Safety of a home exercise programme in patients with polymyositis and dermatomyositis: a pilot study.

OBJECTIVES: To investigate whether a home exercise programme could safely be performed by patients with stable, inactive polymyositis (PM) and dermatomyositis (DM), regarding disease activity, muscle function, health status and pain. METHODS: Ten patients with reduced muscle function completed the study. A home exercise programme including exercises for strength in the upper and lower limbs, neck and trunk, for mobility in the upper limbs and moderate stretching was developed. The patients exercised for 15 min and took a 15 min walk 5 days a week during a 12 week period. Assessments included clinical evaluation of disease activity, serum creatinine phosphokinase (CPK) levels, magnetic resonance imaging (MRI) of the quadriceps, repeated muscle biopsy of the vastus lateralis, a muscle function index (FI), a walking test and a health status instrument (the SF 36) performed at the start of the study and after 12 weeks. RESULTS: After 12 weeks of exercise, there were no signs of increased disease activity as assessed clinically, by CPK values, MRI or muscle biopsy findings. On an individual basis, all patients improved regarding muscle function according to the FI, in six cases the improvement reached statistical significance (P < 0.05). A significant improvement regarding muscle function in the upper and lower limbs, walking distance and general health status was achieved. CONCLUSIONS: Our results indicate that this home exercise programme can be safely employed in patients with stable, inactive PM and DM, with beneficial effects on muscle function.     

Benefits of intensive resistance training in patients with chronic polymyositis or dermatomyositis

OBJECTIVE: To investigate the benefits and safety of an intensive muscular training program in patients with chronic polymyositis (PM) and dermatomyositis (DM). METHODS: Nine patients with chronic PM or DM (median age 53 years, range 44-61) were included. Assessments of impairment (10-15 voluntary repetition maximum [VRM], the Functional Index 2 [FI-2], the Grippit, and pain rated on the Borg CR-10 scale), activity limitation (Myositis Activities Profile), and participation restriction (patients' disease impact on well-being) were performed 4 weeks prior to baseline, at baseline, and after 7 weeks of exercise. A 6-item core set of disease activity measures was administered and muscle biopsy samples of vastus lateralis were obtained at baseline and after 7 weeks of exercise. Response criteria at an individual level were set for disability and disease activity. The patients exercised 3 days per week for 7 weeks on loads allowing 10 VRM. RESULTS: On a group level there were no significant differences between assessments at 4 weeks before baseline compared with baseline. The group improved significantly regarding 10-15 VRM and FI-2 at 7 weeks compared with baseline (P < 0.05). All patients were responders with respect to impairment and 2 were activity limitation responders whereas participation restriction remained unchanged in all. Two patients were responders with reduced disease activity and no patient had signs of increased muscle inflammation in the muscle biopsy sample after 7 weeks of exercise. CONCLUSION: Patients with chronic, stable PM and DM can perform this intensive resistive exercise program with beneficial effects on impairment and activity limitation without increased muscle inflammation.     

皮肌炎/多发性肌炎与恶性肿瘤

目的　了解皮肌炎 (dermatomyositis ,DM)及多发性肌炎 (polymyositis,PM )患者的恶性肿瘤发生情况。方法　收集 1989— 1998年北京协和医院收治的资料完整的DM/PM患者 143例 ,全部符合 1975年Bohan和Peter提出的DM /PM诊断标准 ,对其中 18例合并肿瘤的患者与非肿瘤患者进行临床分析 ,肿瘤诊断及分型标准根据我国卫生部制定的肿瘤诊断分级标准而定。两组资料采用t及 χ2 检验。结果　 143例患者男性 46 ,女性 97例。发病年龄 4～ 76岁 ,平均年龄 (42± 38)岁 ,其中PM5 0例 ,男 14例 ,女 36例 ,DM93例 ,男 2 4例 ,女 6 9例。PM的肿瘤发生率为 8% ,DM的肿瘤发生率为 15 6 %。肿瘤分型 :卵巢癌 3例 ,鼻咽癌 3例 ,恶组 2例 ,乳癌 2例 ,宫颈癌、子宫内膜卵巢样癌、肺癌、胃癌、前列腺癌、直肠癌、多发性骨髓瘤、淋巴瘤各 1例。肿瘤先行发生者 3例 (16 6 % ) ,后续发生者 5例 (2 7 8% ) ,同时发生者 10例 (5 5 6 % )。本组病例中 10例死亡 ,从PM /DM诊断到死亡的时间为 1～ 84个月 [中位数为 (18± 2 7)个月 ],死亡原因包括肿瘤扩散 ,肿瘤相关的多脏器功能衰竭及感染。发病年龄、肺部和心脏受累 ,吞咽困难等指标在两组间显示有统计学意义。其中 1例在肿瘤切除 1年后 ,PM症状消失 ,另 1例在肿瘤切除后 1 5年     

