American College of Rheumatology Guidance Statement for Diagnosis and Management of VEXAS Developed by the International VEXAS Working Group Expert Panel.

Objective: Vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.

Methods: Given the variability in disease presentation and the limited number of studies to date, no clinical documents currently exist to provide guidance to health care providers about the management of VEXAS.

Serum interferon-beta level determined by an ultrasensitive electrochemiluminescence immunoassay is increased in clinically active and inactive systemic lupus erythematosus.

Type I interferons, which play an important role in the pathogenesis of various autoimmune diseases such as systemic lupus erythematosus (SLE), are expressed at very low levels under physiological conditions. In this study, we focused on IFN-beta (IFN) β-for its potential use as a biomarker of SLE activity and compared three different technologies for its quantification in the serum of healthy donors and patients with SLE. A total of 93 serum samples from healthy donors and 463 serum samples from lupus patients were tested using either ELISA, digital ELISA based on Single Molecule Array (Simoa®) technology, or a novel ultrasensitive immunoassay (S-Plex®) based on electrochemiluminescence.

Single-Cell Profiling of Lichen Planus Reveals Type I IFN Response Triggering the Interface Dermatitis Reaction.

Cutaneous lichen planus (LP) is an inflammatory skin disease characterized by an interface dermatitis with lymphocyte infiltration and keratinocyte (KC) cell death. The aim of this study was to investigate the immunopathogenesis of LP and assess the underlying mechanisms that drive the interface dermatitis reaction. We first performed single-cell RNA sequencing on lesional skins from patients with LP compared with healthy control skins and demonstrate that LP skin is imprinted by a type I IFN-rich environment.

[New treatments for cutaneous lupus].

NEW TREATMENTS FOR CUTANEOUS LUPUS. Cutaneous lupus may present as an isolated skin disease, or may be associated with systemic lupus, of which it is one of the most frequent manifestations. The management of this disease is poorly codified, and treatment depends on the severity of the disease, whether or not it is associated with systemic lupus, and many other factors.

Cluster analysis identifies three clinical patterns of patients with systemic autoimmune diseases and anti-Ku antibodies.

Objective: To determine distinct patterns of patients with autoimmune diseases harbouring anti-Ku antibodies and their respective prognosis.

Methods: Anti-Ku-positive patients were retrieved through four immunology departments. Clusters were derived from unsupervised multiple correspondence analysis, not including the disease's diagnosis, followed by hierarchical clustering.

ERN ReCONNET-SLICC-SLEuro expert consensus on the therapeutic management of rare systemic lupus erythematosus manifestations.

Existing guidelines for systemic lupus erythematosus (SLE) predominantly focus on common and major organ involvements. An international taskforce involving experts from three SLE expert groups (ie, the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases, the Systemic Lupus Erythematosus International Collaborating Clinics group, and the European Lupus Society) was established. A total of 119 participants contributed to the development of consensus therapeutic strategies for 24 rare SLE manifestations, using a multistep process.

Progressive repigmentation of hypopigmented lesions in discoid lupus erythematosus with anifrolumab: A report of two cases.

We report two cases of patients with refractory discoid lupus erythematosus (DLE) and dark skin phototypes who experienced progressive perifollicular repigmentation of depigmented lesions under anifrolumab therapy, with sustained improvement over 18 months. These observations highlight a potential novel effect of anifrolumab on cicatricial dyspigmentation in DLE. This effect may represent a valuable therapeutic option for DLE-related damage, particularly in patients with darker skin types.

IgA vasculitis associated with chronic myelomonocytic leukemia.

IgA vasculitis is a predominantly pediatric autoimmune disease characterized by IgA deposit in small vessels. Chronic myelomonocytic leukemia (CMML) is a rare hematological malignancy classified within myelodysplastic syndromes. Here, we present a previously unrecognized case of CMML associated with IgA vasculitis.

Inflammatory Signatures in VEXAS Syndrome, Myelodysplasia Cutis, and Sweet Syndrome.

Importance: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a monogenic disease caused by UBA1 somatic variants in hematopoietic progenitor cells, mostly involving adult men. It is associated with inflammatory-related symptoms, frequently involving the skin and hematological disorders. Recently described myelodysplasia cutis (MDS-cutis) is a cutaneous manifestation of myelodysplasia in which clonal myelodysplastic cells infiltrate the skin.

Framework for implementing treat-to-target in systemic lupus erythematosus routine clinical care: consensus statements from an international task force.

Implementation of Treat-to-Target (T2T) in routine clinical practice remains low in systemic lupus erythematosus (SLE). Real-world data reveal excessive use of glucocorticoids (GCs) and frequently inadequate disease control. Here, an international task force convened to develop a consensus framework for implementing T2T in routine clinical care of adult patients with SLE.

Interferon-α biological activity is associated with disease activity and risk of flare in cutaneous lupus erythematosus: A monocentric study of 184 patients.

Background: Cutaneous lupus erythematosus (CLE) is associated with unpredictable flares and may induce permanent damage. There is currently no biomarker routinely available in CLE.

Objective: To evaluate the performance of interferon-α (IFN-α) biological activity as biomarker of CLE activity and risk of flare.

Off-label use of biologics in urticarial vasculitis: a European retrospective cohort study.

Objectives: Urticarial vasculitis (UV) is characterized by atypical urticarial lesions and leukocytoclastic vasculitis, sometimes with extracutaneous manifestations. First-line treatment is based on colchicine, hydroxychloroquine, dapsone or low-dose glucocorticoids. In refractory forms, the use of biologics has been anecdotally described as potentially effective.

Erratum: Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.

[This corrects the article DOI: 10.1016/j.eclinm.

Prognostic factors for patients with cancer-associated dermatomyositis: a retrospective, multicentre cohort study of 73 patients.

Objectives: To investigate factors associated with DM complete clinical response and overall survival with a focus on the use of immunosuppressive therapies in patients with cancer-associated DM.

Methods: We performed a multicentre, retrospective cohort study. Multivariable survival analyses used a Cox model with time-dependent covariates and adjustments with inverse probability censoring weighting.

Immunopathogenesis of systemic lupus erythematosus: An update.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by dysregulated immune responses leading to widespread inflammation and damage in various organs. Environmental factors such as infections, hormonal influences and exposure to ultraviolet light can trigger the disease in genetically predisposed individuals. Genome-wide association studies have identified over 100 susceptibility loci linked to immune regulation, interferon (IFN) signaling and antigen presentation in SLE.

French protocol for the diagnosis and management of systemic lupus erythematosus.

Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice.