Abstract
Objectives: The aim of this study was to determine the relationship between disease activity in adult patients with dermatomyositis (DM) and other biomarkers of disease activity such as C-reactive protein creatinine kinase and nailfold video capillaroscopy (NVC).Methods: We performed a prospective single center study of 15 adult patients with DM. Study participants underwent two assessments at least 9 months apart including clinical, laboratory and NVC evaluations. Patients received immunosuppressive medications for their dermatomyositis, and ongoing disease activity was measured by the Myositis Intention to Treat Index (MITAX). NVC evaluation included assessment of capillary density, capillary apical diameter (mm), and the number of microhemorrhages per digit.Results: Microvascular abnormalities were present in most DM patients. Of these, capillary density (4.71 vs. 6.84, p = 0.006) and mean apical diameter (56.09 vs. 27.79 μm, p = 0.003) significantly improved over the study period in concordance with improving disease control (MITAX 8.53 vs. 2.64, p = 0.002). Longitudinal analysis demonstrated that capillary density was independently associated with MITAX (β = −1.49 [CI −2.49, −0.33], p = 0.013), but not other parameters such as C-reactive protein and creatinine kinase.Conclusions: Nailfold capillary density is a dynamic marker of global disease activity in adult DM. NVC may be utilized as a non-invasive point-of-care tool to monitor disease activity and inform treatment decisions in patients with DM.
Highlights
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by proximal muscle weakness and characteristic cutaneous findings
The diagnosis of DM is based on clinical features, complemented by detection of myositis-specific antibodies (MSAs), elevation in muscle enzymes such as creatinine kinase (CK), muscle biopsy, and/or imaging
Given that small vessel vasculopathies leads to both clinical disease and nailfold capillary abnormalities in DM, it is reasonable to hypothesize that Nailfold video capillaroscopy (NVC) and disease activity may be correlated
Summary
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by proximal muscle weakness and characteristic cutaneous findings. Monitoring response to treatment remains a challenge as no single measure is able to capture global disease activity in DM. The International Myositis Assessment and Clinical Studies Group (IMACS) has developed a core set of disease activity and treatment response criteria for adult DM. In these criteria, monitoring response to treatment is largely clinical, with inclusion of muscle enzyme evaluation as the only biomarker. The relationship between disease activity and muscle enzymes is not firmly established [1] and they are assigned low weighting in treatment response criteria [2].
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