Perspective: Application of the American College of Medical Genetics Variant Interpretation Criteria to Common Variable Immunodeficiency Disorders
AbstractCommon variable immunodeficiency disorders (CVIDs) are rare primary immunodeficiency diseases (PIDs) mostly associated with late onset antibody failure leading to immune system failure. Patients with CVID are predisposed to disabling complications such as bronchiectasis and systemic autoimmunity. In recent years a large number of genetic defects have become associated with these disorders. Patients with a causative mutation are deemed to have CVID-like disorders, while those with mutations predisposing to or modifying disease severity remain within the spectrum of CVID as defined by current diagnostic criteria. Nex...
Source: Clinical Reviews in Allergy and Immunology - April 5, 2021 Category: Allergy & Immunology Source Type: research

The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency
In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinica l characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions. (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - March 31, 2021 Category: Allergy & Immunology Source Type: research

Unnecessary Abdominal Surgeries in Attacks of Hereditary Angioedema with Normal C1 Inhibitor
AbstractHereditary angioedema (HAE) is an autosomal dominant disease mostly due to the deficiency of C1 inhibitor (C1-INH). HAE with normal C1-INH was first described in 2000 and associated with mutations in the coagulation factor XII in 2006. Both diseases are associated with high bradykinin production, resulting in increased vascular permeability. Gastrointestinal edema due to HAE can be misdiagnosed as acute abdomen and unnecessary surgical procedures may be performed. The present study evaluates the prevalence of surgical procedures and/or acute abdomen in HAE patients with the coagulation factor XII mutation. It is a ...
Source: Clinical Reviews in Allergy and Immunology - March 23, 2021 Category: Allergy & Immunology Source Type: research

Thrombin in the Activation of the Fluid Contact Phase in Patients with Hereditary Angioedema Carrying the F12 P.Thr309Lys Variant
Abstract Hereditary angioedema due to pathogenic FXII variants (HAE-FXII) is a rare dominant disease caused by increased activation of the plasma contact system. The most prevalent HAE-FXII variant, c.1032C  >  A p.Thr309Lys (FXII309Lys), results in a smaller FXII protein with increased sensitivity to fluid-phase activation by poorly understood mechanisms. We aimed to investigate the functionality of the FXII309Lys variant in 33 HAE-FXII  patients, 25 healthy controls and 46 patients with congenital disorders of glycosylation (CDG). Activation of the plasma contact system was assessed by western blo...
Source: Clinical Reviews in Allergy and Immunology - March 16, 2021 Category: Allergy & Immunology Source Type: research

Long-term Outcome of Children Born to Women with Autoimmune Rheumatic Diseases: A Multicentre, Nationwide Study on 299 Randomly Selected Individuals
AbstractThe concern about the offspring ’s health is one of the reasons for a reduced family size of women with rheumatic diseases (RD). Increased risk of autoimmune diseases (AD) and neurodevelopmental disorders (ND) has been reported in children born to patients with RD. Within a nationwide survey about reproductive issues of women wi th RD, we aimed at exploring the long-term outcome of their children. By surveying 398 patients who received their diagnosis of RD during childbearing age (before the age of 45), information about the offspring were obtained from 230 women who declared to have had children. A total of...
Source: Clinical Reviews in Allergy and Immunology - March 16, 2021 Category: Allergy & Immunology Source Type: research

Molecular Dambusters: What Is Behind Hyperpermeability in Bradykinin-Mediated Angioedema?
AbstractIn the last few decades, a substantial body of evidence underlined the pivotal role of bradykinin in certain types of angioedema. The formation and breakdown of bradykinin has been studied thoroughly; however, numerous questions remained open regarding the triggering, course, and termination of angioedema attacks. Recently, it became clear that vascular endothelial cells have an integrative role in the regulation of vessel permeability. Apart from bradykinin, a great number of factors of different origin, structure, and mechanism of action are capable of modifying the integrity of vascular endothelium, and thus, ma...
Source: Clinical Reviews in Allergy and Immunology - March 16, 2021 Category: Allergy & Immunology Source Type: research

