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VEXAS Syndrome-Review.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined refractory adult-onset autoinflammatory syndrome caused by somatic mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene in hematopoietic stem and progenitor cells, resulting in a shift in UBA1 isoform expression. Thus, patients develop a spectrum of systemic inflammatory manifestations and hematologic symptoms. To date, patients respond poorly to immune suppressive drugs, except high-dose glucocorticoids, and no treatment guidelines have been established. Given the high mortality ra

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… proves that we need to consider VEXAS as a differential diagnosis for refractory vasculitis with abnormal hematological manifestations. 40 Lung Involvement Lung involvement, such as neutrophilic alveolitis, pleural inflammation, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, and bronchiolitis …

Bone Marrow versus Peripheral Blood Grafts for Haploidentical Hematopoietic Cell Transplantation with Post-Transplantation Cyclophosphamide.

In the coronavirus disease 19 (COVID-19) pandemic era, the number of haploidentical hematopoietic cell transplantations (HCTs) with peripheral blood (PB) grafts increased significantly compared with HCTs with bone marrow (BM) grafts, which may be associated with adverse outcomes. We compared outcomes of HCT in BM graft and PB graft recipients age ≥18 years with hematologic malignancies who underwent T cell- replete haploidentical HCT and received graft-versus-host disease (GVHD) prophylaxis with post-transplantation cyclophosphamide, tacrolimus, and mycophenolate mofetil. Among the 264 patient

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… A majority of these patients (57.1%; n = 12) had skin involvement; 38.1% (n = 8) had ocular involvement, 28.5% (n = 6) had oral involvement, 33.3% (n = 7) had gastrointestinal tract involvement, and 2 patients had bronchiolitis obliterans with or without other organ involvement (Supplementary Table S2 …

Salivary Osteopontin as a Potential Biomarker for Oral Mucositis.

Osteopontin (OPN), a multifunctional phosphoglycoprotein also presents in saliva, plays a crucial role in tumour progression, inflammation and mucosal protection. Mucosal barrier injury due to high-dose conditioning regimen administered during autologous and allogeneic peripheral stem cell transplantation (APSCT) has neither efficient therapy nor established biomarkers. Our aim was to assess the biomarker role of OPN during APSCT, with primary focus on oral mucositis (OM). Serum and salivary OPN levels were determined by ELISA in 10 patients during APSCT at four stages of transplantation (day

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… Plasma osteopontin is a biomarker specifically associated with bronchiolitis obliterans syndrome after HCT. Biol. Blood Marrow Transplant. 2016;22:S417–S418. doi: 10.1016/j.bbmt.2015.11.955. 54. Da Silva Santos P.S., Coracin F.L., José Carlos de Almeida B., Gallottini M.H.C. …

C-Reactive Protein: Pathophysiology, Diagnosis, False Test Results and a Novel Diagnostic Algorithm for Clinicians.

The current literature provides a body of evidence on C-Reactive Protein (CRP) and its potential role in inflammation. However, most pieces of evidence are sparse and controversial. This critical state-of-the-art monography provides all the crucial data on the potential biochemical properties of the protein, along with further evidence on its potential pathobiology, both for its pentameric and monomeric forms, including information for its ligands as well as the possible function of autoantibodies against the protein. Furthermore, the current evidence on its potential utility as a biomarker of

The emerging role of exosomes in innate immunity, diagnosis and therapy.

Exosomes, which are nano-sized transport bio-vehicles, play a pivotal role in maintaining homeostasis by exchanging genetic or metabolic information between different cells. Exosomes can also play a vital role in transferring virulent factors between the host and parasite, thereby regulating host gene expression and the immune interphase. The association of inflammation with disease development and the potential of exosomes to enhance or mitigate inflammatory pathways support the notion that exosomes have the potential to alter the course of a disease. Clinical trials exploring the role of exo

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… Lung transplant recipients who displayed both the phenotypes of obstructive bronchiolitis obliterans syndrome and restrictive allograft syndrome had exosomes with distinct molecular and immunological profiles. …

Current State of Knowledge on Primary Sjögren's Syndrome, an Autoimmune Exocrinopathy.

Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most frequent connective tissue disease after rheumatoid arthritis, affecting 0.3-3% of the population. Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the saliv

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… (mainly follicular bronchiolitis). … Lymphocytic interstitial pneumonitis (LIP) arrives in 3rd position (15% of ILD cases) and can be considered as a more specific benign diffuse lymphoproliferative disorder of pSS, probably starting from the follicular bronchiolitis. …

Viruses

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… affected muscles (most commonly the legs), with elevation of serum creatinine phosphokinase, myoglobinuria, and renal failure Parainfluenza Upper respiratory tract infection Fever, cough, sore throat, rhinorrhea, otitis media Lower respiratory tract infection Fever, cough, dyspnea, and hypoxia, with bronchiolitis … affected muscles (most commonly the legs), with elevation of serum creatinine phosphokinase, myoglobinuria, and renal failure Parainfluenza | Upper respiratory tract infection | Fever, cough, sore throat, rhinorrhea, otitis media Lower respiratory tract infection | Fever, cough, dyspnea, and hypoxia, with bronchiolitis …

A combined biomarker and clinical panel for chronic graft versus host disease diagnosis.

Whilst many chronic graft versus host disease (cGVHD) biomarkers have been previously reported, few have been verified in an independent cGVHD cohort. We aimed to verify the diagnostic accuracy of previously reported markers of cGVHD in a multi-centre Chronic GVHD Consortium. A total of 42 RNA and 18 protein candidate biomarkers were assessed amongst 59 cGVHD cases and 33 matched non-GVHD controls. Total RNA was isolated from PBMC, and RNA markers were quantified using PCR. Serum protein markers were quantified using ELISA. A combined 3 RNA biomarker (IRS2, PLEKHF1 and IL1R2) and 2 clinical va

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… Decreased serum levels of clara cell secretory protein (CC16) are associated with bronchiolitis obliterans and may permit early diagnosis in patients after allogeneic stem‐cell transplantation . …

Adverse Events of Oncologic Immunotherapy and Their Management.

Over the past two decades, immunotherapy has emerged as a promising treatment option for patients with cancer. However, newer versions of immunotherapy, such as checkpoint inhibitors, may be associated with unusual adverse effects (AEs) that can range in severity from mild to life-threatening. Unlike common AEs of conventional chemotherapy, which have a predictable nadir or cyclic pattern after administration, AEs of these newer immunotherapies are variable, depending on the type of immunotherapy, route of administration, and mechanism of action. The onset and resolution of these AEs may be pr

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… such as cytopenias is unclear, AEs such as Stevens–Johnson syndrome, urticaria, serum sickness, and anaphylaxis are generally mediated by the immune system.[ 33 ] The mechanism behind pulmonary AEs such as interstitial pneumonitis, acute respiratory distress syndrome, hypersensitivity pneumonitis, or bronchiolitis …

Pilot study of lithium to restore intestinal barrier function in severe graft-versus-host disease.

UNLABELLED: Severe intestinal graft-vs-host disease (GVHD) after allogeneic hematopoietic cell transplantation (HCT) causes mucosal ulceration and induces innate and adaptive immune responses that amplify and perpetuate GVHD and the associated barrier dysfunction. Pharmacological agents to target mucosal barrier dysfunction in GVHD are needed. We hypothesized that induction of Wnt signaling by lithium, an inhibitor of glycogen synthase kinase (GSK3), would potentiate intestinal crypt proliferation and mucosal repair and that inhibition of GSK3 in inflammatory cells would attenuate the deregula

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… NR indicates no response; ARDS, adult respiratory distress syndrome; BOS, bronchiolitis obliterans syndrome; DAH, diffuse alveolar hemorrhage; HUS, hemolytic uremic syndrome; cGVHD, chronic GVHD; CMV, cytomegalovirus; and MOF, multiorgan failure. …

Potential drug development and therapeutic approaches for clinical intervention in COVID-19.

