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Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process.

Objectives Chronic glucocorticoid use is complicated by osteoporosis and increases the risk of fragility fractures. EULAR guidelines on SLE management recommend reducing chronic glucocorticoid dosage to ≤7.5 mg/day to minimize this risk. We examined the relationship of glucocorticoid dose to fragility fracture risk in a cohort of SLE patients. Methods Retrospective analysis of SLE patients attending University College Hospital over 28 years was undertaken. Collected data included consecutive steroid dose, dual-energy X-ray absorptiometry scans and fragility fractures. Results We collected data on 250 patients with a median of 17 years’ follow-up. Fragility fractures were diagnosed in 28 (11.2%) patients and the mean ± s.d. age of first fracture was 51 ± 16 years. A total of 94% received glucocorticoids, the average dose being 6.20 mg/day. Patients with fragility fractures had a lower average daily dose (5.36 vs 6.23 mg/day) but a higher median cumulative dose (25.19 vs 20.96 g). These differences were not significant (P = 0.127 and 0.229, respectively). Some 93% of patients received vitamin D, and 85% received calcium. Cox regression analysis showed older age at SLE diagnosis, osteoporosis and secondary hyperparathyroidism were associated with fragility fractures. Glucocorticoid dose was not significantly associated with the occurrence of fragility fractures. Twenty-two patients with fractures were treated with bisphosphonates, two with denosumab and two with teriparatide. Conclusions We found no significant association between glucocorticoid treatment and fragility fractures in our group of patients; however, a prospective study including more patients not treated with CS would be necessary to confirm these results.

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BackgroundSystemic lupus erythematosus (SLE) is associated with an increased cardiovascular risk. Several traditional and disease-specific risk factors have been shown to correlate with the occurrence of cardiovascular events (CVE) in patients with SLE. However, results of previous studies are diverse.ObjectivesThe objectives of this study were to report number, type and those factors associated with CVE in patients with SLE in a large, single-centre, ethnically diverse cohort with a long follow-up duration.MethodsMedical records of patients treated at the Lupus Clinic at University College London Hospital (UCLH) between 1979 and 2020 were retrospectively reviewed. Data about CVE, traditional cardiovascular risk factors, demographic and disease features, and treatment history were collected. Only patients with complete available information were included in the study. Regression analyses were performed to identify factors associated with CVE.ResultsFour hundred and nineteen patients were included in the study. Maximum follow-up length was 40 years. Seventy-one (17%) patients had at least one CVE. Forty (50%) events were VTE, 26 (32.5%) were CVA, and 14 (17.5%) were CAD. Mean age at CVE was 44 (SD 12) years. Median time from diagnosis to CVE was 8 (IQR 4-15) years. Descriptive comparative analysis showed an increased prevalence of hypercholesterolemia (41% vs 28%, p-value=0.035), diabetes (10% vs 4%, p-value=0.027), and antiphospholipid antibodies (aPL) positivity (58% vs 27%, p-value<0.001) among patients with CVE. Furthermore, these patients were treated less frequently with hydroxychloroquine (80% vs 89%, p-value=0.049). However, univariable analysis (Table 1) showed that only aPL positivity was significantly associated with CVE. This association was confirmed at multivariable analysis (p<0.001). Survival curve for CVE according to the presence of aPL is shown inFigure 1. When analysing different types of CVE, aPL were specifically associated with both venous thromboembolic events (p-value<0.001) and cerebrovascular events (p-value=0.007). Dedicated subanalyses revealed that cumulative glucocorticoid dose (p-value=0.010) and a diagnosis of SLE before 2000 (p-value<0.001) were also significantly associated with CVE.ConclusionCardiovascular disease is highly prevalent among patients with SLE and is associated with aPL, glucocorticoid therapy, and diagnosis before 2000.Figure 1.Survival curves for cardiovascular events according to the presence of antiphospholipid antibodies.aPL, antiphospholipid antibodies.Table 1.Univariable Cox regression analysis to evaluate the association between independent variables and cardiovascular events in the whole study population.CovariateHazard Ratio95% Confidence Intervalp-valueAge at diagnosis1.0120.994 – 1.0310.178Female sex0.6190.307 – 1.2500.182Caucasian ethnicity1.3790.834 – 2.2810.210Raised BMI (>25kg/m2)0.9480.579 – 1.5530.834Ever smoker1.1770.713 – 1.9420.522Hypertension1.0980.670 – 1.8010.708Hypercholesterolemia1.4960.921 – 2.4290.103Diabetes2.1650.988 – 4.7460.054Lupus nephritis1.1320.693 – 1.8470.619adsDNA positivity1.2080.724 – 2.0140.468Decreased C31.2170.755 – 1.9600.419aPL positivity3.2051.975 – 5.201< 0.001Use of hydroxychloroquine0.6320.350 – 1.1430.129SDI0.9860.788 – 1.2350.907aPL, antiphospholipid antibodies; BMI, body mass index; SDI, Systemic Lupus International Collaborating Clinics Damage Index score.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.