Search

Objective: To assess the longitudinal changes in nailfold videocapillaroscopy (NVC) in patients expressing myositis-specific autoantibodies [anti-aminoacyl-tRNA synthetase (ARS), anti-transcriptional intermediary factor 1 (TIF1), and anti-melanoma differentiation-associated gene 5 (MDA5)]., Methods: This study was performed retrospectively, at a single site, on an observational cohort. Seventy-one idiopathic inflammatory myopathy patients were included (25 patients expressed anti-MDA5 Abs, 24 patients expressed anti-TIF1 Abs, and 22 patients expressed anti-ARS Abs). NVC findings included giant, enlarged, and reduced capillaries, haemorrhages, capillary ramification, disorganization of the vascular array, and capillary loss. NVC findings were compared from baseline to after disease activity stabilization., Results: The frequency of enlarged capillaries at baseline was different among the three groups, and was significantly higher in patients with anti-TIF1 Abs compared with those with anti-ARS Abs (88% vs 55%, P < 0.05). Reduced capillaries were significantly increased in patients with anti-TIF1 Abs compared with those with anti-MDA5 (96% vs 44%, P < 0.0001) or anti-ARS Abs (96% vs 50%, P < 0.0005). Both enlarged and reduced capillaries improved after stabilization in patients with anti-MDA5 Abs (P < 0.0001 and P < 0.05, respectively). These improvements were not observed in patients expressing anti-TIF1 and anti-ARS Abs. However, a significant reduction in haemorrhages was observed in all three groups (P < 0.0001 for each group)., Conclusions: The results of this study demonstrate that longitudinal changes in NVC findings may vary depending on myositis-specific Ab expression. Therefore, it is crucial to assess individual NVC findings separately, as each finding may impact disease activity in a different manner., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers., Patients and Methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan)., Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups., Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2023, Turkish League Against Rheumatism.)

We aimed to examine the prevalence of injury after physical therapy intervention for muscle tightness and injury prevention in male high school soccer players. A randomized controlled trial was conducted. Participants comprised 124 players from two high schools who competed in national tournament soccer games held from April 2018 to March 2019. Players were randomly divided into intervention (with a 12-week stretching intervention by physical therapists) and control groups (without the intervention). Players and coaches provided written information regarding injuries and daily training and match times; physical therapists visited each team weekly to collect data and review documentation. Muscle tightness and injury incidence, number, type, location, circumstances, situations, severity, and contents during the 12-week intervention period and a subsequent 40-week observation period were compared between groups. Injuries were significantly lower with intervention during the 40-week observation period (P < .01) but not during the 12-week intervention period (P = .44). Injury types mainly included disorder, non-contact, lower-limb/trunk, and muscle/tendon injuries. Significant interactions were observed for all tightness-test measurement items. The intervention group showed significant improvements in heel-buttock distance, and straight leg-raise and hip rotation angles (pre-intervention < 12 weeks < 52 weeks), as well as significant improvements in ankle dorsiflexion angles at 12 and 52 weeks (relative to pre-intervention values). Instructed stretching exercises, personally designed by physical therapists to address muscle tightness, improved the range of motion and trunk flexibility, with a positive effect on the injury rate in male high school soccer players, especially for non-contact disorder injuries during training., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: To assess the functional disability in Japanese patients with systemic sclerosis (SSc) using the EuroQol-5-Domain-5-Level health questionnaire (EQ-5D-5L), which was developed in Europe to demonstrate the cost utility of treatments for non-specific diseases. Methods: The EQ-5D-5L and Disability Index of the Health Assessment Questionnaire (HAQ-DI), which is a questionnaire for the quality of life for rheumatic diseases, were completed by 109 Japanese patients with SSc, and the clinical findings and laboratory data were collected at the same time. Results: There was a correlation between the EQ-5D-5L score and HAQ-DI score. The EQ-5D-5L index score was affected by the % of predicted vital capacity (%VC), pulmonary arterial hypertension, and renal crisis. The %VC and renal crisis were also indicated as factors reducing the quality of life in the HAQ-DI. There was no difference in the EQ-5D-5L score between the SSc subtypes or among autoantibodies. Conclusion: Our single-center study demonstrated the EQ-5D-5L to be a valuable assessment tool for functional disability in Japanese SSc patients, similarly to the disease specific HAQ-DI.