Herpes zoster in patients with polymyositis and dermatomyositis

Twenty-two patients with polymyositis and dermatomyositis (PM-DM) were retrospectively studied with regard to development of herpes zoster. Herpes zoster occurred with high frequency in patients with PM-DM. The clinical courses of zoster infections were uneventful; no severe complications nor deaths occurred, and only one patient had post-therapeutic neuralgia. No specific therapy for this infection was necessary. Zoster tended to occur in the inactive stage of PM-DM. PM-DM patients with herpes zoster had a significantly higher incidence of antinuclear antibody. There seemed to be no relationship between steroid therapy and herpes zoster infection.     

皮肌炎/多发性肌炎患者糖皮质激素受体的研究

目的探讨皮肌炎，多发性肌炎患者外周血单个核细胞糖皮质激素受体（GR）的变化意义及其机制。方法采用放射配基结合位点分析法与反转录聚合酶链反应（RT—PCR）方法检测36例皮肌炎，多发性肌炎治疗前后及30名正常对照组外周血单个核细胞糖皮质激素受体位点数及GRmRNA表达水平，同时采用放射免疫分析法检测患者血中糖皮质激素水平。结果皮肌炎，多发性肌炎患者血中糖皮质激素水平与正常对照组相比，差异无统计学意义（P〉0．05）；而糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平，较正常对照组降低，差异有统计学意义（P〈0．01）；治疗前糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平高者，治疗效果好，而治疗前糖皮质激素受体位点数[位点，细胞]及GRmRNA表达水平低者治疗效果差。结论皮肌炎，多发性肌炎患者糖皮质激素受体位点数及GRmRNA表达水平降低，糖皮质激素受体的变化不仅与皮肌炎，多发性肌炎的发病有关，还与治疗反应有关。皮肌炎，多发性肌炎患者糖皮质激素受体位点目的减少可能与GRmRNA表达水平降低有关。     

多发性肌炎或皮肌炎心脏损害的临床分析

目的分析多发性肌炎或皮肌炎（polymyositis/dermatomyositis,PM/DM）心脏损害的特征,探讨心脏损害发生的危险因素.方法收集423例PM/DM的临床资料,分析心脏损害的发生率、心脏损害的类型以及可能诱发心脏损害的危险因素.结果心脏损害的发生率为42.6%（180/423）,表现为心肌缺血、心律失常、心包炎、心肌损害、肺动脉高压、瓣膜病变、传导阻滞、肢导低电压和其他心脏损害;多因素分析发现血沉是心脏损害发生的危险因素.结论心脏损害是PM/DM的常见并发症,表现复杂多样.血沉增快提示心脏损害的危险性增加,而与年龄、性别及病程长短等因素无关.     

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.     

多发性肌炎和皮肌炎自身抗体研究进展

特发性炎性肌病是一组临床异质性自身免疫疾病,临床上以多发性肌炎（PM）和皮肌炎（DM）较为多见.自身抗体在特发性炎性肌病的发病过程中发挥重要作用,而且与不同的临床表现密切相关.以下就肌炎自身抗体领域的研究进展进行综述.     

Survival analysis of patients with dermatomyositis and polymyositis

To estimate the mortality rate and identify factors predicting survival in patients with polymyositis (PM) and dermatomyositis (DM). The medical records of 192 PM/DM patients who were treated at Chang Gung Memorial Hospital from 1999 through 2008 were retrospectively reviewed. The Taiwan National Death Registry (1999–2008) was used to obtain their survival status. Thirty-one (16.1%) of the 192 patients with PM/DM had an associated malignancy; 41 (21.4%) had interstitial lung disease (ILD). During the follow-up period, 55 (28.6%) patients died and the overall cumulative survival rate was 79.3% at 1 year, 75.7% at 2 years, 69.9% at 5 years, and 66.2% at 10 years. In univariate analysis, older age at PM/DM onset, anemia, thrombocytopenia, leukopenia, diabetes mellitus, ILD, cancer, and non-use of azathioprine were associated with higher mortality (p = 0.0172, 0.0484, <0.0001, 0.0008, 0.0001, 0.0036, 0.0010, and 0.0019, respectively). In multivariate Cox regression analysis, thrombocytopenia (hazard ratio [HR] 4.94, 95% confidence interval [CI] 2.60–9.37, p < 0.0001), diabetes mellitus (HR 2.57, 95% CI 1.38–4.80, p < 0.0001), cancer (HR 2.30, 95% CI 1.26–4.22, p = 0.0030), and ILD (HR 1.98, 95% CI 1.11–3.51, p = 0.0182) were positively associated with mortality. Use of azathioprine (HR 0.35, 95% CI 0.16–0.74, p = 0.0064) was negatively associated with mortality. This study confirmed the high mortality rate (28.6%) in PM/DM patients. Survival time was significantly reduced in patients with thrombocytopenia, diabetes mellitus, ILD, and cancer patients than in those without these conditions.     

