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• W4206175105 abstract "Common variable immunodeficiency (CVID) is characterized by antibody deficiency and recurrent infections. However, many CVID patients develop noninfectious complications including lymphadenopathy, autoimmunity, and lung disease. Although noninfectious complications are associated with an IFN-γ-driven immune dysregulation, the precise mechanisms are unknown, and treatment is restricted to nonspecific immune suppression therapy. In this issue, Hultberg et al (p 417) used high-sensitivity targeted plasma proteomics and a scoring system, grading the noninfectious complications, in a cohort of CVID patients to demonstrate the following:•Unsupervised clustering of plasma protein profiles identified a subgroup of CVID patients characterized by a higher level of autoimmunity and interstitial lung disease in addition to the presence of aberrant B-cell and T-cell subpopulations.•Plasma protein profiling showed that immune dysregulation was strongly associated with IFN-γ signaling. In summary, high-sensitivity plasma proteomics can provide useful information on the pathophysiology of CVID-related immune dysregulation, thereby serving as potential biomarkers of immune dysregulation and guiding tailored treatment strategies in CVID. Fibrostenosis is a serious complication of eosinophilic esophagitis (EoE) and is associated with epithelial-mesenchymal transition (EMT). A decrease in e-cadherin and increase in vimentin expression are effective biomarkers of EMT. In the phase 2 HEROES trial, 69 patients received subcutaneous injections of placebo (n = 24) or the anti-IL-13 antibody RPC4046 180 mg (n = 19) or 360 mg (n = 26) once weekly for 16 weeks. Gann et al (p 367) report on EMT biomarkers from esophageal biopsies taken at baseline and at week 16. Key findings included:•Compared with pretreatment values, vimentin-positive cells decreased by 9.4%, 30.6%, and 37.4% at week 16 in the placebo, 180-mg, and 360-mg groups (360 mg vs placebo, P = .032).•Mean e-cadherin expression increased 5.6-fold in both RPC4046 groups vs placebo (360-mg dose, P = .047).•E-cadherin increases correlated with improvements in histology, eosinophil counts, endoscopic findings, and symptoms. These findings show that pharmacologic inhibition of IL-13 with RPC4046 decreased indicators of EMT in patients with active EoE, potentially reducing the risk of fibrostenosis. As type 2 cytokines in chronic rhinosinusitis without nasal polyps (CRSsNP) are moderately expressed, the clinical significance of type 2 immune responses in these patients have been underestimated. Delemarre et al (p 337) demonstrated a significant role for type 2 inflammation in the clinical outcome of CRSsNP patients with following key-findings:•Forty-nine percent of 240 CRSsNP patients displayed a typical type 2 immune response similar to, but less severe than, chronic rhinosinusitis with nasal polyps (CRSwNP). This was, associated with eosinophilic inflammation and resulted in eosinophil extracellular trap cell death (EETosis) and deposition of Charcot-Leyden crystals in the mucosal tissues.•CRSsNP patients with an underlying type 2 immune response had higher rates of recurrence after surgery and comorbid asthma with CRSwNP patients.•A switch from type 2 CRSsNP to CRSwNP was observed in only a few patients over 12 years. These findings may open new insights for the implementation of innovative treatments for patients with severe CRSsNP. There is no curative treatment for peanut allergy as of yet. Accidental exposures are common and can be severe. In this issue, Santos et al (p 344) analyzed the utility of various biomarkers to identify children who had severe reactions during oral peanut challenges in the Learning Early about Peanut Allergy (LEAP), Persistence of Oral Tolerance to Peanut (LEAP-On), and Peanut Allergy Sensitization (PAS) studies. Findings included:•The basophil activation test (BAT) predicted peanut sensitization with high specificity (98%) and sensitivity (75%) and predicted severe peanut allergic reactions with even higher sensitivity (100%) and specificity (97%).•Skin prick test (SPT), specific IgE to peanut allergens, and IgG4/IgE to peanut also predicted severe peanut allergic reactions.•BAT and SPT were the best biomarkers to identify patients who reacted to 0.1 g of peanut protein (about half a peanut kernel) or less. Combining biomarkers improved the prediction of allergic children at highest risk, which could allow to improve strategies to prevent severe allergic reactions in the future. Autoimmune cytopenia (AIC) is increasingly recognized following hematopoietic cell transplantation (HCT). Lum et al (p 406) examined AIC in a cohort of 502 children with primary immunodeficiency (PID) who were transplanted between 1987 to 2018. Findings included:•Five-year cumulative incidence of post-HCT AIC was 9% in children with PID, which was higher than most reports in pediatric cohorts.•Isolated autoimmune hemolytic anemia (53%) was the most common post-HCT AIC.•Factors associated with post-HCT AIC included presence of graft-versus-host disease (see Figure panel A) and the use of alemtuzumab and ATG (Figure panel B). This study also examined long-term B-lymphocyte function after rituximab. Of 12 patients who received rituximab and survived without second procedures, 7 (58%) had B-lymphocyte recovery while 5 (42%) had persistent low B-lymphopenia and remained on IVIg replacement. Multicenter studies are underway to study post-HCT nonhematological autoimmune diseases and impact of donor chimerism, immune reconstitution, and thymopoiesis on development of post-HCT autoimmunity. Mastocytosis is characterized by pathologic accumulation of mast cells associated with KIT mutations. In 2017, midostaurin, a multi-kinase/KIT inhibitor was approved for advanced systemic mastocytosis based on results demonstrating decreased mast cell burden and mastocytosis-related organ damage. In this issue, Hartmann et al (p 356) examined the effects of midostaurin on quality of life and mast cell mediator-related symptoms in enrolled patients. Key findings included the following:•Midostaurin significantly improved quality of life and symptom burden in advanced systemic mastocytosis. Improvements were durable during 36 months of follow-up.•Midostaurin substantially ameliorated mast cell mediator-related symptoms such as anaphylaxis, flushing, pruritus, and diarrhea. Moreover, skin lesions improved over time. These findings provide a strong rationale to further explore midostaurin in nonadvanced systemic mastocytosis and other mast cell diseases, in which mediator symptoms are the predominant manifestation." @default.
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• W4206175105 date "2020-08-01" @default.
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• W4206175105 title "The Editors’ Choice" @default.
• W4206175105 doi "https://doi.org/10.1016/j.jaci.2020.06.017" @default.
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