Introduction: The COPD Assessment Test (CAT) is utilised to evaluate the treatment outcome regarding the health status in idiopathic interstitial pneumonia (IIP). However, the ability of the CAT to evaluate the lung-specific quality of life in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is not established. Therefore, we investigated whether CAT scores can be used to evaluate SSc-ILD as they are for IIP., Methods: A total of 150 patients with IIP or SSc-ILD who were evaluated by the CAT were retrospectively assigned to this study. Clinical data at the visit for the CAT were analysed., Results: The forced vital capacity and distance walked during the 6-minute walk test (6MWD) were significantly correlated with the CAT score for SSc-ILD and IIP, and the CAT scores were similarly distributed in SSc-ILD and IIP. The CAT score of SSc-ILD patients was negatively affected by pulmonary arterial hypertension, but not by corticosteroids, which affected it in IIP patients. CAT scores of patients with either disease receiving home oxygen therapy were poor. In multiple regression analysis, pulmonary arterial hypertension and 6MWD were independent predictors for the CAT score in patients with SSc-ILD, while corticosteroid administration was selected as an independent factor in patients with IIP., Conclusions: Our study suggests that the CAT can be applied to evaluate the lung-specific quality of life in SSc-ILD similar to IIP regarding the pulmonary function, but it should be noted that pulmonary arterial hypertension in SSc-ILD influences the CAT score., (© 2020 The Authors. The Clinical Respiratory Journal published by John Wiley & Sons Ltd.)

Objective: Severe skin sclerosis in patients with systemic sclerosis (SSc) can result in a loss of hand function. The aim of this study is to examine the long-term changes of finger passive range of motion (ROM) in Japanese SSc patients treated with self-administered stretching., Methods: This is a single-center, retrospective, observational cohort study. Forty-three Japanese patients with SSc were given instructions on self-administered stretching. ROM was assessed using a goniometer on their first visit and after 1 year, 3 years, 5 years and 9 years. Hand function was assessed by the Health Assessment Questionnaire disability index (HAQ-DI) at their first visit and after 9 years., Results: Total passive ROM significantly improved in each finger after 3 years of finger stretching. Most patients (37 of 43 patients, 86%) improved or maintained total passive ROM and hand function within 9 years after their first visit. However, significant improvement of total passive ROM was lost in 6 of 43 SSc patients (14%) 9 years after their first visit. The HAQ-DI also was increased in these six patients. Multivariable analyses revealed that re-elevation of modified Rodnan total skin thickness score during the clinical course (OR = 5.260e + 7, 95% CI 1.52e + 150-uncalculated p = .0096) was the independent factor associated with deterioration of total passive ROM at 9 years., Conclusion: Patients with progressive skin sclerosis during the clinical course need multimodality therapy to maintain finger joint motion, since the effect of self-administered stretching is limited in these patients.

Introduction: High-dose chemotherapy for remission induction can induce cardiac toxicity prior to hematopoietic stem cell transplantation (HSCT). However, the details of cardiac function in patients undergoing HSCT are unclear, particularly during exercise. The aim of present study was to evaluate cardiac output responses to exercise in patients after high-dose chemotherapy before HSCT compared with in age-matched healthy controls., Methods: Twenty-nine patients before HSCT (age 44.6 ± 15.2 years) and 13 controls (45.8 ± 16.0 years) performed the 6-min walk test (6MWT). Cardiac output (CO), stroke volume (SV), heart rate (HR), and cardiac index (CI) were continuously measured during the 6MWT using the noninvasive thoracic impedance method., Results: No significant difference was observed in the six-minute walk distance (6MD) between the two groups. SV, CO, and CI both at rest and the end of the 6MWT in the patients were significantly lower, compared with the controls, but there was no significant difference in HR. In all subjects, the 6MD was positively correlated with SV, CO, CI, and HR after the 6MWT., Conclusion: These findings suggest that cardiac output response to exercise indicates exercise intolerance, which may not be detected by 6MD in patients before HSCT.

