Functional impairment of systemic scleroderma patients with digital ulcerations: results from the DUO Registry

OBJECTIVES: Digital ulcers (DUs) are frequent manifestations of systemic scleroderma (SSc). This study assessed functional limitations due to DUs among patients enrolled in the Digital Ulcer Outcome (DUO) Registry, an international, multicentre, observational registry of SSc patients with DU disease. METHODS: Patients completed at enrolment a DU-specific functional assessment questionnaire with a 1-month recall period, measuring impairment in work and daily activities, and hours of help needed from others. Physician-reported clinical parameters were used to describe the population. For patients who completed at least part of the questionnaire, descriptive analyses were performed for overall results, and stratified by number of DUs at enrolment. RESULTS: This study included 2327 patients who completed at least part of the questionnaire. For patients with 0, 1–2, and ≥3 DUs at enrolment, mean overall work impairment during the prior month among employed/self-employed patients was 28%, 42%, and 48%, respectively. Across all included patients, ability to perform daily activities was impaired on average by 35%, 54%, and 63%, respectively. Patients required a mean of 2.0, 8.7, and 8.8 hours of paid help and 17.0, 35.9, and 63.7 hours of unpaid help, respectively, due to DUs in the prior month. Patients with DUs had more complications and medication use than patients with no DUs. CONCLUSIONS: With increasing number of DUs, SSc patients reported more impairment in work and daily activities and required more support from others

Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry

OBJECTIVES: The Digital Ulcers Outcome (DUO) Registry was designed to describe the clinical and antibody characteristics, disease course and outcomes of patients with digital ulcers associated with systemic sclerosis (SSc). METHODS: The DUO Registry is a European, prospective, multicentre, observational, registry of SSc patients with ongoing digital ulcer disease, irrespective of treatment regimen. Data collected included demographics, SSc duration, SSc subset, internal organ manifestations, autoantibodies, previous and ongoing interventions and complications related to digital ulcers. RESULTS: Up to 19 November 2010 a total of 2439 patients had enrolled into the registry. Most were classified as either limited cutaneous SSc (lcSSc; 52.2%) or diffuse cutaneous SSc (dcSSc; 36.9%). Digital ulcers developed earlier in patients with dcSSc compared with lcSSc. Almost all patients (95.7%) tested positive for antinuclear antibodies, 45.2% for anti-scleroderma-70 and 43.6% for anticentromere antibodies (ACA). The first digital ulcer in the anti-scleroderma-70-positive patient cohort occurred approximately 5 years earlier than the ACA-positive patient group. CONCLUSIONS: This study provides data from a large cohort of SSc patients with a history of digital ulcers. The early occurrence and high frequency of digital ulcer complications are especially seen in patients with dcSSc and/or anti-scleroderma-70 antibodies

Psoriasis induced by anti-tumor necrosis factor therapy - A paradoxical adverse reaction

Administration of anti-tumor necrosis factor (anti-TNF) agents is beneficial in a variety of chronic inflammatory conditions, including psoriasis. We describe 5 patients in whom psoriasiform skin lesions developed 6-9 months after the initiation of anti-TNF therapy for longstanding, seropositive rheumatoid arthritis (etanercept or adalimumab), typical ankylosing spondylitis (infliximab), and Adamantiades-Behcet’s disease (infliximab). In all 5 patients, the underlying disease had responded well to anti-TNF therapy. Four patients developed a striking pustular eruption on the palms and/or soles accompanied by plaque-type psoriasis at other skin sites, while 1 patient developed thick erythematous scaly plaques localized to the scalp. In 3 patients there was nail involvement with onycholysis, yellow discoloration, and subungual keratosis. Histologic findings from skin biopsies were consistent with psoriasis. None of these patients had a personal or family history of psoriasis. In all patients, skin lesions subsided either with topical treatment alone, or after discontinuation of the responsible anti-TNF agent. The interpretation of this paradoxical side effect of anti-TNF therapy remains unclear but may relate to altered immunity induced by the inhibition of TNF activity in predisposed individuals

AB0169 ENHANCED PLATELET ACTIVATION AMONG PATIENTS WITH SYSTEMIC SCLEROSIS

Background:The function of platelets (PLT), which can serve as reservoirs of transforming growth beta, leads to the question whether they are activated in systemic sclerosis (SSc) to such an extent as to associate with clinical severity.Objectives:To compare the level of PLT activation in SSc and to correlate with physical findingsMethods:Blood was sampled from 12 patients participating in the randomized clinical trial LIGHT (ClinicalTrials.gov NCT040045743) evaluating the safety and efficacy of bermekimab in SSc. Patients had to meet the 2013 ACR/EULAR classification criteria and have a modified Rodnan skin score (mRSS) between 15 and 40. Sampling was done before allocation to treatment. Nil patient was on DMARDs. Ten healthy donors were used for comparison. Citrated whole blood was exposed to 0.1 mM adenosine diphosphate (ADP) to stimulate PLT. Total platelet populations were defined as CD42b-positive population; P-selectin (CD62p) served as an activation marker.Results:Expression of 42b(+)/62p(+) PLT was greater among patients (Figure 1). Mean fluorescence intensity (MFI) of 42b(+)/62p(+) PLTs was 0.16 +/- 0.08 in comparators and 0.51 +/- 0.07 in patients (p= 0.015). Negative correlation was found between the expression of 42b(+)/62p(+) on PLT and mRSS (r= -0.73, p= 0.028). Patients with grade 2 findings at capillaroscopy had greater expression of 42b(+)/62p(+) than patients with grade 3 findings (45.9 +/- 7.2 % vs 27.2 +/- 6.7 %; p: 0.041).Conclusion:PLTs are greatly activated in SSc and this is associated with disease progression. Findings suggest that this activation is greater at less severe patients.References:[1]Morel A, Rywaniak J, Bijak M, Miller E, Niwald M, Saluk J. Flow cytometric analysis reveals the high levels of platelet activation parameters in circulation of multiple sclerosis patients Mol Cell Biochem. 2017;430:69-80. doi: 10.1007/s11010-017-2955-7Disclosure of Interests:None declared</jats:sec