ADABs developed in 13 % of patients. All five TNF inhibitors were associated with ADABs, but to varying degrees depending on the specific TNF inhibitor and the disease. ADABs are associated with reduced clinical response and an increased incidence of infusion reactions and injection site reactions. Concomitant use of immunosuppressives can reduce ADAB formation.

Objective. Survival in patients with systemic sclerosis (SSc)-associated pulmonary hypertension (PH) and interstitial lung disease (ILD) is poor. Evidence supporting the efficacy of aggressive pulmonary arterial hypertension (PAH)-targeted therapy in this population is limited. The aim of this study was to investigate transplant-free survival in patients with isolated SScrelated PAH or SSc-related PH-ILD who were treated with aggressive PAH-targeted therapy.Methods. SSc patients with right-sided heart catheterization (RHC)-diagnosed precapillary PH (mean pulmonary artery pressure >25 mm Hg, pulmonary capillary wedge pressure <15 mm Hg, and pulmonary vascular resistance >240 dynes ؋ second/cm 5 )were included. Patients were classified as having ILD based on review of high-resolution computed tomography (CT) chest imaging and spirometry. The Kaplan-Meier method was applied and Cox proportional hazards models were constructed to analyze survival and identify predictive variables. Results. Of 99 patients with SSc-related precapillary PH, 28% had SSc-related PAH and 72% hadSSc-related PH-ILD. The 1-and 2-year survival estimates were, respectively, 72% and 59% in the SScrelated PH-ILD group versus 82% and 66% in the SSc-related PAH group (P ‫؍‬ 0.5). Within 6 months of the diagnostic RHC, 24% of all patients were started on prostanoid therapy; an additional 24% were started on prostanoid therapy after 6 months. In the multivariate model, male sex (hazard ratio [HR] 0.7, P ‫؍‬ 0.01) and prostanoid therapy initiation within 6 months of the RHC (HR 1.4, P ‫؍‬ 0.01) were the only factors significantly associated with transplant-free survival, after accounting for the presence of ILD and severity of PH.Conclusion. In this study, survival of patients with SSc-related PH-ILD was modestly improved relative to historical series. While these findings may not be generalizable, improved survival may be due partly to aggressive PAH-targeted therapy.

Purpose: There is a lack of a valid, definition for skin ulcers in SSc to be used in clinical trials. Our aim was to develop a consensus definition for SSc-skin ulcers based on the results of a systematic literature review (SLR) for skin ulcer definitions and expert opinion; and to evaluate its face validity, reliability and feasibility. Methods: SLR for skin ulcer definitions was conducted using PubMed, Web of Science, and Cochrane library for articles published from inception to January 1st, 2016. SSc experts were to discuss the definitions’ categories and vote for the relevant terms. Reliability of the definition were tested in a second expert meeting, seven SSc experts evaluated 7 SSc pts with skin lesions twice. Face validity and feasibility evaluated by sending out case report forms(CRFs) to 4 SSc experts, they were asked to use the definition in 5 pts each. Results: A total of 3464 abstracts and titles were screened, and 446 articles were fully evaluated. Of these, 66 met eligibility criteria and skin ulcer definitions were extracted. SSc experts discussed, refined and voted on the consensus definition using nominal process. Kappa for inter-, intra-rater rater agreement was 0.51, 0.90 respectively. The mean time to decide if the lesion is an ulcer was 7.4 sec. All investigators endorsed the face validity of the new definition in the CRFs. Conclusion: Using a SLR and a nominal technique, we developed a preliminary consensus-based definition of SSc-skin ulcers. Face validity, feasibility and reliability were demonstrated for the developed definition.

Introduction Both arterial and venous thrombotic events of the extremities occur in COVID-19 infection, but the etiology of these events remains unclear. This study sought to evaluate pathology specimens of COVID-19 positive patients post-amputation, who were found to have Rutherford 3 acute limb ischemia requiring amputation. Methods A retrospective review was performed of all vascular surgery emergency room and inpatient consultations in patients who presented to the Mount Sinai Health System from March 26, 2020 to May 10, 2020. Pathology specimens were examined using hematoxylin and eosin (H&E) stain. The specimens were assessed for the following: inflammatory cells associated with endothelium/apoptotic bodies, mononuclear cells, small vessel congestion, and lymphocytic endotheliitis. Of the specimens evaluated, two patients with a known history of peripheral vascular disease were excluded. Results Inflammatory cells associated with endothelium/apoptotic bodies were seen in all four patients and in four out of five specimens. Mononuclear cells were found in two out of four patients. Small vessel congestion was seen in all patients. Lymphocytic endotheliitis was seen in one out of four patients. Conclusion This study shows endotheliitis in amputation specimens of four patients with COVID-19 disease and Rutherford Class 3 acute limb ischemia. The findings in these patients is more likely an infectious angiitis due to COVID-19.

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