Prevalence of anti-neutrophil cytoplasmic antibody-associated vasculitis in the south of France, using the capture-recapture method.

Objectives: This study aimed to estimate the prevalence of ANCA-associated vasculitis (AAV). That is, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), in Southern France in 2018, and evaluate differences among Europeans and non-Europeans.

Methods: This population-based, cross-sectional study used four sources (hospitals, community-based physicians, laboratories, National Health Insurance) to identify adults ≥15 years diagnosed with GPA, MPA or EGPA, living in Hérault and Gard in 2018.

Single cell RNA sequencing reveals distinct clusters of Irf8-expressing pulmonary conventional dendritic cells.

A single population of interferon-regulatory factor 8 (Irf8)-dependent conventional dendritic cell (cDC type1) is considered to be responsible for both immunogenic and tolerogenic responses depending on the surrounding cytokine . Here, we challenge this concept of an omnipotent single Irf8-dependent cDC1 cluster through analysis of pulmonary cDCs at single cell resolution. We report existence of a pulmonary cDC1 cluster lacking Xcr1 with an immunogenic signature that clearly differs from the Xcr1 positive cDC1 cluster.

Interleukin-17A and interleukin-22 production by conventional and non-conventional lymphocytes in three different end-stage lung diseases.

Objectives: The contribution of adaptive innate lymphocytes to IL-17A and IL-22 secretion at the end stage of chronic lung diseases remains largely unexplored. In order to uncover tissue- and disease-specific secretion patterns, we compared production patterns of IL-17A and IL-22 in three different human end-stage lung disease entities.

Methods: Production of IL-17A, IL-22 and associated cytokines was assessed in supernatants of re-stimulated lymphocytes by multiplex assays and multicolour flow cytometry of conventional T cells, iNKT cells, γδ T cells and innate lymphoid cells in bronchial lymph node and lung tissue from patients with emphysema ( = 19), idiopathic pulmonary fibrosis ( = 14) and cystic fibrosis ( = 23), as well as lung donors ( = 17).

Diagnostic performance of F-FDG-PET/CT in inflammation of unknown origin: A clinical series of 317 patients.

Background: Inflammation of unknown origin (IUO) is a challenging situation in internal medicine.

Objectives: To describe the final diagnoses in IUO and assess the helpfulness of F-fluorodesoxyglucose positron emission tomography with computerized tomography ( F-FDG-PET/CT) in the diagnosis strategy.

Results: A total of 317 IUO patients with F-FDG-PET/CT were enrolled.

IL-17A from innate and adaptive lymphocytes contributes to inflammation and damage in cystic fibrosis lung disease.

Background: Elevated levels of interleukin (IL)-17A were detected in the airways of patients with cystic fibrosis (CF), but its cellular sources and role in the pathogenesis of CF lung disease remain poorly understood. The aim of this study was to determine the sources of IL-17A and its role in airway inflammation and lung damage in CF.

Methods: We performed flow cytometry to identify IL-17A-producing cells in lungs and peripheral blood from CF patients and β-epithelial Na channel transgenic (-Tg) mice with CF-like lung disease, and determined the effects of genetic deletion of and on the pulmonary phenotype of -Tg mice.

S100A8 and S100A9 Are Important for Postnatal Development of Gut Microbiota and Immune System in Mice and Infants.

Background & Aims: After birth, the immune system matures via interactions with microbes in the gut. The S100 calcium binding proteins S100A8 and S100A9, and their extracellular complex form, S100A8-A9, are found in high amounts in human breast milk. We studied levels of S100A8-A9 in fecal samples (also called fecal calprotectin) from newborns and during infancy, and their effects on development of the intestinal microbiota and mucosal immune system.

Efficient homing of T cells via afferent lymphatics requires mechanical arrest and integrin-supported chemokine guidance.

Little is known regarding lymph node (LN)-homing of immune cells via afferent lymphatics. Here, we show, using a photo-convertible Dendra-2 reporter, that recently activated CD4 T cells enter downstream LNs via afferent lymphatics at high frequencies. Intra-lymphatic immune cell transfer and live imaging data further show that activated T cells come to an instantaneous arrest mediated passively by the mechanical 3D-sieve barrier of the LN subcapsular sinus (SCS).

Clever-1 contributes to lymphocyte entry into the spleen via the red pulp.

Lymphocytes recirculate continuously between the blood and lymphoid organs, a process that is of fundamental importance for proper functioning of the immune system. The molecular mechanisms underlying lymphocyte trafficking to the spleen remain an enigma. Here, we show that lymphocytes enter the spleen preferentially from vessels in the red pulp rather than the marginal sinus or the vasculature in the white pulp.

Hematopoietic stem cell gene therapy for IFNγR1 deficiency protects mice from mycobacterial infections.

Mendelian susceptibility to mycobacterial disease is a rare primary immunodeficiency characterized by severe infections caused by weakly virulent mycobacteria. Biallelic null mutations in genes encoding interferon gamma receptor 1 or 2 ( or ) result in a life-threatening disease phenotype in early childhood. Recombinant interferon γ (IFN-γ) therapy is inefficient, and hematopoietic stem cell transplantation has a poor prognosis.

Mechanisms and Dynamics of T Cell-Mediated Cytotoxicity In Vivo.

Cytotoxic T lymphocytes (CTLs) are critical in the elimination of infected or malignant cells and are emerging as a major therapeutic target. How CTLs recognize and kill harmful cells has been characterized in vitro but little is known about these processes in the living organism. Here we review recent insights into CTL-mediated killing with an emphasis on in vivo CTL biology.

[Opsoclonus-myoclonus syndrome: a case report].

Opsoclonus-myoclonus syndrome is a rare disorder. We report a 44-year-old patient with opsoclonus associated with a cerebellar syndrome revealing a small cell lung carcinoma. The treatment with chemotherapy initially improved the clinical symptoms but these eventually recurred.

[Anticoagulant induced intramural duodenal hematoma presenting as duodenal obstruction].

An intramural hematoma of the duodenum was first reported in an article in the Lancet in 1838. At least, 90% of cases are caused by blunt abdominal trauma, typically accidents involving bicycles or motor vehicles. Intramural duodenal hematoma is a rare complication of anticoagulant therapy.

[Environmental factors and iatrogenic elements in systemic scleroderma and related syndromes. Review of the literature].

The etiology of scleroderma remains unknown. Although a genetic susceptibility seems to play a role, some environmental and iatrogenic factors have been suggested to trigger the disease. Contact for many months or years with natural or synthetic "toxic" products (by inhalation, cutaneous contact, injection, swallowing or surgical implant) could be implicated in the development of typical scleroderma or pseudo-scleroderma.

Remitting seronegative symmetrical synovitis with pitting oedema: disease or syndrome?

Objective: To evaluate the outcome of patients with remitting seronegative symmetrical synovitis with pitting oedema (RS3PE).

Methods: In a retrospective chart review study, we identified all the patients presenting with polyarthritis and pitting oedema in the past 20 years. We tried to recall the 24 patients with characteristics of RS3PE according to McCarty et al.