In the past years, the peripheral nervous system involvement in systemic lupus erythematosus (PNSLE) has received little attention despite its potentially significant impact on disease outcome.Objectives. To assess the prevalence and clinical features of PNS involvement in a large cohort of Systemic Lupus Erythematosus (SLE) patients.Methods. SLE patients consecutively observed at two tertiary referral centres over a period of 14 years (from 2000 to 2014) were selected. PNS manifestations were ascertained according to the 1999 American College of Rheumatology case definitions and by using an attribution algorithm for neuropsychiatric (NP) events.Prevalence of PNSLE, demographic, clinical and laboratory data were assessed. Patients with PNS manifestations were compared with a control group of SLE patients without PNS involvement.Results. In a retrospective cohort of 1,224 patients, the overall prevalence of PNS involvement was 6.9% (85 patients, 95% Confidence Interval (95%CI) 0.06-0.08), with 68% of the events attributable to SLE. Polyneuropathy was the most common manifestation observed (42 events, 43.3%), followed by cranial neuropathy in 30 cases (30.9%), 12 singlesingle (12.4%) or multiple (8 events, 8.2%) mononeuritis. The average age of SLE onset was significantly higher in patients with PNS manifestations than in controls (mean ± standard deviation (SD): 45.9±14.8 vs 37.1±14.0) and they were more likely to have a higher SLEDAI-2K and SLICC/ACR Damage Index (SDI) scores, and hypertension and livedo reticularis. A subgroup analysis for events deemed to be SLE-related provided similar results.Conclusion. PNS involvement is an uncommon, but not so rare complication of SLE. A careful neurological evaluation for this manifestation should be included in the diagnostic workup, especially in patients with later onset and with higher damage and disease activity.

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BackgroundThe aim was to assess the attainability and outcome of the lupus low disease activity state (LLDAS) in the early stages of systemic lupus erythematosus (SLE).MethodsLLDAS prevalence was evaluated at 6 (T1) and 18 (T2) months after diagnosis and treatment initiation (T0) in a monocentric cohort of 107 (median disease duration 9.7 months) prospectively followed Caucasian patients with SLE. Reasons for failure to achieve LLDAS were also investigated. Multivariate models were built to identify factors associated with lack of LLDAS achievement and to investigate the relationship between LLDAS and Systemic Lupus International Collaboration Clinics (SLICC)/Damage Index (SDI) accrual.ResultsThere were 47 (43.9%) patients in LLDAS at T1 and 48 (44.9%) at T2. The most frequent unmet LLDAS criterion was prednisolone dose >7.5 mg/day (83% of patients with no LLDAS at T1). Disease manifestations with the lowest remission rate during follow up were increased anti-double-stranded DNA (persistently present in 85.7% and 67.5% of cases at T1 and T2, respectively), low serum complement fractions (73.2% and 66.3%) and renal abnormalities (46.4% and 28.6%). Renal involvement at T0 was significantly associated with failure to achieve LLDAS both at T1 (OR 7.8, 95% CI 1.4–43.4; p = 0.019) and T2 (OR 3.9, 95% CI 1.4–10.6; p = 0.008). Presence of any organ damage (SDI ≥1) at T2 was significantly associated with lack of LLDAS at T1 (OR 5.0, 95% CI 1.5–16.6; p = 0.009) and older age at diagnosis (OR 1.05 per year, 95% CI 1.01–1.09; p = 0.020).ConclusionLLDAS is a promising treatment target in the early stages of SLE, being attainable and negatively associated with damage accrual, but it fit poorly to patients with renal involvement.

Objective The Physician Global Assessment (PGA) is a visual analogue score that reflects the clinician’s judgement of overall SLE disease activity. The aim of this systematic literature review is to describe and analyse the psychometric properties of the PGA. Methods This systematic literature review was conducted by two independent reviewers in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. All articles published through 1 July 2019 in PubMed were screened, with no limitation on year of publication, language or patients’ age. Psychometric properties data were analysed according to the OMERACT Filter methodology version 2.1. Results The literature search identified 91 studies. Face validity was reported in all the articles retrieved in which the PGA was used alone or as part of composite indices (Systemic Responder Index, Safety of Estrogen in Lupus Erythematosus National Assessment Flare Index, Lupus Low Disease Activity State, Definitions of Remission in Systemic Lupus Erythematosus criteria). Content validity was reported in 89 studies. Construct validity was demonstrated by a good correlation (r ≥ 0.50) between the PGA with the SLEDAI (12 studies), SLAM (4 studies), LAI, BILAG and ECLAM (2 studies each). Criterion validity was assessed exploring the PGA correlation with quality of life measurements, biomarker levels and treatment changes in 28 studies, while no study has evaluated correlation with damage. A good responsiveness for PGA was shown in eight studies. A high variability in scales was found, causing a wide range of reliability (intraclass correlation coefficient 0.67–0.98). Conclusion PGA is a valid, responsive and feasible instrument, though its reliability was impacted by the scale adopted, suggesting the major need for standardization of its scoring.