Next-Generation Sequencing in the Field of Primary Immunodeficiencies: Current Yield, Challenges, and Future Perspectives
AbstractPrimary immunodeficiencies comprise a group of inborn errors of immunity that display significant clinical and genetic heterogeneity. Next-generation sequencing techniques and predominantly whole exome sequencing have revolutionized the understanding of the genetic and molecular basis of genetic diseases, thereby also leading to a sharp increase in the discovery of new genes associated with primary immunodeficiencies. In this review, we discuss the current diagnostic yield of this generic diagnostic approach by evaluating the studies that have employed next-generation sequencing techniques in cohorts of patients wi...
Source: Clinical Reviews in Allergy and Immunology - March 5, 2021 Category: Allergy & Immunology Source Type: research

Correction to: Serum Immunoglobulin G Levels Predict Biochemical and Histological Remission of Autoimmune Hepatitis Type 1: A Single ‑Center Experience and Literature Review
Correction to: Clinical Reviews in Allergy& Immunology (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - March 5, 2021 Category: Allergy & Immunology Source Type: research

How Angioedema Quality of Life Questionnaire Can Help Physicians in Treating C1-Inhibitor Deficiency Patients?
AbstractThe Angioedema Quality of Life Questionnaire (AE-QoL) is an angioedema (AE)-specific validated questionnaire, which surveys the quality of life of diagnosed patients. The questionnaire has been used in multiple clinical trials. Our aim was to investigate how the questionnaire can assist physicians in the everyday practice of following up and managing C1-inhibitor deficiency patients. In a prospective trial conducted in our center between 2016 and 2018, 125 hereditary angioedema and 10 diagnosed with acquired angioedema completed an AE-QoL during their annual follow-up visit. Laboratory indices (i.e., complement lev...
Source: Clinical Reviews in Allergy and Immunology - March 3, 2021 Category: Allergy & Immunology Source Type: research

Systematic Review and Meta-analysis on the Association of Occupational Exposure to Free Crystalline Silica and Rheumatoid Arthritis
AbstractKey messages•Occupational exposure to free crystalline silica and tobacco smoking are associated with an increased risk rheumatoid arthritis, with the evidence of an interaction in seropositive subjects.•Further studies in the field are needed to support such associationWe carried out a systematic search for all published epidemiological studies concerning the association between occupational exposure to free crystalline silica (FCS) and subsequent development of rheumatoid arthritis (RA). A meta-analysis was conducted on relevant studies. We searched PubMed and Embase, search engines, for original articl...
Source: Clinical Reviews in Allergy and Immunology - March 2, 2021 Category: Allergy & Immunology Source Type: research

A Review of Persistent Post-COVID Syndrome (PPCS)
AbstractPersistent post-COVID syndrome, also referred to aslong COVID, is a pathologic entity, which involves persistent physical, medical, and cognitive sequelae following COVID-19, including persistent immunosuppression as well as pulmonary, cardiac, and vascular fibrosis. Pathologic fibrosis of organs and vasculature leads to increased mortality and severely worsened quality of life. Inhibiting transforming growth factor beta (TGF- β), an immuno- and a fibrosis modulator, may attenuate these post-COVID sequelae. Current preclinical and clinical efforts are centered on the mechanisms and manifestations of COVID-19 a...
Source: Clinical Reviews in Allergy and Immunology - February 20, 2021 Category: Allergy & Immunology Source Type: research

Pathways of Neutrophil Granulocyte Activation in Hereditary Angioedema with C1 Inhibitor Deficiency
AbstractHereditary angioedema (HAE) with C1-inhibitor deficiency belongs to bradykinin-mediated angioedemas. It is characterized by recurrent subcutaneous and/or submucosal swelling episodes (HAE attacks) and erythema marginatum skin rash as a pre-attack (prodromal) phase. HAE attacks were shown to be accompanied by peripheral blood neutrophilia. We aimed to find molecular mechanisms that may explain the distinct role of neutrophil granulocytes in HAE. Plasma levels of blood cells and factors related to neutrophil activation (cytokines, chemokines, chemotactic factors, enzymes, and neutrophil extracellular trap) were measu...
Source: Clinical Reviews in Allergy and Immunology - February 19, 2021 Category: Allergy & Immunology Source Type: research