Idiopathic inflammatory myopathies involve skeletal muscles and can be associated with interstitial lung disease and/or heart dysfunction, which may reduce exercise capacity. We aimed to clarify cardiopulmonary factors affecting the 6-min walk distance in patients who were able to walk without leg pain or fatigue. Twenty-three patients with inactive adult idiopathic inflammatory myopathies, and 18 age- and gender-matched healthy controls were evaluated for hemodynamic responses using noninvasive impedance cardiography during the 6-min walk test. The patients were also examined by the pulmonary function test for forced vital capacity and diffusing capacity for carbon monoxide (DLCO), and by echocardiography for left ventricular ejection fraction and right ventricular systolic pressure. Interstitial lung disease was diagnosed in 19 patients using high-resolution computed tomography. There was no difference in 6-min walk distance or cardiac output after walking between the patients and healthy controls. However, stroke volume during the 6-min walk test was significantly lower in the patients than in healthy controls, suggesting malfunction in the heart. Moreover, the increased heart rate matched the cardiac output. Spearman's correlation analysis demonstrated a correlation between 6-min walk distance and stroke volume, cardiac output after walking and DLCO, but not left ventricular ejection fraction or right ventricular systolic pressure, as this study lacked the patients with pulmonary hypertension. In conclusion, impaired DLCO due to interstitial lung disease was suggested to be a fundamental parameter affecting exercise capacity, in addition to heart involvement, in patients with idiopathic inflammatory myopathies.

Objective: The present study examined the association between early rehabilitation for mechanically ventilated intensive care unit (ICU) patients and oral ingestion., Method: Among 1055 consecutive patients who were transported to the study facility via ambulance, newly admitted to the ICU, and treated with rehabilitation during hospitalization, 234 were included in the current study. The patients were allocated to early rehabilitation and control groups to retrospectively examine the proportion able to orally ingest three meals per day, the period needed to achieve such independence, and course-related factors., Results: A total of 77 matched pairs were selected using propensity score matching. Analysis using the Kaplan-Meier estimator revealed that the early rehabilitation group needed a markedly shorter period to achieve oral ingestion of three meals per day. There were significant differences between the groups in the periods from hospital admission to first physical therapy and to mobilization as well as differences in the frequency of delirium., Conclusion: Early rehabilitation for mechanically ventilated ICU patients may facilitate earlier mobilization. It may also shorten the period needed to achieve oral ingestion of three meals per day by preventing complications such as delirium., Competing Interests: CONFLICT OF INTEREST: The authors declare that there are no conflicts of interest., (©2018 The Japanese Association of Rehabilitation Medicine.)

The chronic obstructive pulmonary disease (COPD) Assessment Test (CAT), which was developed to measure the health status of patients with COPD, was applied to patients with interstitial lung disease, aiming to examine the CAT as a predictor of outcome. Over a follow-up period of more than one year, 101 consecutive patients with interstitial lung disease were evaluated by the CAT. The CAT scores of 40 in total were categorized into four subsets according to the severity. Patients with higher (more severe) scores exhibited lower forced vital capacity and lung diffusion capacity for carbon monoxide. The survival rate was significantly lower in patients with higher scores (log-rank test, P = 0.0002), and the hazard ratios for death of the higher scores and lower lung diffusion capacity for carbon monoxide were independently significant. These findings suggest that CAT can indicate the risk of mortality in patients with interstitial lung disease., Competing Interests: Authors disclose no potential conflicts of interest.

Objectives: Systemic sclerosis impairs the dilatation of affected pulmonary blood vessels and myocardial diastolic function at rest, but few studies have examined cardiac hemodynamic response during exercise. This study aimed to evaluate the factors affecting cardiac response during submaximal exercise in patients with systemic sclerosis., Methods: Fifty-nine consecutive patients and 27 age- and sex-matched healthy controls underwent the 6-minute walk test with a non-invasive impedance cardiograph device., Results: Stroke volume and cardiac output in patients with systemic sclerosis were significantly lower than those in controls at rest and at the end of the 6-minute walk test, and the distance walked was significantly shorter in patients. Percent predicted of forced vital capacity and diffusion capacity of the lung in patients showed correlations with stroke volume at the end of the 6-minute walk test and the increase in stroke volume during walking. The echocardiographic findings of right ventricular systolic pressure and left ventricular diastolic dysfunction showed no relationship to stroke volume during the 6-minute walk test. The increase in stroke volume during the 6-minute walk test was significantly smaller in patients with pulmonary hypertension diagnosed by right-heart catheterization than in those without pulmonary hypertension., Conclusions: Impaired stroke volume in patients with systemic sclerosis was observed at rest and during exercise, and the factors relating to the cardiac response seemed to be pulmonary function and the extent of pulmonary hypertension.

