-43.
[34]Jamoussi SK, Dhaou BB, Boussema F, et al. [Interstitial pneumonia complicating amyopathic dermatomyositis:a case report]. Rev Pneumol Clin. 2009,65(6):353-356.
[35]Mittal GA, Wadhwani R, Shroff M, et al. Ultrasonography in the diagnosis and follow-up of idiopathic inflammatory myopathies--a preliminary study. Assoc Physicians India.2003,51:252-256.
[36]lijham PJ, Hengstman GJ, Hama-Amin AD, et al. Needle electromyo-graphic findings in 98 patients with myositis. Eur Neurol.2006,55(4):183-188.
[37]宋玉强,沈定国多发性肌炎和皮肌炎患者的临床与肌电图分析.临床神经电生学杂志2002,11(4)：217-219
[38]郭玉璞,王建明,娄连第等多发性肌炎并发周围神经病(附7例临床病理观察).中华神经精神科杂志.1990,23(6)：351-353
[39]Laraki R, Bletry O, Agbalika F, et al. Do neuromyositis exist? Ann Med Interne,1994,145:88.
[40]王维治.神经病学(第4版).北京：人民卫生出版社。2001,297-299.
[41]Bennett Plum主编,白永权总主译.西塞尔内科学四安：世界图书出版西安公司,1999,1542.
[42]王艳晰,陈松林,任廷文.15例神经肌炎临床分析.临床神经病学杂志.2002,15(3)：181-182
[43]袁云,陈清棠,吴丽娟等.多发性肌炎的周围神经改变中华神经精神科杂 志.1993,26(1)：35-37.
[44]熊成仁.多发性肌炎并发周围神经损害.中国综合临床.1996,12(6)：303.
[45]Camdessanche JP, Antoine JC, Honnorat J, et al. Paraneoplastic peripheral neuropathy associated with anti-Hu antibodies. A clinical and electrophysiological study of 20 patients. Brain. 2002.125(1):166-175.
[46]Amato A, Russel JA. Neuromuscular disorders. New York:McGraw-Hill Medical Company.2007,271-288.
[47]Chai J,Herrmann DN, Stanton M, et al. Painful small-fiber neuropathy in Sj(o)gren syndrome. Neurology.2005,65:925-927.
[48]G(o)ransson LG,Tjensvoll AB,Herigstad A, et al. Small-diameter nerve fiber neuropathy in systemic lupus erythematosus. Arch Neurol. 2006,63:401-404.
[49]Matsui N, Mitsui T, Endo I, et al. Dermatomyositis with peripheral nervous system involvement:activation of vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) in vasculitic lesions, Intern Med.2003,42 (12):1233-1239.
[1]Dalakas MC. Autoimmune muscular pathologies. Neurol Sci,2005,26 (Supp 11):S72-81.
[2]Mastaglia FL, Garlepp MJ, Phillips BA, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve,2003,27(4):407-425.
[3]Dalakas Mc, Hohlfeld R. Polymyositis and dermatomyositis. Lancet,2003,362 (9388):971-9821.
[4]Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis:a quantitative study. Ann Neurol 1990;27:343-56.
[5]Dalakas MC. Immunopathogenesis of inflammatory mayopathies. Neurol 1995;37(Suppl.1):S74-S86.
[6]Wiendl H, Hohlfeld R, Kieseier BC. Immunobiology of muscle:advances in understanding an immunological icroenvironment.Trends Immunol 2005;26:373-80.
[7]Michaelis D, Goebels N, Hohlfeld R. Constitutive and cytokineinduced expression of human leukocyte antigens and cell adhesion molecules by human myotubes. Am J Pathol 1993; 143:1142-9.
[8]Nagaraju K, Casciola-Rosen L, Lundberg I, et al. Activation of the endoplasmic reticulum stress response in autoimmune myositis:potential role in muscle fiber damage and dysfunction. Arthritis Rheum 2005,52:1824-35.