While the vaccination is now available to many countries and will slowly dissipate to others, effective therapeutics for COVID-19 is still illusive. The SARS-CoV-2 pandemic has posed an unprecedented challenge to researchers, scientists, and clinicians and affected the wellbeing of millions of people worldwide. Since the beginning of the pandemic, a multitude of existing anti-viral, antibiotic, antimalarial, and anticancer drugs have been tested, and some have shown potency in the treatment and management of COVID-19, albeit others failed to leave any positive impact and a few also became cont

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… The first four CoVs were capable of causing pneumonia and bronchiolitis in children and are also associated with enteric and neurological diseases [9] , [10] , [11] , [12] , [13] , [14] . …

The Burden of Human Metapneumovirus (hMPV) Disease in Older and High-Risk Adults in Developed Countries: A Systematic Literature Review.

IntroductionHuman metapneumovirus (hMPV) causes respiratory infections in individuals of all age groups. While the evidence on hMPV epidemiology is growing, the hMPV burden in adults has not been synthesised. We conducted a critical review of published evidence on the burden of hMPV in older adults and adults with comorbidities.MethodsArticles reporting the burden of hMPV disease in adults ⩾50 years or ⩾18 years with comorbidities in developed countries were searched in Embase and MEDLINE (1/January/2000-12/June/2023) databases. Observational and interventional studies on community-based or me

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… The clinical spectrum of hMPV infection ranges from asymptomatic cases to those with mild symptoms, such as fever, cough, and wheezing, to severe manifestations including bronchiolitis, pneumonia, and exacerbation of asthma and chronic obstructive pulmonary disease (COPD) [ 2 ]. …

Summary for clinicians: ERS guidelines on pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and management. Autoimmune PAP (aPAP) is the most common form, driven by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies. Recommended diagnostic tools include bronchoalveolar lavage and quantitative GM-CSF antibody testing. Whole lung lavage and inhaled GM-CSF are first-line treatm

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… Other adverse events included recurrent infections, post-transplant lymphoproliferative disease and bronchiolitis obliterans syndrome. There were no instances of graft rejection. …

Agaricus blazei-Based Mushroom Extract Supplementation to Birch Allergic Blood Donors: A Randomized Clinical Trial.

Since Agaricus blazei Murill (AbM) extract reduced specific IgE and ameliorated a skewed Th1/Th2 balance in a mouse allergy model, it was tested in blood donors with self-reported, IgE-positive, birch pollen allergy and/or asthma. Sixty recruited donors were randomized in a placebo-controlled, double-blinded study with pre-seasonal, 7-week, oral supplementation with the AbM-based extract AndosanTM. Before and after the pollen season, questionnaires were answered for allergic rhino-conjunctivitis, asthma, and medication; serum IgE was measured, and Bet v 1-induced basophil activation was determ

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… Since the extract also had antibacterial effects in murine sepsis models [ 42 , 43 ], it would have been interesting to examine the anti-allergic effects of Andosan TM in patients with combined allergic and infectious conditions as often seen in asthma/bronchiolitis and atopic dermatitis in infants and …

From Sjögren syndrome to Sjögren disease: rethinking classification and diagnostic complexity.

Sjögren syndrome (SS), traditionally regarded as a sicca-predominant condition, is now recognized as a systemic autoimmune disease characterized by type I interferon activation, B-cell hyperactivity, and periepithelial lymphocytic infiltration. This conceptual shift has prompted changes in terminology, with "SS" increasingly replaced by "Sjögren disease (SjD)" and "secondary SS" by "associated SjD," reflecting the concept of polyautoimmunity and ensuring equal clinical standing. In this review, we trace the historical evolution of SS classification criteria and examine their limitations in rea

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… Airway disease in SjD includes tracheobronchial sicca, bronchiectasis, and chronic bronchiolitis. Tracheobronchial sicca, often presenting as a chronic, dry, non-productive cough, may mimic airway hyperresponsiveness [ 47 ]. …