Objective: Dysphagia develops with low frequency in patients with dermatomyositis. Our objective was to determine the clinical and laboratory features that can estimate the development of dysphagia in dermatomyositis., Methods: This study included 92 Japanese patients with adult-onset dermatomyositis. The associations between dysphagia and clinical and laboratory features including disease-specific autoantibodies determined by immunoprecipitation assays were analyzed., Results: Videofluoroscopy swallow study (VFSS) was performed for all patients with clinical dysphagia (n = 13, 14.1%) but not for patients without clinical dysphagia. Typical findings of dysphagia (pharyngeal pooling, n = 11 and/or nasal regurgitation, n = 4) was detected by VFSS in all patients with clinical dysphagia. Eleven patients with dysphagia (84.6%) had anti-transcription intermediary factor 1γ (TIF-1γ) antibody. By univariate analysis, the average age and the male to female ratio, internal malignancy, and anti-TIF-1γ antibody were significantly higher and the frequency of interstitial lung diseases and manual muscle testing (MMT) scores of sternomastoid and dertoid muscles were significantly lower in patients with dysphagia than in patients without dysphagia. Among patients with anti-TIF-1γ antibody, the mean age, the ratios of male to female and internal malignancy were significantly higher and mean MMT scores of sternomastoid muscle were significantly lower in patients with dysphagia compared with patients without dysphagia. By multivariable analysis, the risk of dysphagia was strongly associated with the existence of internal malignancy and ant-TIF-1γ antibody and was also associated with reduced scores of manual muscle test of sternomastoid muscle. Dysphagia was markedly improved after the treatment against myositis in all 13 patients., Conclusion: These findings indicate that dysphagia can develop frequently in patients with internal malignancy, anti-TIF-1γ antibody, or severe muscle weakness of sternomastoid muscle.

[Purpose] Lateral ankle sprains are common injuries suffered while playing sports, and abnormal forward- and inward-directed ground reaction force occurs during a jumping task. However, the influence of hip muscle strength training on jumping performance after ankle injuries has not been fully examined. This study thus examined changes in ground reaction force during a rebound-jump task after training to strengthen hip muscles. [Subjects and Methods] Ten of 30 female high school basketball players were assigned as subjects who showed a difference of 7 or more degrees in dorsiflexion ranges between the bilateral ankles. The subjects underwent 12 weeks of training to strengthen hip abductors and external rotators. Comparisons between before and after training were made regarding ground reaction force components, hip and knee joint angles, percentage of maximum voluntary contraction in leg muscles, and muscle strength of hip muscles during the rebound-jump task. [Results] After training, the subjects showed increased strength of external rotator muscles, increased percentage of maximum voluntary contraction in the gluteus medius muscle, decreased inward ground reaction force, and increased flexion angles of the hip and knee joints. [Conclusion] This study suggests that training to strengthen hip muscles may ameliorate the inward ground reaction force in athletes with ankle dorsiflexion restriction.

Background: Exercise capacity is evaluated using the 6-minute walk test in various diseases. Variety in the distances walked was also shown in healthy subjects. Moreover, age-related influences on cardiac hemodynamic response to the 6-minute walk test have not been clarified. The purpose of this study was thus to investigate the hemodynamic response to the 6-minute walk test and to detect factors related to the distance walked in healthy subjects., Methods: Thirteen young adults (age 20.5 ± 0.7 years, BMI 22.0 ± 4.3) and 26 elderly individuals (age 60.2 ± 6.1 years, BMI 21.7 ± 2.2) were enrolled to measure real-time hemodynamic responses using non-invasive impedance cardiography during the 6-minute walk test., Results: Stroke volume was higher in the young than in the elderly and reached a plateau within 30 s of starting to walk in all subjects. An increase in heart rate took more than 1 min in the elderly, while it took less than 30 s in the young, which resulted in slower increases in cardiac output and cardiac index in the elderly. There was no difference in the distance in the 6-minute walk test between the young and the elderly. The distance walked was correlated with heart rate, cardiac output, and cardiac index, but not with stroke volume, at the end of the 6-minute walk test., Conclusions: The distance walked appeared to depend on increased cardiac output based on heart rate, but did not appear to be limited by stroke volume, in healthy subjects.