[9]Cox FM, Boon EM, van der Lans CA, et al. TREX1 mutations are not associated with sporadic inclusion body myositis.Eur J Neurol.2010 Feb 23.
[10]Starck CS, Sutherland-Smith AJ. Cytotoxic aggregation and amyloid formation by the myostatin precursor protein. PLoS One.2010 Feb 11;5(2):e9170.
[11]Prieto S, Grau JM. The geoepidemiology of autoimmune muscle disease. Autoimmun Rev.2010 Mar; 9(5):A330-A334.
[12]Doppler K, Mittelbronn M, Lindner A. Basement membrane remodelling and segmental fibrosis in sporadic inclusion body myositis. Neuromuscul Disord. 2009 Jun;19(6):406-11.
[13]Dalakas MC, Rakocevic G, Schmidt J, et al. Effect of Alemtuzumab (CAMPATH 1-H) in patients with inclusion-body myositis. Brain.2009 Jun;132(Pt 6):1536-44. Epub 2009 May 19.
[14]崔丽英,潘华,翦凡,等.单纤维肌电图在30例炎性肌病患者诊断中的应用.中华神经科杂志,2005,38(5)：290-292.
[15]. Jian F, Cui LY, Li BH, et al. Changes of single fiber electromyography in patients with inflammatory myopathies. Chin Med Sci J.2005 Mar;20(1):1-4.
[16]. Blijham PJ, Hengstman GJ, Hama-Amin AD, et al. Needle electromyographic findings in 98 patients with myositis. Eur Neurol,2006,55(4):183-188.
[17].宋玉强,沈定国.多发性肌炎和皮肌炎患者的临床与肌电图分析.临床神经电生学杂志,2002,11(4)：217-219.
[18]郑菊阳,樊东升,张朔,等.80例多发性肌炎皮肌炎患者的肌电图研究.临床神经电生学杂志,2006,15(2)：71-73.
[19]Hatanaka Y, Oh SJ. Single-fiber electromyography in sporadic inclusion body myopathy. Clin Neurophysiol,2007,118(7):1563-1568.
[20]Luciano CA, Dalakas MC. Inclusion body myositis:no evidence for a neurogenic component. Neurology,1997,48(1):29-33.
[21]Dabby R, Lange DJ, Trojaborg W, et al. Inclusion body myositis mimicking motor neuron disease. Arch Neurol,2001,58(8):1253-1256.
[22]Brannagan TH, Hays AP, Lange DJ, et al. The role of quantitative electromyo-graphy in inclusion body myositis. J Neurol Neurosurg Psychiatry,1997,63(6):776-779.
[2]Mastaglia FL, Garlepp MJ, Phillips BA, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve.2003,27(4):407-425.
[3]Dalakas Mc, Hohlfeldr. Polymyositis and dermatomyositis. Lancet.2003,362 (9388):971-982
[4]Bohan A, Peter JB. Polymyositis and dermatomyositis(second of two parts). N Engl J Med.1975,292(8):403-407.
[5]王保进.多变量分析(统计软件与数据分析).北京大学出版社.2007.
[6]陈超,邹滢SPSS15.0中文版常用功能与应用实例精讲.电子工业出版社.2009.
[7]Sato JO, Sallum AM, Ferriani VP, et al. A Brazilian registry of juvenile dermatomyositis:onset features and classification of 189 cases. Clin Exp Rheumatol.2009,7(6):1031-1038.
[8]Rios G. Retrospective review of the clinical manifestations and outcomes in Puerto Ricans with idiopathic inflammatory myopathies. Clin Rheumatol. 2005,11(3):153-156.
[9]Wilson FC, Ytterberg SR, St Sauver JL, et al. Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota. Rheumatol.2008,35(3):445-447.
[10]Lynn SJ, Sawyers SM, Moller PW, et al. Adult-onset inflammatory myopathy: North Canterbury experience 1989-2001. Intern Med.2005,35(3):170-173.
[11]Prieto S, Grau JM. The geoepidemiology of autoimmune muscle disease. Autoimmun Rev.2010,9(5):A330-334.