Objective To evaluate the reliability of virtual video-assisted visits, added to the tight control strategy for inflammatory rheumatic diseases (IRDs), in identifying patients that need treatment adjustment. Methods Tightly followed-up adult patients with rheumatoid arthritis, psoriatic arthritis (PsA), ankylosing spondylitis, and systemic lupus erythematosus (SLE) performed a video consultation during COVID19 lockdown and repeated the same rheumatology evaluations through a face-to-face visit within 2-weeks. Sensitivity and specificity of virtual visits for treatment decisions (categorized as unchanged, adjusted/escalate, tapered/discontinued, need for further examinations), and the intraclass correlation coefficient (ICC) for virtually measured disease activity and patient-reported outcomes (PROs) were calculated with 95% confidence interval (95%CI) using face-to-face visits as the reference method. Results In 89 out of 106 (84.0%) patients, face-to-face visits confirmed the remotely delivered treatment decision. Video-visiting showed excellent sensitivity (94.1% with 95%CI 71.3%-99.9%) and specificity (96.7%; 95%CI 90.8% to 99.3%) in identifying the need for treatment adjustment due to inadequate disease control. The major driver for the low sensitivity of virtual video consultation (55.6%; 95%CI 21.2%-86.3%) in identifying the need for treatment tapering was SLE diagnosis (OR 10.0; 95%CI 3.1-32.3; p < 0.001), mostly because of discordance with face-to-face consultation in glucocorticoid tapering. Remotely evaluated PROs showed high reliability (ICC range 0.80 to 0.95) whilst disease activity measures had less consistent data (ICC range 0.50 to 0.95), especially those requiring more extensive physical examination such as in SLE and PsA. Conclusion Video-visiting proved high reliability in identifying the need for treatment adjustment and might support the IRDs standard tight-control strategy.

A systematic review and meta-analysis were conducted, according to the PRISMA methodology, to summarize current evidence on the prevalence and predictors of long-term glucocorticoid (GC) treatment and disease relapses in the real-life management of polymyalgia rheumatica (PMR).Out of 5442 retrieved studies, 21 were eligible for meta-analysis and 24 for qualitative analysis. The pooled proportions of patients still taking GCs at 1, 2, and 5 years were respectively 77% (95%CI 71–83%), 51% (95%CI 41–61%), and 25% (95CI% 15–36%). No significant difference was recorded by distinguishing study cohorts recruited before and after the issue of the international recommendations in 2010. The pooled proportion of patients experiencing at least one relapse at 1 year from treatment initiation was 43% (95%CI 29–56%). Female gender, acute-phase reactants levels, peripheral arthritis, starting GCs dosage, and tapering speed were the most frequently investigated potential predictors of prolonged GC treatment and relapse, but with inconsistent results. Only a few studies and with conflicting results evaluated the potential role of early treatment with methotrexate in reducing the GC exposure and the risk of relapse in PMR.This study showed that a high rate of prolonged GC treatment is still recorded in the management of PMR. The relapse rate, even remarkable, can only partially explain the long-term GC treatment, suggesting that other and not yet identified factors may be involved. Additional research is needed to profile patients with a higher risk of long-term GC treatment and relapse and identify more effective steroid-sparing strategies. Key Points:• High rate of long-term glucocorticoid (GC) treatment is recorded in polymyalgia rheumatica (PMR), being 77%, 51%, and 25% of patients still on GCs after respectively 1, 2, and 5 years.• A pooled relapse rate of 43% at 1 year, even remarkable, can only partially explain the long-term GC treatment in PMR.• Several studies have attempted to identify potential predictors of prolonged treatment with GCs and relapse, but with inconsistent results.• Additional research is needed to profile patients with a higher risk of long-term GC treatment and relapse and identify more effective steroid-sparing strategies.

Demyelinating syndrome (DS) is a rare manifestation of systemic lupus erythematosus (SLE) (1%) with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1) neuromyelitis optica; 2) neuromyelitis optica spectrum disorders; 3) DS prevalently involving the brain; 4) DS prevalently involving the brainstem; 5) clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options.