Longitudinal changes in nailfold videocapillaroscopy findings differ by myositis-specific autoantibody in idiopathic inflammatory myopathy.

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.

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

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.

Oropharyngeal Dysphagia in Dermatomyositis: Associations with Clinical and Laboratory Features Including Autoantibodies.

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.

Predictors of exercise-induced oxygen desaturation in systemic sclerosis patients with interstitial lung disease.

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.

Exercise capacity in relation to autoantibodies in systemic sclerosis patients.

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.

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.