[12]Kee SJ, Kim TJ, Lee SJ, et al. Dermatomyositis associated with hepatitis B virus-related hepatocellular carcinoma. Rheumatol Int.2009,29(5):595-599.
[13]Rosti G, Pauni Z, Vojvodi D, et al. Systemic lupus erythematosus and dermatomyositis-case report. Srp Arh Celok Lek.2005,133(Suppl 2):137-140.
[14]Voermans NC, Pillen S, de Jong EM, et al. Morphea profunda presenting as a neuromuscular mimic. Clin Neuromuscul Dis.2008,9(4):407-414.
[15]Holmes MV, Ioannou Y, Borysiewicz C, et al. Juvenile dermatomyositis with Sjogren's syndrome. Clin Rheumatol.2008,27 (Suppl 1):S3-5.
[16]Tsuruta Y, Ikezoe K, Nakagaki H, et al. A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis. Clin Rheumatol. 2004,23(2):160-162.
[17]Szabo N, Lukacs S, Kulcsar I, et al. Association of idiopathic inflammatory myopathy and Crohn's disease. Clin Rheumatol.2009,28(1):99-101.
[18]Crinquette C, De Seze J, Maurage CA, et al. [Polymyositis and cranial neuropathy].Rev Neurol (Paris).2007,163(11):1075-1081.
[19]Yoshidome Y, Morimoto S, Tamura N, et al. A case of polymyositis complicated with myasthenic crisis. Clin Rheumatol.2007,26(9):1569-1570.
[20]Ito Y, Kawabata D, Yukawa N, et al. Severe subcutaneous generalized edema in a patient with dermatomyositis. Mod Rheumatol.2007; 17(2):171-173.
[21]Kokotis P, Theodossiadis P, Bouros C, et al. Bilateral ocular myositis as a late complication of dermatomyositis.Rheumatol.2005,32(2)379-381.
[22]Saygi S, Alehan F, Baskin E, et al. Juvenile dermatomyositis presenting with anasarca. Child Neurol.2008,23(11):1353-1356.
[23]Zedan M, El-Ayouty M, Abdel-Hady H, et al. Anasarca:not a nephrotic syndrome but dermatomyositis. Eur J Pediatr.2008,167(7):831-834.
[24]Ota S, Kasahara A, Yamada T, et al. Dermatomyositis with massive ascites. Rheumatol Int.2007,27(9):877-879.
[25]Allen JA, Greenberg SA, Amato AA. Dermatomyositis-like muscle pathology in patients with chronic graft-versus-host disease. Muscle Nerve. 2009,40(4):643-647.
[26]Tahiri L, Guignard S, Pinto P, et al. Antisynthetases syndrome associated with right heart failure. Joint Bone Spine.2009,76(6):715-717.
[27]Shingu A, Abe S, Seo Y, et al. [A case of polymyositis with concomitant diffuse alveolar damage following interstitial pneumonia] Nihon Kokyuki Gakkai Zasshi.2006,44(12):938-943.
[28]Dressler F, Huppertz HI. [Juvenile dermatomyositis]. Z Rheumatol. 2006,65(7):587-590.
[29]Nishikai M, Akiya K. [Clinical significance of magnetic resonance imaging of skeletal muscles in idiopathic inflammatory myopathies of adults]. Ryumachi. 2000,40(6):881-890.
[30]Pautas E, Cherin P, Piette JC, et al. Features of polymyositis and dermatomyositis in the elderly:a case-control study. Clin Exp Rheumatol. 2000,18(2):241-244.
[31]Dieng MT, Diallo M, Dia D, et al. [Dermatomyositis in Senegal. Study of 56 cases]. Dakar Med.2005;50(3):123-127.
[32]Mebazaa A, Boussen H, Nouira R, et al. Dermatomyositis and malignancy in Tunisia:a multicenter national retrospective study of 20 cases. Am Acad Dermatol.2003,48(4):530-534.