The Diagnosis and Management of Allergic Reactions Caused by Chinese Materia Medica
AbstractTraditional Chinese medicines (TCM) have been used in China for thousands of years. Although TCM has been generally perceived to be safe, adverse reactions to Chinese materia medica (CMM) have been reported. Most of the adverse reactions are allergic in nature, but other mechanisms may play a role. This review focuses on the mechanism and clinical presentation of these allergic reactions. Allergic reactions can occur as a result of the active and inactive ingredients of CMM. Impurities and chemicals generated during the production process can also lead to allergic or adverse reactions. Environmental factors such as...
Source: Clinical Reviews in Allergy and Immunology - February 19, 2021 Category: Allergy & Immunology Source Type: research

Correction to: Pathophysiology of Hereditary Angioedema (HAE) Beyond the SERPING1 Gene
The original version of this article unfortunately contained a mistake. The image of Fig.  1 should be corrected. In the previous figure, we had shown that the plasma kallikrein cleaves both high molecular weight kininogen (HMWK) and low molecular weight kininogen (LMWK) to produce bradykinin. However, the plasma kallikrein activates only high molecular weight kininogen (HMWK) while low molecular weight kininogen (LMWK) is activated by tissue kallikrein. Hence we have removed low molecular weight kininogen (LMWK) in the revised figure. We wish to acknowledge Dr. Allen P. Kaplan, Medical University of South Carolina, C...
Source: Clinical Reviews in Allergy and Immunology - February 17, 2021 Category: Allergy & Immunology Source Type: research

Biomarkers in Hereditary Angioedema
AbstractA biomarker is a defined characteristic measured as an indicator of normal, biologic, pathogenic processes, or biological responses to an exposure or intervention. Diagnostic biomarkers are used to detect a disease or a subtype of a disease; monitoring biomarkers are measured serially to assess a medical condition; response biomarkers are used to check biologic response following a medical intervention; predictive biomarkers are used to identify patients who are more likely to respond to a medical intervention; and prognostic biomarkers are used to assess the future likelihood of a clinical event. Although biomarke...
Source: Clinical Reviews in Allergy and Immunology - February 9, 2021 Category: Allergy & Immunology Source Type: research

New Instrument for the Evaluation of Prodromes and Attacks of Hereditary Angioedema (HAE-EPA)
AbstractA disease-specific, patient-reported outcome instrument suitable for evaluation of prodromes and attacks of hereditary angioedema (HAE) is a clinical unmet need. We constructed such instrument and examined its validity, acceptability, and discriminative ability. Sixty-six patients participated in a survey addressing their demographics, social, and medical status. Discriminant content validity involved: (1) construct definition by in-depth cognitive debriefing interviews, (2) item selection identifying relevant categories, and (3) judgment of the format whereby questionnaires were tested on experienced patients and ...
Source: Clinical Reviews in Allergy and Immunology - February 4, 2021 Category: Allergy & Immunology Source Type: research

B Cell Aberrance in Lupus: the Ringleader and the Solution
AbstractSystemic lupus erythematosus (SLE) is a prototypical autoimmune disease with high heterogeneity but the common characterization of numerous autoantibodies and systemic inflammation which lead to the damage of multiple organs. Aberrance of B cells plays a pivotal role in the immunopathogenesis of SLE via both antibody-dependent and antibody-independent manners. Escape of autoreactive B cells from the central and peripheral tolerance checkpoints, over-activation of B cells and their excessive cytokines release which  drive T cells and dendritic cells stimulation, and dysregulated surface molecules, as well as in...
Source: Clinical Reviews in Allergy and Immunology - February 3, 2021 Category: Allergy & Immunology Source Type: research

Differences and Similarities in the Mechanisms and Clinical Expression of Bradykinin-Mediated vs. Mast Cell –Mediated Angioedema
AbstractAngioedema (AE), transient localized swelling due to extravasated fluid, is commonly classified as mast cell mediator-induced, bradykinin-mediated or of unknown cause. AE often occurs more than once, and it is these recurrent forms of AE that are challenging for patients and physicians, and they are the ones we focus on and refer to as AE in this review. Since effective treatment depends on the causative mediator, reliable and early diagnosis is essential. Although their clinical presentations bear similarities, many forms of angioedema exhibit specific patterns of clinical appearance or disease history that may ai...
Source: Clinical Reviews in Allergy and Immunology - February 3, 2021 Category: Allergy & Immunology Source Type: research