PNEUMONIA | Community Acquired Pneumonia, Bacterial and Other Common Pathogens

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… to consider infection with a drug-resistant or unusual pathogen (tuberculosis, anthrax, C. burnetii , Burkholderia pseudomallei , Pasteurella multocida , endemic fungi, or hantavirus); a pneumonic complication (lung abscess, endocarditis, empyema); or a noninfectious process that mimics pneumonia (bronchiolitis …

Perspective Applications and Associated Challenges of Using Nanocellulose in Treating Bone-Related Diseases.

Bone serves to maintain the shape of the human body due to its hard and solid nature. A loss or weakening of bone tissues, such as in case of traumatic injury, diseases (e.g., osteosarcoma), or old age, adversely affects the individuals quality of life. Although bone has the innate ability to remodel and regenerate in case of small damage or a crack, a loss of a large volume of bone in case of a traumatic injury requires the restoration of bone function by adopting different biophysical approaches and chemotherapies as well as a surgical reconstruction. Compared to the biophysical and chemothe

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… Several studies have reported brown lung, alveo-bronchiolitis, fibrosis, and granulomatous inflammation in vivo following the exposure to cellulose ( Ttrai et al., 1995 ). …

Toxicology and carcinogenesis studies of divinylbenzene-HP (Cas No. 1321-74-0) in F344/N rats and B6C3F1 mice (inhalation studies).

UNLABELLED: Divinylbenzene-HP is used for producing vinyl polymers. Divinylbenzene-HP was nominated for study by the National Cancer Institute because of the potential for worker exposure and the structural similarity of divinylbenzene to styrene, a potential human carcinogen. Male and female F344/N rats and B6C3F1 mice were exposed to divinylbenzene-HP (80%) by inhalation for 2 weeks, 3 months, or 2 years. Genetic toxicology studies were conducted in Salmonella typhimurium, Escherichia coli, and mouse peripheral blood erythrocytes. 2-WEEK STUDY IN RATS: Groups of five male and five female rat

Review of Paraneoplastic Syndromes in Children with Malignancy.

A paraneoplastic syndrome (PNS) is a set of symptoms and signs that can accompany the formation of a cancer but is not due to its direct infiltration or metastasis. PNS results from the secretion of peptides or hormones by tumor cells or from an immunological cross-reaction between the tumor antigens and host antigens. In the adult population, PNS affects up to 15% of patients with cancer, but data on pediatric patients remain lacking. The remarkable fact is that PNS can precede an oncology diagnosis, even by months or years. PNS can involve virtually any organ of the human body. In children,

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… Constrictive bronchiolitis and paraneoplastic pemphigus caused by unicentric Castleman disease in a young woman: A case report. Front Med (Lausanne) 2024;11:1468251. doi: 10.3389/fmed.2024.1468251. 20. Ouedraogo E, Gottlieb J, de Masson A, et al. …

Açai Berry Attenuates Cyclophosphamide-Induced Damage in Genitourinary Axis-Modulating Nrf-2/HO-1 Pathways.

Cyclophosphamide (CYP) is used to treat different malignancies and autoimmune disorders in men. This chemotherapy frequently reduces tumors, which is beneficial, but also causes infertility because of severe oxidative stress, inflammation, and apoptosis in the bladder and testes brought on by its metabolite, acrolein. The goal of this study was to assess the efficacy of a novel food, açai berry, in preventing CYP-induced damage in the bladder and testes.MethodsCYP was administered intraperitoneally once during the experiment at a dose of 200 mg/kg body weight diluted in 10 mL/kg b.w. of water.

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… Modulation of NLRP3 Inflammasome through Formyl Peptide Receptor 1 (Fpr-1) Pathway as a New Therapeutic Target in Bronchiolitis Obliterans Syndrome. Int. J. Mol. Sci. 2020;21:2144. doi: 10.3390/ijms21062144. 49. …