[33]Dourmishev LA, Popov JM, Rusinova D. Paraneoplastic dermatomyositis associated with testicular cancer:a case report and literature review. Acta Dermatovenerol Alp Panonica Adriat.2010,19(1):39-43.
[34]Jamoussi SK, Dhaou BB, Boussema F, et al. [Interstitial pneumonia complicating amyopathic dermatomyositis:a case report]. Rev Pneumol Clin. 2009,65(6):353-356.
[35]Mittal GA, Wadhwani R, Shroff M, et al. Ultrasonography in the diagnosis and follow-up of idiopathic inflammatory myopathies--a preliminary study. Assoc Physicians India.2003,51:252-256.
[36]lijham PJ, Hengstman GJ, Hama-Amin AD, et al. Needle electromyo-graphic findings in 98 patients with myositis. Eur Neurol.2006,55(4):183-188.
[37]宋玉强,沈定国多发性肌炎和皮肌炎患者的临床与肌电图分析.临床神经电生学杂志2002,11(4)：217-219
[38]郭玉璞,王建明,娄连第等多发性肌炎并发周围神经病(附7例临床病理观察).中华神经精神科杂志.1990,23(6)：351-353
[39]Laraki R, Bletry O, Agbalika F, et al. Do neuromyositis exist? Ann Med Interne,1994,145:88.
[40]王维治.神经病学(第4版).北京：人民卫生出版社。2001,297-299.
[41]Bennett Plum主编,白永权总主译.西塞尔内科学四安：世界图书出版西安公司,1999,1542.
[42]王艳晰,陈松林,任廷文.15例神经肌炎临床分析.临床神经病学杂志.2002,15(3)：181-182
[43]袁云,陈清棠,吴丽娟等.多发性肌炎的周围神经改变中华神经精神科杂 志.1993,26(1)：35-37.
[44]熊成仁.多发性肌炎并发周围神经损害.中国综合临床.1996,12(6)：303.
[45]Camdessanche JP, Antoine JC, Honnorat J, et al. Paraneoplastic peripheral neuropathy associated with anti-Hu antibodies. A clinical and electrophysiological study of 20 patients. Brain. 2002.125(1):166-175.
[46]Amato A, Russel JA. Neuromuscular disorders. New York:McGraw-Hill Medical Company.2007,271-288.
[47]Chai J,Herrmann DN, Stanton M, et al. Painful small-fiber neuropathy in Sj(o)gren syndrome. Neurology.2005,65:925-927.
[48]G(o)ransson LG,Tjensvoll AB,Herigstad A, et al. Small-diameter nerve fiber neuropathy in systemic lupus erythematosus. Arch Neurol. 2006,63:401-404.
[49]Matsui N, Mitsui T, Endo I, et al. Dermatomyositis with peripheral nervous system involvement:activation of vascular endothelial growth factor (VEGF) and VEGF receptor (VEGFR) in vasculitic lesions, Intern Med.2003,42 (12):1233-1239.
[1]Dalakas MC. Autoimmune muscular pathologies. Neurol Sci,2005,26 (Supp 11):S72-81.
[2]Mastaglia FL, Garlepp MJ, Phillips BA, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve,2003,27(4):407-425.
[3]Dalakas Mc, Hohlfeld R. Polymyositis and dermatomyositis. Lancet,2003,362 (9388):971-9821.
[4]Emslie-Smith AM, Engel AG. Microvascular changes in early and advanced dermatomyositis:a quantitative study. Ann Neurol 1990;27:343-56.
[5]Dalakas MC. Immunopathogenesis of inflammatory mayopathies. Neurol 1995;37(Suppl.1):S74-S86.
[6]Wiendl H, Hohlfeld R, Kieseier BC. Immunobiology of muscle:advances in understanding an immunological icroenvironment.Trends Immunol 2005;26:373-80.
[7]Michaelis D, Goebels N, Hohlfeld R. Constitutive and cytokineinduced expression of human leukocyte antigens and cell adhesion molecules by human myotubes. Am J Pathol 1993; 143:1142-9.
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