The Enigma of Prodromes in Hereditary Angioedema (HAE)
AbstractA prodrome is a premonitory set of signs and symptoms indicating the onset of a disease. Prodromes are frequently reported by hereditary angioedema (HAE) patients, antedating attacks by a few hours or even longer. In some studies, high incidence of prodromes was reported by patients, with considerable number being able to predict oncoming attacks. Regrettably, prodromes have never received a consensual definition and have not been properly investigated in a systematic fashion. Therefore, their nature remains elusive and their contribution to the diagnosis and treatment of disorders is uncertain. The term “pro...
Source: Clinical Reviews in Allergy and Immunology - February 3, 2021 Category: Allergy & Immunology Source Type: research

Serum Immunoglobulin G Levels Predict Biochemical and Histological Remission of Autoimmune Hepatitis Type 1: A Single-Center Experience and Literature Review
AbstractAutoimmune hepatitis (AIH) is characterized by interface hepatitis, elevated serum alanine aminotransferase and aspartate aminotransferase levels, circulating autoantibodies, and elevated predominantly immunoglobulin G (IgG)  levels. The goal in the treatment of autoimmune hepatitis (AIH) is complete disease remission. Here we took advantage of a large cohort of AIH patients to clarify predictors associated with biochemical and histological remission. Of 705 patients with complete follow-up, 569 (80.7%) patients achie ved complete biochemical remission. Lower IgG levels (17.8 vs. 25 g/L,p 
Source: Clinical Reviews in Allergy and Immunology - January 29, 2021 Category: Allergy & Immunology Source Type: research

Leveraging Genetics for Hereditary Angioedema: A Road Map to Precision Medicine
AbstractBiochemical studies performed during the last decades resulted in the development of various innovative medicinal products for hereditary angioedema (HAE). These therapeutic agents target the production or the function of bradykinin —the main mediator of HAE due to C1-inhibitor (C1-INH) deficiency. However, despite these remarkable achievements, current knowledge cannot provide convincing explanations for the clinical variability of the disease. As a consequence, treatment indications apply for drugs available for C1-INH defi ciency. The advent of high-throughput next-generation sequencing technologies may as...
Source: Clinical Reviews in Allergy and Immunology - January 28, 2021 Category: Allergy & Immunology Source Type: research

Eosinophils in the Field of Nasal Polyposis: Towards a Better Understanding of Biologic Therapies
AbstractEosinophils are often considered as the pathologic landmark of chronic rhinosinusitis with nasal polyps (CRSwNP). Many studies emphasize their pivotal role in mucosal remodeling by their innate action via cytotoxic proteins degranulation. Eosinophil nasal recruitment from the bloodstream through endothelium diapedeses requires the intricate action between the nasal epithelium, epithelial cell-activated type 2 innate lymphoid cells, and adaptive immune cells secreting alarmins, cytokines, and specific chemokines. This immune pathway refers to a T-helper 2 (T2)-driven lymphocyte response, often considered as the main...
Source: Clinical Reviews in Allergy and Immunology - January 26, 2021 Category: Allergy & Immunology Source Type: research

In Search of an Association Between Genotype and Phenotype in Hereditary Angioedema due to C1-INH Deficiency
AbstractHereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is caused by mutations affecting the SERPING1 gene. Adult patients ( ≥ 18 years old) diagnosed with C1-INH-HAE were clustered according to a modified SERPING1 gene mutation classification [5]. Demographic, clinical, and laboratory data were studied. Published manuscripts on the genotype/phenotype relationship were reviewed. Eighty-eight patients participated in the study, with 78 having a classifiable mutation. We compared the data in the 3 largest groups: class 0 only (n = 32), class II only (n = 18), class III ...
Source: Clinical Reviews in Allergy and Immunology - January 19, 2021 Category: Allergy & Immunology Source Type: research

Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review
AbstractThere are two major clinical subsets of scleroderma: (i) systemic sclerosis (SSc) is a complex systemic autoimmune disorder characterized by inflammation, vasculopathy, and excessive fibrosis of the skin and multiple internal organs and (ii) localized scleroderma (LoS), also known as morphea, is confined to the skin and/or subcutaneous tissues resulting in collagen deposition and subsequent fibrosis. SSc is rare but is associated with significant morbidity and mortality compared with other rheumatic diseases. Fatal outcomes in SSc often originate from organ complications of the disease, such as lung fibrosis, pulmo...
Source: Clinical Reviews in Allergy and Immunology - January 15, 2021 Category: Allergy & Immunology Source Type: research