Mortality of Chinese patients with polymyositis and dermatomyositis

Clinical Rheumatology - To investigate the mortality and the causes of death in Chinese patients with polymyositis (PM) and dermatomyositis (DM). The clinical data of all consecutive adult PM/DM...     

Pregnancy outcomes in adult patients with dermatomyositis and polymyositis

ObjectiveThe idiopathic inflammatory myopathies dermatomyositis (DM) and polymyositis (PM) are autoimmune diseases that can affect females of childbearing potential. We assessed pregnancy outcomes in DM and PM patients compared with the general obstetric population.MethodsThe Nationwide Inpatient Sample (NIS) (1993–2007) was used to identify delivery-associated hospitalizations in women with DM or PM (DM/PM, n = 853). Controls were from the general obstetric population delivery-associated hospitalizations matched to each case by year of delivery. Pregnancy outcomes included hospital length of stay (LOS), hypertensive disorders (HTN), premature rupture of membranes (PROM), intrauterine growth restriction (IUGR), and cesarean delivery. Multivariate regression analyses were performed using maternal age, race/ethnicity, and diabetes mellitus as covariates.ResultsOn multivariate analysis, patients with DM/PM had longer LOS compared to controls (p < 0.001). DM/PM was associated with an increased risk of hypertensive disorders compared to controls (OR = 2.90, 95% CI: 2.00–4.22). There were no differences in rates of PROM, IUGR, or cesarean section in patients with DM/PM compared with controls. Independent of a DM/PM diagnosis, African-American race, older age, and diagnosis of diabetes increased the hospital LOS (p < 0.001). African-American race and diabetes increased the risk of hypertensive disorders (OR = 1.38, 95% CI: 1.19–1.60; OR = 2.94, 95% CI: 2.04–4.23, respectively) compared to controls.ConclusionThese data suggest that patients with inflammatory myopathies are at increased risk of hypertensive disorders of pregnancy and longer length of hospitalization. Vigilant monitoring of blood pressure is advisable in pregnant patients with DM or PM.     

Anti-p53 antibodies in patients with dermatomyositis/polymyositis

Dermatomyositis/polymyositis (DM/PM), which is often accompanied by various immunological abnormalities, was reported to be associated with an increased incidence of malignancies. In this study, we analyzed serum levels of anti-p53 antibody (anti-p53 Ab) in DM/PM patients and in normal controls. Serum levels of anti-p53 Abs were significantly higher in DM/PM patients than those in healthy controls. However, there was no significant difference between serum levels in patients with malignancies and those in patients without malignancies. Anti-p53 Abs were positive in 13% (4 out of 31) of the DM/PM patients. Of these four patients, only one had an internal malignancy. Immunoglobulin G levels were significantly higher in patients positive for anti-p53 Ab than those who were not. These results seemed to suggest that the presence of anti-p53 Abs in DM/PM patients is due to immunological abnormalities in this disease.     

多肌炎和皮肌炎患者病理切片浸润淋巴细胞分型及其意义

目的 观察确诊的多肌炎和皮肌炎（ＰＭ／ＤＭ）患者皮肤,肌肉病理切片中浸润淋巴细胞的分型情况,研究分型与ＰＭ／ＤＭ临床表现的关系。方法 采用免疫组化法测定４０例确依的ＰＭ／ＤＭ患者皮肤,肌肉病理切片中淋巴细胞的具体百分比。结果 ＤＭ患者皮下及肌膜间浸润淋巴细胞以Ｔ细胞为主,血管周围以Ｂ细胞多见。ＰＭ患者浸润淋巴细胞在皮肤,肌膜间及血管周围均以Ｔ细胞为主。结论细胞免疫反应参与ＰＭ发病,而在ＤＭ,细胞免     

The reliability of an aerobic and an anaerobic exercise tolerance test in patients with juvenile onset dermatomyositis

