Outcomes following immunosuppressive therapy withdrawal after complete renal response in proliferative lupus nephritis.

Objective: To investigate the rate and factors influencing renal relapse (RR) in proliferative lupus nephritis (LN) patients who discontinued immunosuppressive therapy (IST), as well as the long-term renal outcomes following RR.

Methods: Retrospective, single-centre study of biopsy-confirmed LN patients who had received IST for at least 36 months and maintained complete renal response (CRR) for a minimum of 12 months before therapy discontinuation.

Results: Of a total of 106 patients meeting the inclusion criteria, 76 with proliferative classes were selected for analysis.

Key Genes of the Immune System and Predisposition to Acquired Hemophilia A: Evidence from a Spanish Cohort of 49 Patients Using Next-Generation Sequencing.

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the presence of autoantibodies against factor VIII (FVIII). As with other autoimmune diseases, its etiology is complex and its genetic basis is unknown. The aim of this study was to identify the immunogenetic background that predisposes individuals to AHA.

Predictive Factors of the Use of Rituximab and Belimumab in Spanish Lupus Patients.

: To analyze the characteristics and the predictive factors of the use of rituximab and belimumab in daily practice in patients from the inception cohort Registro Español de Lupus (RELES). : The study included 518 patients. We considered patients treated with biologics who received at least one dose of rituximab or belimumab, and possible indications of those manifestations registered at the same time or in the previous 2 months of the start of the therapy.

The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity.

C4b-binding protein (C4BP) is a well-known regulator of the complement system that holds additional and important activities unrelated to complement inhibition. Recently, we have described a novel immunomodulatory activity in the minor C4BP(β-) isoform directly acting over inflammatory phagocytes. Here we show that incorporation of the β-chain to the C4BP α-chain oligomer interferes with this immunomodulatory activity of C4BP.

Methylprednisolone Pulses Plus Tacrolimus in Addition to Standard of Care vs. Standard of Care Alone in Patients With Severe COVID-19. A Randomized Controlled Trial.

Severe lung injury is triggered by both the SARS-CoV-2 infection and the subsequent host-immune response in some COVID-19 patients. We conducted a randomized, single-center, open-label, phase II trial with the aim to evaluate the efficacy and safety of methylprednisolone pulses and tacrolimus plus standard of care (SoC) vs. SoC alone, in hospitalized patients with severe COVID-19.

Pragmatic, open-label, single-center, randomized, phase II clinical trial to evaluate the efficacy and safety of methylprednisolone pulses and tacrolimus in patients with severe pneumonia secondary to COVID-19: The TACROVID trial protocol.

Introduction: Some COVID-19 patients evolve to severe lung injury and systemic hyperinflammatory syndrome triggered by both the coronavirus infection and the subsequent host-immune response. Accordingly, the use of immunomodulatory agents has been suggested but still remains controversial. Our working hypothesis is that methylprednisolone pulses and tacrolimus may be an effective and safety drug combination for treating severe COVID-19 patients.

Beneficial effect of corticosteroids in preventing mortality in patients receiving tocilizumab to treat severe COVID-19 illness.

Objectives: To assess the characteristics and risk factors for mortality in patients with severe coronavirus disease-2019 (COVID-19) treated with tocilizumab (TCZ), alone or in combination with corticosteroids (CS).

Methods: From March 17 to April 7, 2020, a real-world observational retrospective analysis of consecutive hospitalized adult patients receiving TCZ to treat severe COVID-19 was conducted at our 750-bed university hospital. The main outcome was all-cause in-hospital mortality.

Comparative study between two European inception cohorts of patients with early systemic lupus erythematosus.

Objectives: To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up.

Methods: Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared.

Results: One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared.

The value of repeat biopsy in lupus nephritis flares.

Whether a repeat renal biopsy is helpful during lupus nephritis (LN) flares remains debatable. In order to analyze the clinical utility of repeat renal biopsy in this complex situation, we retrospectively reviewed our series of 54 LN patients who had one or more repeat biopsies performed only on clinical indications. Additionally, we reviewed 686 well-documented similar cases previously reported (PubMed 1990-2015).

Enteric-coated mycophenolate sodium versus azathioprine in patients with active systemic lupus erythematosus: a randomised clinical trial.

Objective: To compare the efficacy and safety of enteric-coated mycophenolate sodium (EC-MPS) versus azathioprine (AZA) in patients with active systemic lupus erythematosus (SLE) disease.