Pathophysiology of Hereditary Angioedema (HAE) Beyond the SERPING1 Gene
AbstractHereditary Angioedema (HAE) is an autosomal dominant disorder characterized clinically by recurrent episodes of swelling involving subcutaneous tissues, gastrointestinal tract, and oro-pharyngeal area. Gene mutations are the most common genetic cause of HAE and observed in more than 90% of patients. More than 700 mutation variants have been described so far. Patients with angioedema who have no mutations in the gene for C1-INH and normal levels and activity of this inhibitor are labelled: normal C1 inhibitor HAE. These include genetic mutations in factor 12 gene, plasminogen gene, angiopoietin gene, kininogen 1, an...
Source: Clinical Reviews in Allergy and Immunology - January 14, 2021 Category: Allergy & Immunology Source Type: research

External Environmental Pollution as a Risk Factor for Asthma
AbstractAir pollution is a worrisome risk factor for global morbidity and mortality and plays a special role in many respiratory conditions. It contributes to around 8 million deaths/year, with outdoor exposure being responsible for more than 4.2 million deaths throughout the world, while more than 3.8 million die from situations related to indoor pollution. Pollutant agents induce several respiratory symptoms. In addition, there is a clear interference in numerous asthma outcomes, such as incidence, prevalence, hospital admission, visits to emergency departments, mortality, and asthma attacks, among others. The particulat...
Source: Clinical Reviews in Allergy and Immunology - January 12, 2021 Category: Allergy & Immunology Source Type: research

Current and Prospective Targets of Pharmacologic Treatment of Hereditary Angioedema Types 1 and 2
In conclusion, an increasing shift towards the use of highly effective long-term prophylaxis is anticipated, which should drastically abate the burden on patients with hereditary angioedema. (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - January 9, 2021 Category: Allergy & Immunology Source Type: research

Are Physicochemical Properties Shaping the Allergenic Potency of Animal Allergens?
AbstractKey determinants for the development of an allergic response to an otherwise ‘harmless’ food protein involve different factors like the predisposition of the individual, the timing, the dose, the route of exposure, the intrinsic properties of the allergen, the food matrix (e.g. lipids) and the allergen modification by food processing. Various physicochemical parameters c an have an impact on the allergenicity of animal proteins. Following our previous review on how physicochemical parameters shape plant protein allergenicity, the same analysis was proceeded here for animal allergens.We found that each p...
Source: Clinical Reviews in Allergy and Immunology - January 7, 2021 Category: Allergy & Immunology Source Type: research

The Roles of Orphan G Protein-Coupled Receptors in Autoimmune Diseases
Abstract G protein-coupled receptors (GPCRs) constitute the largest family of plasma membrane receptors in nature and mediate the effects of a variety of extracellular signals, such as hormone, neurotransmitter, odor, and light signals. Due to their involvement in a broad range of physiological and pathological processes and their accessibility, GPCRs are widely used as pharmacological targets of treatment. Orphan G protein-coupled receptors (oGPCRs) are GPCRs for which no natural ligands have been found, and they not only play important roles in various physiological functions, such as sensory perception, reproduction, ...
Source: Clinical Reviews in Allergy and Immunology - January 7, 2021 Category: Allergy & Immunology Source Type: research

Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis
AbstractAnti-melanoma differentiation-associated gene 5 –positive dermatomyositis (MDA5+ DM) is a rare autoimmune disease predominantly reported in East Asia. MDA5+ DM is an intractable disease with impressively high mortality due to rapid-progressive interstitial lung disease (RPILD). Other typical clinical manifestations comprise DM-specific rash (Gottron ’s papules, heliotrope rash) and amyopathic/hypomyopathic muscle involvement. Multiple prognostic factors have been identified. Baseline forced vital capacity (FVC) %-based staging could serve as a simplified risk stratification system. Serum biomarkers incl...
Source: Clinical Reviews in Allergy and Immunology - January 6, 2021 Category: Allergy & Immunology Source Type: research

New Insights on the Role of TRP Channels in Calcium Signalling and Immunomodulation: Review of Pathways and Implications for Clinical Practice
AbstractCalcium is the most abundant mineral in the human body and is central to many physiological processes, including immune system activation and maintenance. Studies continue to reveal the intricacies of calcium signalling within the immune system. Perhaps the most well-understood mechanism of calcium influx into cells is store-operated calcium entry (SOCE), which occurs via calcium release-activated channels (CRACs). SOCE is central to the activation of immune system cells; however, more recent studies have demonstrated the crucial role of other calcium channels, including transient receptor potential (TRP) channels....
Source: Clinical Reviews in Allergy and Immunology - January 6, 2021 Category: Allergy & Immunology Source Type: research

