Systems immunology integrates the complex endotypes of recessive dystrophic epidermolysis bullosa.

Endotypes are characterized by the immunological, inflammatory, metabolic, and remodelling pathways that explain the mechanisms underlying the clinical presentation (phenotype) of a disease. Recessive dystrophic epidermolysis bullosa (RDEB) is a severe blistering disease caused by COL7A1 pathogenic variants. Although underscored by animal studies, the endotypes of human RDEB are poorly understood.

Evolution of therapeutic management of patients with ANCA associated vasculitis in France after licensing Rituximab use.

Introduction: In 2013, rituximab was approved in France for the treatment of ANCA-associated vasculitis (AAV). The aim of the study was to compare the treatment and health events of adult incident patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), included before rituximab approval (over 2010-2012, Group 1) and those included after rituximab approval (over 2014-2017, Group 2).

Method: Data were extracted from the French National Health Insurance database (SNDS) including outpatient health care consumption and hospital discharge forms.

[Diagnosis and management of pruritus associated with chronic kidney disease in hemodialyzed patients].

Chronic kidney disease-associated pruritus (CKD-aP) is a disabling symptom which is frequent and often underestimated. Pa-MRC has a negative impact on quality of life, and is frequently accompanied by sleep disorders and depression. The approval of difelikefalin – a kappa opioid receptor agonist – in this indication requires updated recommendations.

[Pruritus associated with chronic kidney disease in hemodialysis patients: a survey in French nephrologists].

Background: Chronic kidney disease-associated pruritus (CKD-aP) is common in hemodialysis patients and severely impairs their quality of life, but the practices of nephrologists remain poorly known.

Methods: The objective of this on-line survey was to describe the management of CKD-aP in French nephrologists affiliated with the French-speaking Society of Nephrology, Dialysis and Transplantation (SFNDT) and involved in hemodialysis.

Results: In total, 122 questionnaires were completed and 100 were usable.

Impact and Tolerance of Immunosuppressive Treatments in Patients Living with HIV with Inflammatory or Autoimmune Diseases.

Background: Patients living with HIV (PLWHIV) can develop autoimmune diseases (AD) needing immunosuppressive treatments (IST). This study aims to describe the impact of IST in PLWHIV.

Methods: This was a multicentric retrospective observational study in six HIV referral centers on PLWHIV under IST for AD.

Two pediatric cases of multisystem inflammatory-like syndrome following COVID-19 vaccination.

Multisystem inflammatory syndrome in children (MIS-C) is a novel post-infectious disease occurring in the context of SARS-CoV2 infection. COVID-19 vaccines have been authorized since December 2020, and adverse events including myocarditis have been reported following vaccination. We describe the cases of two pediatric patients presenting with clinical and laboratory features suggestive of MIS-C a few days after receiving their first dose of the Pfizer BNT162b2 vaccine.

Epidemiology of granulomatosis with polyangiitis and microscopic polyangiitis in adults in France.

Background: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare systemic necrotizing vasculitis. The national incidence and prevalence of GPA/MPA and patient mortality remain unknown in France. A real-life study using retrospective data from the French National Health Data System was set up to describe the epidemiology and demographic characteristics of hospitalized GPA and MPA patients, overall and by disease.

Renal and vascular outcomes in patients with isolated antiphospholipid syndrome nephropathy.

Background: Antiphospholipid syndrome (APS) nephropathy (APSN) is a rare pattern with specific features resulting from microvascular lesions. The prognosis of APSN, outside of lupus nephritis, is unknown. The aim of this study was to describe the renal, vascular and overall outcomes of patients with APSN.

Antineutrophil Cytoplasm Antibody-Associated Vasculitides Valvular Impairment: Multicenter Retrospective Study and Systematic Review of the Literature.

Objective: While myocardial impairment is a predictor of poor prognosis in antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), little is known about valvular involvement. This study aims at describing the clinical presentation, management, and outcome of endocarditis associated with AAV.

Methods: We conducted a multicenter retrospective study in centers affiliated with the French Vasculitis Study Group.

[Current status of persistent chronic hyperkalaemia in France: An expert consensus based on a Delphi approach].

Introduction: In France, no consensus document on the management of persistent hyperkalaemia is currently available. Variability in clinical practices has been observed.

Methods: A consensus statement on the definition and the management of persistent hyperkalaemia was developed by a Delphi panel of French nephrologists between December 2019 (26 voting participants among 40 invited panellists in first round) and June 2020 (20 voting participants among 26 panellists in second round).

Paradoxical reactions and biologic agents: a French cohort study of 9303 patients.

Background: Paradoxical reactions (PRs) are defined as the occurrence during biologic therapy of a pathological condition that usually responds to these drugs.

Objectives: To estimate the incidence of PRs and identify risk factors.

Methods: Multicentre study of the database for the Greater Paris University Hospitals, including biologic-naive patients receiving anti-tumour necrosis factor-α, anti-interleukin-12/23, anti-interleukin-17 or anti-α4β7-integrin agents for psoriasis, inflammatory rheumatism or inflammatory bowel disease (IBD).

Early Renal Recovery after the First Flare of Pauci-Immune Glomerulonephritis.

Introduction: Renal involvement is a severe manifestation of antineutrophil cytoplasmic antibody-associated vasculitis. Patients often progress to end-stage renal disease. The potential for renal recovery after the first flare has seldom been studied.

Cutaneous drug-induced lupus erythematosus: Clinical and immunological characteristics and update on new associated drugs.

Cutaneous drug-induced lupus erythematosus (CDILE) is a lupus-like syndrome related to drug exposure which typically resolves after drug discontinuation. It can present as a systemic or a sole cutaneous form and different drugs may be associated with each form. CDILE pharmacoepidemiology is constantly changing.

ANCA-Negative Pauci-immune Necrotizing Glomerulonephritis: A Case Series and a New Clinical Classification.

Rationale & Objective: Pauci-immune necrotizing glomerulonephritis (PING) is usually associated with the presence of antineutrophil cytoplasmic antibodies (ANCA). However, a minority (2%-3%) of patients with PING do not have detectable ANCA. We assessed the clinical spectrum and outcome of patients with ANCA-negative PING.

Performance of the Kleihauer Betke test in the prediction of neonatal anemia.

Objectives: The aim of this study was to correlate antenatal Kleihauer (KT) test results with fetal hemoglobin at birth to find a threshold for predicting severe fetal anemia. The secondary objectives were to assess the impact of KT on obstetric management and to study the correlation between the middle cerebral artery peack systolic velocity and fetal anemia.

Results: One thousand forty-six KT were positive over the 10-year period, but only 147 were included from 88 patients, of which 17 fetuses were anemic.