Methods: A multicentre, 24-month, superiority, open-label, randomised controlled trial (NCT01112215) was conducted with 240 patients (120 per arm) receiving either EC-MPS (target dose: 1440 mg/day) or AZA (target dose: 2 mg/kg/day) in addition to prednisone and/or antimalarials. The primary endpoint was the proportion of patients achieving clinical remission, assessed by SLE Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG), at 3 and 24 months.

Efficacy of anakinra in an adult patient with recurrent pericarditis and cardiac tamponade as initial manifestations of tumor necrosis factor receptor-associated periodic syndrome due to the R92Q TNFRSF1A variant.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a dominantly inherited autoinflammatory disease caused by TNFRSF1A mutations. Patients with TRAPS suffer from recurrent, long episodes with fever, arthralgia/arthritis, migratory myalgias, abdominal pain, serositis, conjunctivitis and migratory erythematous skin rash. More than 70 different TNFRSF1A mutations have been reported to date, and as consequence of its genetic heterogeneity, TRAPS shows a variable phenotypic expression.

Clinical characteristics during diagnosis of a prospective cohort of patients with systemic lupus erythematosus treated in Spanish Departments of Internal Medicine: The RELES study.

Introduction: Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES).

Patients And Methods: RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed.

First month prednisone dose predicts prednisone burden during the following 11 months: an observational study from the RELES cohort.

Aim: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2-12).

Methods: 223 patients from the Registro Español de Lupus Eritematoso Sistémico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2-12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.

Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature.

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015).

Patterns of drug therapy in newly diagnosed Spanish patients with systemic lupus erythematosus.

Objectives: This is the first Spanish multicentric inception lupus cohort, formed by SLE patients attending Spanish Internal Medicine Services since January 2009. We aimed to analyse drug therapy during the first year of follow-up according to disease severity.

Methods: 223 patients who had at least one year of follow-up were enrolled upon diagnosis of SLE.

[Proliferative mesangial lupus nephritis: description of a cohort of 27 patients].

Background And Objective: To describe our cohort of 27 biopsy-proven patients and their long-term follow-up, with special attention to prognostic factors.

Patients And Methods: Twenty seven patients were retrospectively collected. They were controlled in the Internal Medicine Department of the Bellvitge's Hospital (Spain) between 1974 and 2010.

Patient registries of acute coronary syndrome: assessing or biasing the clinical real world data?

Background: The risk of selection bias in registries and its consequences are relatively unexplored. We sought to assess selection bias in a recent registry about acute coronary syndrome and to explore the way of conducting and reporting patient registries of acute coronary syndrome.

Methods And Results: We analyzed data from patients of a national acute coronary syndrome registry undergoing an audit about the comprehensiveness of the recruitment/inclusion.

Predicting in-hospital mortality with coronary bypass surgery using hospital discharge data: comparison with a prospective observational study.

Introduction And Objectives: The aim was to determine the usefulness of the hospital discharge Minimum Basic Data Set (MBDS) for predicting in-hospital mortality with coronary bypass surgery by using data from a prospective observational study as a reference.

Methods: The observational study involved collecting data on all patients undergoing first coronary bypass surgery at five hospitals in Catalonia, Spain between November 2001 and November 2003. In addition, data covering the same period and hospitals were obtained from the MBDS for procedure code 36.

High disease activity at baseline does not prevent a remission in patients with systemic lupus erythematosus.

Objective: To evaluate the utility of systemic lupus erythematosus (SLE) initial clinical manifestations and the SLE Disease Activity Index (SLEDAI) for identifying patients who will have a remission.

Methods: We studied 100 SLE patients (85 females, 15 males) and identified all patients who had remission (defined as at least one continuous year during which lack of disease activity permitted withdrawal of all treatment to suppress general lupus activity of a particular clinical manifestation). Changes in laboratory parameters without clinical activity, thus not requiring treatment, did not invalidate remission.

Mild presentation of systemic lupus erythematosus in elderly patients assessed by SLEDAI. SLE Disease Activity Index.

Objective: Systemic lupus erythematosus (SLE) predominantly affects young patients. SLE starting in later life has a clinical presentation different than in younger patients. We have used the SLE Disease Activity Index (SLEDAI) to explore the relationship between age of onset and disease activity.

[The involvement of the parenchyma of the central nervous system in Behçet disease].

Introduction: Behçet disease is a systemic form of vasculitis which presents with neurological symptoms with a frequency varying between 16 and 40%. Involvement of the parenchyma has been found to worsen the prognosis in patients with neuroBehçet (NB).

Objective: To review the clinical features and course of patients with NB involving the parenchyma of the central nervous system (CNS).