Correction to: Immunometabolic Pathways and Its Therapeutic Implication in Autoimmune Diseases
The online version of the original article can be found athttps://doi.org/10.1007/s12016-020-08821-6 (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - November 27, 2020 Category: Allergy & Immunology Source Type: research

Small Airway Disease in Pediatric Asthma: the Who, What, When, Where, Why, and How to Remediate. A Review and Commentary
AbstractAsthma affects all portions of the airways. Small airways, however, comprise a substantial component of the conducting lung air flow. In asthma, inflammatory processes can affect the whole respiratory tract, from central to peripheral/small airways. The emphasis in adult and pediatric respiratory disease clinics is to focus on large airway obstruction and reversibility. This information, although valuable, underemphasizes a large portion of the conduction airway of asthmatics. Standard descriptions of asthma management focus on a multiple medication approaches. We particularly focused on the management of asthma in...
Source: Clinical Reviews in Allergy and Immunology - November 26, 2020 Category: Allergy & Immunology Source Type: research

The Application of Single-Cell RNA Sequencing in Studies of Autoimmune Diseases: a Comprehensive Review
AbstractComplex composition is one of the most important features of the immune system, involving many types of organs, tissues, cells, and molecules that perform immune functions. The normal function of each component of the immune system is the guarantee for maintaining the relatively stable immune function of the body. When the self-immune tolerance mechanism of the body is unregulated or destroyed, the immune system reacts to autoantigens, resulting in damage to self-tissues and organs or an immunopathological state with abnormal functions. Autoimmune diseases are diverse, and their pathogenesis is complicated. Various...
Source: Clinical Reviews in Allergy and Immunology - November 25, 2020 Category: Allergy & Immunology Source Type: research

Inflammasomes and Childhood Autoimmune Diseases: A Review of Current Knowledge
AbstractInflammasomes are multiprotein complexes capable of sensing pathogen-associated molecular patterns (PAMPs), danger-associated molecular patterns (DAMPs), and cellular perturbations. Upon stimulation, the inflammasomes activate the production of the pro-inflammatory cytokines IL-1 β and IL-18 and induce gasdermin D-mediated pyroptosis. Dysregulated inflammasome signaling could lead to hyperinflammation in response to environmental triggers, thus contributing to the pathogenesis of childhood autoimmune/autoinflammatory diseases. In this review, we group childhood rheumatic di seases into the autoinflammation to ...
Source: Clinical Reviews in Allergy and Immunology - November 25, 2020 Category: Allergy & Immunology Source Type: research

IL-23/IL-17 Axis in Inflammatory Rheumatic Diseases
AbstractIn inflammatory rheumatic disorders, the immune system attacks and damages the connective tissues and invariably internal organs. During the past decade, remarkable advances having been made towards our understanding on the cellular and molecular mechanisms involved in rheumatic diseases. The discovery of IL-23/IL-17 axis and the delineation of its important role in the inflammation led to the introduction of many needed new therapeutic tools. We will present an overview of the rationale for targeting therapeutically the IL-23/IL-17 axis in rheumatic diseases and the clinical benefit which has been realized so far....
Source: Clinical Reviews in Allergy and Immunology - November 13, 2020 Category: Allergy & Immunology Source Type: research

Immunometabolic Pathways and Its Therapeutic Implication in Autoimmune Diseases
AbstractAutoimmune diseases (AIDs) are characterized with aberrant immune responses and their respective signaling pathways controlling cell differentiation, death, and survival. Cell metabolism is also an indispensable biochemical process that provides the very fundamental energy and materials. Accumulating evidences implicate that metabolism pathways have critical roles in determining the function of different immune subsets. Mechanisms of how immunometabolism participate in the pathogenesis of AIDs were also under intensive exploration. Here, in this review, we summarize the metabolic features of immune cells in AIDs an...
Source: Clinical Reviews in Allergy and Immunology - November 12, 2020 Category: Allergy & Immunology Source Type: research

