Real-Life Outcome of Lupus Nephritis with Current Therapies: Study Protocol of a Multicentre Observational Study.

Lupus nephritis (LN) affects a significant proportion of patients with systemic lupus erythematosus (SLE) and is characterised by increased morbidity and mortality. The updated joint EULAR/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendations for the management of LN have set as target of therapy the optimisation (preservation or improvement) of kidney function, accompanied by a reduction in proteinuria of at least 25% by 3 months, 50% by 6 months, and below 500-700 mg/g by 12 months (complete clinical response). It is currently unknown what proportion of Greek patients with LN reach these proposed targets with the current available treatments. At the same time, recent successful phase 3 trials have led to the approval of both belimumab and voclosporin for the treatment of patients with LN and have steered discussions as to whether the "induction-maintenance" paradigm should be substituted by an early combination treatment for all patients. To inform future therapeutic decisions and facilitate the positioning of these new drugs in the therapeutic algorithm of LN, the current study protocol aims to map the unmet needs in the treatment of LN in Greece, by quantifying the proportion of patients who attain the recommended treatment targets in everyday clinical practice.

Low disease activity-irrespective of serologic status at baseline-associated with reduction of corticosteroid dose and number of flares in patients with systemic lupus erythematosus treated with belimumab: A real-life observational study.

BACKGROUND: Low disease activity is a validated target of current systemic lupus erythematosus (SLE) therapy. The aim of this study was to assess the ability of belimumab to achieve low disease activity states in real-life settings. METHODS: Multicentre prospective observational study of consecutive SLE patients receiving belimumab for at least 3 months, due to active disease refractory to at least one conventional immunosuppressant. Disease activity, including the recently defined lupus low disease activity state (LLDAS) and remission (clinical SLEDAI-2K = 0), accrual of organ damage, flares and side effects were documented. RESULTS: Ninety-one patients were included [94.5% women, mean (SD) age 45.9 (12.5) years]. Most frequent manifestations were arthritis (76.7%), rash (72.5%), serologic activity (low C3/C4 and/or high anti-dsDNA; 54.9%), hair loss (47.2%) and mucosal ulcers (27.5%). Median (range) duration of treatment was 10.5 (3.0-42.1) months. Belimumab significantly decreased average SLEDAI-2K, physician global assessment (PGA) and daily prednisone dose over time, as early as 3 months after initiation, with over 20% of patients discontinuing corticosteroids. Although reduction in clinical (i.e., excluding serology) SLEDAI-2K was more pronounced in patients who were serologically active (from 8 to 1.5 at 12 months) as compared to serologically inactive (from 6 to 4) at baseline, attainment of LLDAS did not differ between the two groups and was reached by more than 40% of completer patients after 9-12 months. In addition, the number of flares and severe flares was reduced by 62% and 50%, respectively, during the first 12 months of treatment. Twenty patients (22.0%) discontinued treatment due to inadequate response and two due to side effects potentially related to the drug. CONCLUSIONS: In real-life, belimumab is efficacious in achieving low disease activity in over 40% of unselected patients, in combination with reduction of corticosteroid dosage and number of flares. Both serologically active and inactive patients respond to the drug.

Nocardia osteomyelitis in an immunosuppressed patient.

Osteomyelitis is an inflammation process of bone caused by a pathogenic microorganism and associated by edema, thrombosis of small vessels and eventually bone necrosis. Infection of bone occurs as a consequence of hematogenous dissemination of bacteria, invasion from a contiguous focuw of infection and skin breakdown. We report a case of lower limbs osteomyelitis due to Nocardia spp in a 68 years old man with Granulomatosis with Polyangiitis (GPA) during his treatment of underlying vasculitis. This case indicates considering rare pathogens in immunosuppressed patients.

Incident atrial fibrillation in systemic sclerosis: the predictive role of B-type natriuretic peptide.

INTRODUCTION: Atrial fibrillation (AF) is common in patients with systemic sclerosis (SSc) and is associated with significant morbidity, mortality, and healthcare expenditures. The aim of this study was to prospectively determine the incidence and the independent predictors of AF in this patient population. METHODS: Forty-nine patients (age 50.15 ± 9.25 years, 87.8% female) and 21 healthy controls, all in sinus rhythm, were studied. Evaluation included blood sampling, B-type natriuretic peptide (BNP) measurement, comprehensive electrocardiography and echocardiography at baseline, and 24h ambulatory Holter monitoring at baseline and every 6 months. RESULTS: During a mean follow-up of 72 ± 24 months, 18 SSc patients (36.7%) developed AF (SSc-AF group) while 31 remained in sinus rhythm (SSc-SR group); all subjects in the control group (Cl group) remained in SR. Baseline differences between SSc-AF, SSc-SR, and Cl groups included: a) left ventricular (LV) mass: 84.5 ± 26 vs. 71.8 ± 18.6 vs. 60.5 ± 32.6 g/m(2), respectively (p=0.017); b) mitral tissue Doppler imaging E velocity: 14.5 ± 2.8 vs. 17.5 ± 3.4 vs. 20.5 ± 4.4 cm/s (p<0.001); c) left atrial (LA) volume: 18.8 ± 7.8 vs. 13.5 ± 5.1 vs. 9.7 ± 5.4 cm(3)/m(2) (p<0.001); d) LA active emptying volume: 7.6 ± 2.7 vs. 4.7 ± 3.2 vs. 3.3 ± 2.2 cm(3)/m(2) (p<0.001); and e) logBNP: 1.78 ± 0.47 vs. 1.31 ± 0.54 vs. 0.66 ± 0.38 pg/mL (p<0.001). In Cox proportional hazard analysis, BNP was the only independent predictor of incident AF. CONCLUSION: Incident AF was high in SSc, especially in the presence of LV diastolic dysfunction with LA mechanical overload and elevated BNP levels. BNP was the only independent predictor of incident AF; therefore, it should be considered for risk stratification in this population.