[Purpose] To clarify seasonal changes in activity levels among nursing care insurance service users in areas with different climates using the Life Space Assessment. [Subjects] A total of 72 nursing care insurance service users aged ≥65 years, who were from areas along the Sea of Japan or those around the Inland Sea. [Methods] The subjects were divided into 2 groups according to their home prefecture, and each survey was conducted over two successive seasons (Survey I: fall and winter, n=48, Survey II: winter and spring, n=24). We investigated the subjects' basic information, and determined their FIM, the Life Space Assessment, and Modified Falls Efficacy Scale scores. These scores were subjected to between-group and -season comparisons. [Results] In Survey I, there were no significant differences in any investigation item between the 2 groups, but the Japan Sea group showed decreases in the Life Space Assessment, Independent Life space, and Minimal Life space scores in winter. In Survey II, we did not note any between-group or -season differences. [Conclusion] Our findings suggest that the Life Space Assessment, whose scores are influenced by outdoor environments, may be used as a tool to clarify seasonal changes in activity levels of nursing care insurance service users.

[Purpose] To investigate the therapeutic usefulness of treadmill walking using a body weight support device (BWS), changes in circulatory dynamics and muscle activities with various levels of support were investigated. [Subjects and Methods] The subjects were divided into 3 groups: 20% BWS, 40% BWS, and full body weight (FBW). The subjects walked at maximum and normal speeds. Under each condition, H and M waves and skin temperature before and after walking and changes in the heart rate during walking were measured. [Results] The heart rate continued to increase after 3 minutes of FBW at the maximum walking speed, but a steady state was reached after 3 minutes under the other walking conditions. Regarding skin temperature, no significant difference from that at rest was noted 30 minutes after walking at the normal speed, but it was significantly higher than that at rest at 30 minutes after walking at the maximum speed. The H/M ratio was significantly higher after walking at the maximum walking speed in the FBW and 20% BWS groups compared with the 40% BWS groups. [Conclusion] Treatment with 40% BWS at the maximum walking speed was safe for the circulatory system and may be effective in elevating the skin temperature for a prolonged period compared with the effects of the other walking conditions at normal speed.

Background: The diffusion capacity of the lung for carbon monoxide (DLCO) is a good marker of disease severity in patients with idiopathic interstitial pneumonia, and is associated with oxygen saturation; however, little is known about DLCO in systemic sclerosis patients with interstitial lung disease. We studied potential predictors of exercise-induced oxygen desaturation in patients with systemic sclerosis., Methods: Data were collected prospectively from 80 of 110 consecutive systemic sclerosis patients with normal oxygen saturation (> 95%) at rest, who could perform the 6-min walk test without physical discomfort, including leg pain. Pulmonary function tests and echocardiography were collected from all subjects., Results: Thirty subjects showed a ≥ 4% decline in oxygen saturation during the 6-min walk test (desaturation group). The other subjects were assigned to the normoxic group. The percent-of-predicted values for FVC, FEV1, total lung capacity, DLCO, and DLCO/alveolar volume were lower, and FEV1/FVC was higher, in the desaturation group. Logistic regression analysis showed the percent-of-predicted DLCO as a highly accurate predictor of exercise-induced oxygen desaturation: the area under the receiver operating characteristic curve was 0.92 (cutoff point 56.3%, sensitivity 0.83, specificity 0.86). Five subjects over the cutoff point of the percent-of-predicted DLCO in the desaturation group could not be distinguished from the normoxic subjects with the lung-volume measurements or right-ventricular systolic pressure., Conclusions: The factor underlying exercise-induced oxygen desaturation appeared to be reduced percent-of-predicted DLCO, which was useful as a predictor in over 80% of the subjects.