Distinctive Clinical Characteristics and Outcome of ILD-Onset Rheumatoid Arthritis and ACPA-Positive ILD: a Longitudinal Cohort of 282 Cases
AbstractThe aim of this study is to investigate the clinical features and outcome of interstitial lung disease (ILD)-onset rheumatoid arthritis (RA) and anti-citrullinated protein antibody (ACPA)-positive ILD-only patients. Arthritis-onset and ILD-onset RA-ILD and ACPA-positive ILD-only patients consecutively admitted to Peking Union Medical College Hospital from January 2008 to December 2017 were enrolled and followed-up. Their demographic, clinical, and laboratory features as well as outcome were collected and analyzed. Compared with arthritis-onset RA-ILD (n = 166, median arthritis-to-ILD interval: 60 mon...
Source: Clinical Reviews in Allergy and Immunology - November 10, 2020 Category: Allergy & Immunology Source Type: research

The AGE-RAGE Axis and RAGE Genetics in Chronic Obstructive Pulmonary Disease
AbstractChronic obstructive pulmonary disease (COPD) is a heterogeneous group of lung diseases limiting the airflow due to narrowing of airways, chronic bronchitis and emphysema that leads to difficulties in breathing. Chronic inflammation is another important characteristic of COPD which leads to immune cell infiltration and helps in the alveolar destruction. Pathology of COPD is driven by various environmental and genetic factors. COPD is mainly associated with the inhalation of toxic agents mainly the cigarette smoke. Receptor for advanced glycation end products (RAGE) has emerged as a pattern recognition receptor and i...
Source: Clinical Reviews in Allergy and Immunology - November 10, 2020 Category: Allergy & Immunology Source Type: research

Management of Severe Refractory Systemic Lupus Erythematosus: Real-World Experience and Literature Review
AbstractSystemic lupus erythematosus (SLE) is a highly heterogeneous disease affecting multiple organs and is characterized by an aberrant immune response. Although the mortality of SLE has decreased significantly since the application of glucocorticoids, severe or refractory SLE can potentially cause irreversible organ damage and contribute to the disease morbidity and mortality. Early recognition of severe SLE or life-threatening conditions is of great challenge to clinicians since the onset symptoms can be rapid and aggressive, involving the visceral organs of the neuropsychiatric, gastrointestinal, hematologic, renal, ...
Source: Clinical Reviews in Allergy and Immunology - November 7, 2020 Category: Allergy & Immunology Source Type: research

Neutrophils in the Pathogenesis of Rheumatic Diseases: Fueling the Fire
AbstractSystemic rheumatic diseases are a heterogeneous group of disorders characterized by profound immune dysregulation. Recent discoveries have led to a significant resurgence of interest in neutrophils as shapers of immune dysregulation and as triggers of organ damage in rheumatic diseases. Neutrophils contribute to the initiation, promotion, and perpetuation of immune dysregulation through a variety of mechanisms including synthesis of proinflammatory cytokines, direct tissue damage through degranulation and synthesis of reactive oxygen species, and the formation of neutrophil extracellular traps (NETs). The identific...
Source: Clinical Reviews in Allergy and Immunology - November 5, 2020 Category: Allergy & Immunology Source Type: research

Predictive Features and Clinical Presentation of Interstitial Lung Disease in Inflammatory Myositis
AbstractInterstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients.ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and inte...
Source: Clinical Reviews in Allergy and Immunology - November 3, 2020 Category: Allergy & Immunology Source Type: research

Use of Ultraviolet Blood Irradiation Against Viral Infections
AbstractUltraviolet blood irradiation (UBI) was used with success in the 1930s and 1940s for a variety of diseases. Despite the success, the lack of understanding of the detailed mechanisms of actions, and the achievements of antibiotics, phased off the use of UBI from the 1950s. The emergence of novel viral infections, from HIV/AIDS to Ebola, from SARS and MERS, and SARS-CoV-2, bring back the attention to this therapeutical opportunity. UBI has a complex virucidal activity, mostly acting on the immune system response. It has effects on lymphocytes (T-cells and B-cells), macrophages, monocytes, dendritic cells, low-density...
Source: Clinical Reviews in Allergy and Immunology - October 6, 2020 Category: Allergy & Immunology Source Type: research

