Fujiko Someya

Association between nail-fold capillary findings and disease activity in dermatomyositis

OBJECTIVE Although findings of nail-fold capillary changes and reduced red blood cell velocity in SSc patients are well established, studies in adult-onset DM patients are scarce. Our objective was to assess the changes and red blood cell velocity in finger nail-fold capillaries using nail-fold video capillaroscopy (NVC) in patients with adult-onset DM. METHODS This study included 50 patients with adult-onset DM and 20 healthy subjects. A semi-quantitative rating scale was used to score capillaroscopy changes. Red blood cell velocity was evaluated using frame-to-frame determination of the position of capillary plasma gaps. RESULTS Thirty-seven (74%) patients showed the scleroderma NVC pattern. Patients with the scleroderma pattern exhibited elevated serum creatine kinase levels more frequently and increased visual analogue scale of muscle disease activity. Scores of loss of capillaries were associated with muscle and global disease activity, whereas scores of haemorrhages were associated with skin disease activity. However, NVC findings were not significantly associated with lung involvement. The scores of irregularly enlarged capillaries, haemorrhages and loss of capillaries were reduced after stabilization of disease activity by treatment. The mean red blood cell velocity was not significantly reduced in DM patients compared with healthy controls and was not changed by treatment. CONCLUSION Our results suggest that changes in nail-fold capillaries reflect disease activity in DM. Furthermore, the differences found in red blood cell velocity may reflect somewhat distinct microcirculation injuries in DM and SSc.

Reduced hypoxia risk in a systemic sclerosis patient with interstitial lung disease after long-term pulmonary rehabilitation

Pulmonary rehabilitation is effective for improving exercise capacity in patients with interstitial lung disease (ILD), and most programs last about 8 weeks. A 43-year-old male patient with systemic sclerosis and oxygen saturation (SpO2) declining because of severe ILD was hospitalized for treatment of chronic skin ulcers. During admission, he completed a 27-week walking exercise program with SpO2 monitoring. Consequently, continuous walking distance without severe hypoxia (SpO2 > 90%) increased from 60 m to 300 m after the program, although his six-minute walking distance remained the same. This suggests that walking exercise for several months may reduce the risk of hypoxia in patients with ILD, even though exercise capacity does not improve.

Limitations to the 6-minute walk test in dermatomyositis with interstitial lung disease in comparison with idiopathic interstitial pneumonia.

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). PaO2, creatine kinase, percentage predicted 6MWT distance (6MWD%), and SpO2 at rest were similar between patients. Percentage predicted vital capacity, carbon monoxide diffusing capacity (DLCO%), and SpO2 after 6MWT were higher and exertional dyspnea was lower in patients with dermatomyositis than in patients with idiopathic interstitial pneumonia. SpO2 after 6MWT was positively correlated with 6MWD% in patients with dermatomyositis, while DLCO% and PaO2 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.

The COPD Assessment Test as a Prognostic Marker in Interstitial Lung Disease

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.

Seasonal changes in activity levels among nursing care insurance service users in areas with different climates.

[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.

Cardiopulmonary factors affecting 6-min walk distance in patients with idiopathic inflammatory myopathies

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.

Long-term follow-up of finger passive range of motion in Japanese systemic sclerosis patients treated with self-administered stretching

Abstract 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.

Characteristics for Quality of Life during the Clinical Course of Interstitial Lung Disease

Objective: Temporary improvement in health status after available therapy or medication has been reported for interstitial lung disease. However, interstitial lung disease is progressive, and there is no study on the change in quality of life over time. Therefore, we examined the time course of the Chronic obstructive pulmonary disease Assessment Test (CAT) score in relation with pulmonary function and exercise capacity.Methods: Out of 162 subjects with interstitial lung disease, 56 subjects evaluated more than twice by the CAT were assigned retrospectively to the study and divided into two groups according to the duration from disease onset. The percent of predicted forced vital capacity and diffusion capacity of the lung for carbon monoxide, and 6-min walk distance were also collected from the hospital records for analysis.Results: The CAT score improved in 21 subjects with less than five years of disease (15 to 7, p=0.004), but declined in 35 subjects with five or more years (12 to 17, p=0.002). The intervals of evaluation were not different between the groups (21 and 20 months on average, p=0.80). Forced vital capacity and diffusion capacity of the lung for carbon monoxide at baseline were more impaired in the subjects with longer disease history (p=0.001 and p=0.006, respectively). The CAT score and diffusion capacity of the lung for carbon monoxide or 6-min walk distance were correlated in each group at the baseline. Pulmonary function declined on repeated evaluation even in subjects with less than five years of disease history.Conclusion: Quality of life might be improved within five years of disease regardless of deterioration in pulmonary function; however, the improvement would become difficult afterward due to progressive change in pulmonary function or exercise capacity in interstitial lung disease.