OBJECTIVE: To investigate the reliability of an aerobic and an anaerobic exercise test in patients with juvenile dermatomyositis (JDM). METHODS: Sixteen patients with JDM (mean age 13.85 +/- 6.4 yrs, range 6.7-27.2) participated. Anaerobic exercise capacity was measured using the Wingate Anaerobic Exercise Test (WAnT). Aerobic exercise test was performed using a graded exercise test to volitional exhaustion on an electronically braked cycle ergometer. Patients were tested and retested within 12.8 +/- 5.7 days. RESULTS: Correlation coefficients and other reliability statistics indicated that peak power and mean power on the WAnT and peak oxygen uptake (VO2peak) and maximal workload (Wmax) on the aerobic exercise test could be reliably assessed in patients with JDM. Pearson (R) and intraclass correlation coefficients (ICC) were > 0.85, and typical error was < 20% for the peak power and mean power on the WAnT. On the aerobic exercise test the R and ICC were > 0.95, and typical error was < 6% for the VO2peak and Wmax. CONCLUSION: We found acceptable reliability for the WAnT and very good reliability for the aerobic exercise test. This indicated that these exercise tests could be performed reliably in patients with JDM.     

Serum KL-6 in adult patients with polymyositis and dermatomyositis

OBJECTIVE: To determine the serum levels of KL-6, a mucin-like high-molecular-weight glycoprotein, in polymyositis/dermatomyositis (PM/DM) patients. METHODS: Serum samples from 42 adult PM/DM patients and 38 healthy control subjects were examined using specific enzyme-linked immunosorbent assay (ELISA) systems. RESULTS: The serum levels of KL-6 in the PM/DM patients were significantly higher than those of the healthy controls. The elevated serum KL-6 levels correlated with the presence of interstitial lung disease (ILD) and decreased percentage diffusing capacity of carbon monoxide (%DLco) in the PM/DM patients. The serum KL-6 levels inversely correlated with %DLco and percentage vital capacity. Additionally, the serum KL-6 levels were elevated in five of the seven DM patients with malignant neoplasia. CONCLUSIONS: These results suggest that the serum KL-6 level might be a useful serum marker for ILD and internal malignancy in PM/DM.     

The clinical utility of serum IL-35 in patients with polymyositis and dermatomyositis

The objectives of this study are to assess the levels of serum Interleukin-35 (IL-35) in patients with idiopathic inflammatory myopathies (IIMs) and to evaluate the association between IL-35 levels and IIM-related features. Serum IL-35 was detected in 76 patients with dermatomyositis (DM), 28 patients with polymyositis (PM), 98 disease controls (40 rheumatoid arthritis (RA), 34 systemic lupus erythematosus (SLE), 12 systemic sclerosis (SSc), and 12 sjogren syndrome (SS)), and 43 healthy controls by ELISA. Follow-up was conducted on 34 patients. Serum IL-35 was higher in myositis (PM/DM) patients than in healthy controls (median 76.6 pg/ml [interquartile range (IQR) 57.9–136.2] vs. 29.9 pg/ml (IQR 21.9–65.5), P < 0.001) and disease controls. Serum IL-35 in IIM patients negatively correlated with disease duration moderately (r = ?0.35, P < 0.01). Patients with dysphagia had higher IL-35 than those without (median149.35 pg/ml (IQR 87.97–267.32) vs. 70.72 pg/ml (IQR 54.49–123.42), P = 0.001). Cross-sectional correlation analysis showed a weak positive correlation between serum IL-35 and CK (r = 0.293, P = 0.003), moderate positive correlation with erythrocyte sedimentation rate (ESR) (r = 0.304, P = 0.002), serum ferritin (SF) (r = 0.467, P = 0.001) and LDH levels (r = 0.401, P < 0.001). Additionally, serum IL-35 was higher in patients who were positive for anti-HMGCR (median 292.04 pg/ml (IQR 67.9–442.86) vs. 74.66 pg/ml (IQR 57.24–131.32), P = 0.038) and anti-SRP antibody (median 130.33 pg/ml (IQR 88.04–481.28) vs. 73.06 pg/ml (IQR 56.78–134.28), P = 0.009) than in negative patients, respectively. Follow-up study showed that changes in IL-35 levels after treatment correlated with changes in MYOACT scores moderately (r = 0.375, P = 0.029). These data indicate that increased serum IL-35 could act as a disease activity marker and as a risk factor for esophageal involvement in IIM. IL-35 may participate in the pathophysiological processes of IIM, but it still needs further study to confirm.