Autoantibodies have been detected in systemic sclerosis patients, and typical clinical features regarding organ involvement by each autoantibody have been reported. To reveal differences in exercise intolerance in patients with either anti-topoisomerase-I or anti-centromere antibodies, 53 systemic sclerosis patients were investigated retrospectively. A 6-min walking distance showed no significant differences (P = 0.090) between autoantibodies, while exercise-induced hypoxia during the 6-min walking test was significant in subjects with the anti-topoisomerase-I antibody (P = 0.033). The percent predicted of vital capacity, the diffusion capacity of the lung for carbon monoxide, and the modified Rodnan skin score were affected more in subjects with the anti-topoisomerase-I antibody than the anti-centromere antibody. The main parameter affecting the 6-min walking distance was the percent predicted of vital capacity for each autoantibody, and there was a significant positive relationship for all subjects (R (2) = 0.30, P < 0.0001). Exercise-induced hypoxia was also shown in the more affected subjects in the percent predicted of vital capacity and the diffusion capacity of the lung for carbon monoxide. Lung parameters were suggested to be more important factors determining exercise intolerance and induced hypoxia than detected autoantibodies.

Objective: To compare the effect of body-weight-supported treadmill training (BWSTT) and full-body-weight treadmill training (FBWTT) on patients with knee osteoarthritis (OA)., Methods: Design was Randomized controlled trial. Patients with knee osteoarthritis (n = 30; mean age, 76.0±7.5 y) were randomly assigned to BWSTT or FBWTT group. All patients performed 20 min walking exercise twice a week for 6 weeks under the supervision of the therapist. Main measures were 10-meter walking test (10MWT), functional reach test (FRT), timed get up and go test (TUG), one-leg standing test, 6-minute walking test (6MWT), the parameters set on the treadmill, MOS Short-Form 36-Item Health Survey (SF36), Japanese Knee Osteoarthritis Measure (JKOM)., Results: Twenty-five patients (10 men, 15 women; mean age, 76.5 ± 8.0 y) completed the experiment. Exercise capacity, indicated by the heart rate, was similar in both groups. After 3 weeks of BWSTT, the patients performed significantly better in the 10-m and 6-min walking tests. This was not the case with FBWTT even after 6 weeks training. Pain levels assessed were significantly improved after 3 weeks of BWSTT and 6 weeks of FBWTT. There were no significant improvements in either group assessed by the FRT, one-leg standing time test, TUG, or SF -36 questionnaire., Conclusions: BWSTT enhanced exercise capacity in terms of walking speed and pain reduction after 3 weeks; however, there was no significant improvement in patients' functional abilities or quality of life.

Although the relationship between muscle strength and exercise capacity has been demonstrated in dermatomyositis without lung dysfunction, little is known about the association between exercise capacity and interstitial lung disease in dermatomyositis. Eleven patients with dermatomyositis with interstitial lung disease without the manifestation of muscle weakness and 12 patients with idiopathic interstitial pneumonia underwent the 6-minute walk test (6MWT). PaO(2), creatine kinase, percentage predicted 6MWT distance (6MWD%), and SpO(2) at rest were similar between patients. Percentage predicted vital capacity, carbon monoxide diffusing capacity (DL(CO)%), and SpO(2) after 6MWT were higher and exertional dyspnea was lower in patients with dermatomyositis than in patients with idiopathic interstitial pneumonia. SpO(2) after 6MWT was positively correlated with 6MWD% in patients with dermatomyositis, while DL(CO)% and PaO(2) were positively correlated with 6MWD% in patients with idiopathic interstitial pneumonia. Lung dysfunction in dermatomyositis might not be a major limitation factor in exercise capacity.

More prescription drugs are dispensed by pharmacists rather than doctors in response to government healthcare reform. However, the results of our preliminary survey suggested a hypothesis that patients were not always satisfied with explanations provided by pharmacists upon dispensing, and therefore patient satisfaction might be enhanced if pharmacists spent more time on consultation services. The survey was conducted in 1,800 people. 321 people (17.8%) answered that pharmacist explanations were not sufficient or that they expected further consultation with pharmacists. The most common reason why they did not ask for consultation was that other patients were waiting (41.7%). If up to 30-minute consultation service was available at a pharmacy where patient privacy was ensured, 898 people (49.9%) answered that they wanted to use the service. The average willingness-to-pay (WTP) for the service was 338 yen and the mean WTP was 400 yen. When those who answered 0 yen were excluded, the average was 386 yen and the mean was 400 yen. The online survey revealed that some patients were not satisfied with pharmacist explanations upon dispensing, and that there was a need for consultation services at pharmacies. Many of the reasons why patients did not ask for consultation although they wanted to were attributable to pharmacies, and the survey results suggested a need for improvement in their services. In view of the WTP for the service, it is considered worthwhile to discuss the introduction of such a system for consultation services into dispensing fee.