Equine Hypersensitivity: the Dark Horse of Allergy
AbstractA significant amount of medical literature has been published on the prevalence and treatment modalities of allergies to common household pets like dogs and cats. Although sensitization rates to horses are high in many urban areas, allergies to horses are rarely discussed. In order for allergists to treat effectively horse-allergic patients, they must be aware of various clinical presentations and treatment modalities that exist. A literature search was conducted in PubMED using the terms horse, immunotherapy, inhalant allergies, and food allergy limited to human studies from any period. What follows is a review of...
Source: Clinical Reviews in Allergy and Immunology - September 1, 2020 Category: Allergy & Immunology Source Type: research

Are Physicochemical Properties Shaping the Allergenic Potency of Plant Allergens?
AbstractThis review searched for published evidence that could explain how different physicochemical properties impact on the allergenicity of food proteins and if their effects would follow specific patterns among distinct protein families. Owing to the amount and complexity of the collected information, this literature overview was divided in two articles, the current one dedicated to protein families of plant allergens and a second one focused on animal allergens. Our extensive analysis of the available literature revealed that physicochemical characteristics had consistent effects on protein allergenicity for allergens...
Source: Clinical Reviews in Allergy and Immunology - September 1, 2020 Category: Allergy & Immunology Source Type: research

Clinical Manifestations and Management of Pediatric Beh çet’s Disease
AbstractBeh çet’s disease (BD) is a chronic, vasculitic disorder affecting all sizes of vessels. The disease rarely onsets at childhood and an early diagnosis is often challenging. Oral ulceration and fever of unknown cause are common initial manifestations that might confuse other inflammatory disorders. Th e clinical manifestation pattern in pediatric BD is heterogeneous and varies in different genders, ethnicities, and geographic regions. There are also some differences in clinical presentations and prognosis between pediatric and adult BD. The disease also affects children at an extremely young age with mo...
Source: Clinical Reviews in Allergy and Immunology - August 6, 2020 Category: Allergy & Immunology Source Type: research

A Review of Neutrophil Extracellular Traps (NETs) in Disease: Potential Anti-NETs Therapeutics
AbstractActivated neutrophils release neutrophil extracellular traps (NETs) in response to a variety of stimuli. NETosis is driven by protein-arginine deiminase type 4, with the release of intracellular granule components that function by capturing and destroying microbes, including viral, fungal, bacterial, and protozoal pathogens. The positive effects of pathogen control are countered by pro-inflammatory effects as demonstrated in a variety of diseases. Components of NETS are non-specific, and other than controlling microbes, they cause injury to surrounding tissue by themselves or by increasing the pro-inflammatory resp...
Source: Clinical Reviews in Allergy and Immunology - July 31, 2020 Category: Allergy & Immunology Source Type: research

Autoimmunity in 2019
AbstractBased on the PubMed data, we have been performing a yearly evaluation of the publications related to autoimmune diseases and immunology to ascertain the relative weight of the former in the scientific literature. It is particularly intriguing to observe that despite the numerous new avenues of immune-related mechanisms, such as cancer immunotherapy, the proportion of immunology manuscripts related to autoimmunity continues to increase and has been approaching 20% in 2019. As in the previous 13  years, we performed an arbitrary selection of the peer-reviewed articles published by the major dedicated Journals an...
Source: Clinical Reviews in Allergy and Immunology - July 28, 2020 Category: Allergy & Immunology Source Type: research

Fish Allergy: Fishing for Novel Diagnostic and Therapeutic Options
AbstractFish allergy is one of the most common food allergies. The currently recommended treatment commonly consists of avoiding all fish species. Recent literature suggests that these recommendations are overprotective for the majority of fish-allergic patients. This review summarizes recent findings and provides practical information regarding management of fish allergy in the individual patient. After precise history taking supported by additional specific IgE measurements and/or skin prick tests, fish-allergic patients can generally be categorized into the following clinical clusters: (A) poly-sensitized patients react...
Source: Clinical Reviews in Allergy and Immunology - July 24, 2020 Category: Allergy & Immunology Source Type: research

Interleukin-35 Promotes Th9 Cell Differentiation in IgG4-Related Disorders: Experimental Data and Review of the Literature
In conclusion, our data demonstrate that IL-35 actively participates in the process of inflammation and plays an important role in Th9 differentiation resulting in an immunoglobulin class switch towards IgG4. (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - July 24, 2020 Category: Allergy & Immunology Source Type: research