Abstract
BackgroundSystemic sclerosis is a rare connective tissue disorder characterized by a triad of inflammation, vasculopathy, and fibrosis. Vasculopathic disease manifestations occur early in disease development and include Raynaud’s phenomenon, acral ulcers, necrosis, and pulmonary arterial hypertension. Currently, acral vasculopathy is assessed in clinical practice by nailfold capillary microscopy. However, nailfold capillary microscopy is limited to the nailfold and cannot assess the deeper vascular plexus, changes in dynamic perfusion, or inflammatory and fibrotic disease manifestations. These limitations do not occur with dynamic optical coherence tomography (D-OCT). D-OCT is an imaging technique based on an infrared laser. It allows simultaneous three-dimensional, high-resolution imaging of fibrotic and inflammatory changes as well as vasculopathy in systemic sclerosis [1].ObjectivesThe aim of this study was to characterize vascular alterations in the skin of patients with systemic sclerosis and to assess dynamic changes in skin perfusion under vasoactive therapies.MethodsTwenty-six patients diagnosed with systemic sclerosis according to ACR/EULAR classification criteria 2013 were included in this study. Patients diagnosed with systemic sclerosis received a treatment with iloprost infusion for a duration of five days. Alterations in dynamic perfusion were assessed daily in six locations (nailfold, finger, hand, lower arm, upper arm, ear) using VivoSight OCT. Moreover, alterations in dynamic skin perfusion were assessed under topical treatment with diltiazem (n=13) as well as warmth (n=17). Dynamic perfusion was characterized by the parameters vessel density and vessel diameter. In addition, patients filled questionnaires to assess the severity of vasculopathic disease symptoms and to assess subjective treatment response to iloprost infusion.ResultsPatients were mainly female (73.1%) and of a mean age of 53.6 ± 12.6 years. The majority of the patients were characterized as limited cutaneous systemic sclerosis (76.9%). Seven patients had digital ulcers at the time of study inclusion. Moreover, patients reported that Raynaud’s phenomenon occurred with a median frequency of 3.5 attacks per day and the mean duration of Raynaud’s phenomenon was 36.9 minutes per day. On a visual analogue scale ranging from 0 to 100, patients rated limitations in daily life due to Raynaud’s phenomenon with 48.3 ± 37.3. Whereas the majority of patients (83.3%) reported a subjective benefit, 8.3% reported no change and 8.3% of patients a worsening of acral perfusion under treatment with iloprost infusions.Wilcoxon matched pairs signed rank test did not reveal a significant increase in vessel diameter (p=0.4609) and vessel density (p=0.0681) after topical application of diltiazem. Application of warmth led to a significant increase in vessel diameter (p < 0.0001) and vessel density (p < 0.0001). Interestingly, therapeutic efficacy assessed by increase in vessel diameter and vessel density differed markedly between individual patients. Prospective assessment of vessel diameter and vessel density under therapy with iloprost infusions yielded heterogeneous regarding the therapeutic efficacy at different sites and timepoints for individual patients.ConclusionD-OCT might be a valuable tool to assess vasculopathic alterations and response to vasoactive therapies in the skin of patients with systemic sclerosis. Further studies are required to investigate whether D-OCT might be a technology enabling individual vasoactive treatment in systemic sclerosis in the direction of precision medicine.Reference[1]Ha L, Hundt JE. Optical coherence tomography for fast bedside imaging, assessment and monitoring of autoimmune inflammatory skin diseases?J Dtsch Dermatol Ges(2020) 18:937–942. doi: 10.1111/ddg.14266Figure 1.Linear regression analysis revealed a reduced therapeutic efficacy in patients with low baseline vessel density.Acknowledgements:NIL.Disclosure of InterestsHanna Grasshoff Speakers bureau: Abbvie, Katja Adriany: None declared, Linh Ha-Wissel: None declared, Tanja Lange: None declared, Jennifer Hundt: None declared, Gabriela Riemekasten: None declared.
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