The purpose of this study was to test the endurance of the soleus muscle, and to examine the joint position at which it is most active, while simultaneously suppressing the activity of the gastrocnemius. Ten young males performed maximum isometric contraction of the triceps surae for 100 s, and the endurance and plantar flexion torque of this muscle were measured at various angles of the knee and ankle joints. The electromyogram was measured simultaneously and subsequently converted into integrated electromyogram (IEMG) values. With the knee flexed at 130 degrees, the rate of change in IEMG values for the soleus (0.454% x s(-1)) with the ankle in a neutral position was significantly higher than that for the medial and lateral gastrocnemius. Both with the ankle dorsiflexed at 10 degrees and in the neutral position, the rate of change in IEMG for the soleus was significantly higher with the knee flexed at 90 degrees and 130 degrees than with the knee fully extended. With the knee flexed at 90 degrees and 130 degrees, the IEMG activity of the soleus during the initial (5-10 s) and final 5 s tended to be higher than those for the medial and lateral gastrocnemius, regardless of the ankle joint position. We conclude that the position in which the soleus acts most selectively during a sustained maximum isometric contraction of the triceps surae is with the ankle in a neutral position and the knee flexed at 130 degrees.

Rheumatoid arthritis patients have multiple joint problems, often making it difficult to use gait aids after a joint replacement in the leg. To address this problem, we designed a hand splint with a hook on the palmar side for use with parallel bars. Patients put these splints on both hands and they can walk holding the bars with the hooks. Best suited for using this splint are rheumatoid arthritis patients who are unable to hold parallel bars without marked pain in the hands or fingers, contracture of the wrist joint, or dislocation of finger joints. Several patients have tried this splint, which worked safely and satisfactorily during exercise.

We describe the partial characterization and some properties of fibroblast and leucocyte neuraminidase towards 2 leads to 3 and 2 leads to 6 sialyllacose, and 2 leads to 3 and 2 leads to 6 sialylhexasaccharide which were isolated from the urine of a patient with adult sialidosis with partial beta-galactosidase deficiency. Neuraminidase activities were assayed using the radioactive-labeled derivatives of these saccharide substrates. These neuraminidases (acylneuraminyl hydrolase, EC 3.2.1.18) were partially inactivated by homogenization, sonication and freeze-thawing treatment. The leucocyte neuraminidase was more labile than that of fibroblasts. Fibroblast neuraminidase had about a 10-fold higher activity than leucocyte neuraminidase towards the respective substrates. The neuraminidase from fibroblasts and leucocytes were each able to hydrolyze 2 leads to 3 isomers 2-3 times faster than 2 leads to 6 isomers and the sialyllactoses 1.5-3.0-times faster than sialylhexasaccharides. Neuraminidase activities towards all four substrates were deficient in fibroblasts and leucocytes from the patients with adult sialidosis. Loss of activity was especially prominent in fibroblasts, while considerable residual activities (about 20-30%) remaining in leucocytes. In mucolipidosis II and III patients, these neuraminidase activities showed normal levels in leucocytes, although they were decreased in fibroblasts. The discrepancy between neuraminidase activities towards 2 leads to 3 and 2 leads to 6 isomers was not found in all the cases.

We devised a sensitive method to assay for neuraminidase activities towards alpha-(2 leads to 3)-N-acetylneuraminosyl hexasaccharide and alpha-(2 leads to 6)-N-acetylneuraminosyl hexasaccharide, which were isolated from the urine of a patient with adult sialidosis with partial deficiency of beta-galactosidase. Standard assay conditions for the determination of these neuraminidase activities were established and the radiolabeled reduced derivatives of these substrates were used. The fibroblast neuraminidase had its maximum activity at pH 4.0-4.2, with Km values of 2.22 X 10(-3) and 4.17 X 10(-3) mol/l and Vmax values of 76.9 and 28.6 nmol . mg-1 protein . h-1 towards the 2 leads to 3 isomer and the 2 leads to 6 isomer, respectively. Neuraminidase deficiencies were found in the fibroblasts of adult sialidosis, mucolipidosis II and III. These studies were compared with the neuraminidase activity towards alpha-(2 leads to 3)-N-acetylneuraminosyl lactose.