Diagnostic Modalities in Primary Immunodeficiency
AbstractAs the field of inborn errors of immunity expands, providers continually update and fine-tune their diagnostic approach and selection of testing modalities to increase diagnostic accuracy. Here, we first describe a mechanistic consideration of laboratory testing, highlighting both benefits and drawbacks of currently clinically available testing modalities. Next, we provide methods in evaluation of patients presenting with concern for inborn errors of immunity as defined by the International Union of Immunological Societies 2019 phenotypic categories: primary antibody deficiencies, cellular and humoral immune defici...
Source: Clinical Reviews in Allergy and Immunology - March 15, 2022 Category: Allergy & Immunology Source Type: research

The Airway Microbiome-IL-17 Axis: a Critical Regulator of Chronic Inflammatory Disease
AbstractThe respiratory tract is home to a diverse microbial community whose influence on local and systemic immune responses is only beginning to be appreciated. Increasing reports have linked changes in this microbiome to a range of pulmonary and extrapulmonary disorders, including asthma, chronic obstructive pulmonary disease and rheumatoid arthritis. Central to many of these findings is the role of IL-17-type immunity as an important driver of inflammation. Despite the crucial role played by IL-17-mediated immune responses in protection against infection, overt Th17 cell responses have been implicated in the pathogenes...
Source: Clinical Reviews in Allergy and Immunology - March 11, 2022 Category: Allergy & Immunology Source Type: research

Dupilumab-Associated Adverse Events During Treatment of Allergic Diseases
AbstractAmong the new biological therapies for atopic diseases, dupilumab is a fully human monoclonal antibody directed against IL-4R α, the common chain of interleukin-4 and interleukin-13 receptors. Dupilumab showed clinical improvements in patients with atopic dermatitis, asthma, and chronic rhinosinusitis and is currently under development for other indications. While dupilumab is considered to be well tolerated, a number of recent publications have reported various adverse events. This review aims to summarize the current knowledge about these adverse events, which may help clinicians to improve the follow-up of pati...
Source: Clinical Reviews in Allergy and Immunology - March 11, 2022 Category: Allergy & Immunology Source Type: research

Premedication Protocols to Prevent Hypersensitivity Reactions to Chemotherapy: a Literature Review
AbstractHypersensitivity reactions (HSRs) to chemotherapy may prevent patients from receiving the most effective therapy. This review was undertaken to identify evidence-based preventive premedication strategies that reduce the likelihood of HSR in the first instance and improve the safety of subsequent infusions in patients who have demonstrated HSR to a certain class of chemotherapy.  PubMed was searched until October 2021 using the key words: “hypersensitivity to chemotherapeutic drugs,” “hypersensitivity to antineoplastic agents,” “taxanes hypersensitivity,” “platinum compound hypersensitivity,” “pre...
Source: Clinical Reviews in Allergy and Immunology - March 8, 2022 Category: Allergy & Immunology Source Type: research

Clinical Significance of Antinucleolar Antibodies: Biomarkers for Autoimmune Diseases, Malignancies, and others
AbstractNucleolar staining is one of the standard patterns in immunofluorescence antinuclear antibodies (ANA), seen in 5 –9% of ANA in various conditions. Antinucleolar antibodies (ANoA) are classified into 3 patterns in the International Consensus on ANA Patterns (ICAP) classification; AC-8 homogeneous pattern, AC-9 clumpy pattern, and AC-10 punctate pattern. Specificities known to show AC-8 include anti-Th/To, -PM -Scl, -nucleophosmin/B23, -nucleolin/C23, -No55, and others. AC-9 is seen by anti-fibrillarin/U3RNP and AC-10 by anti-RNA polymerase I and hUBF/NOR-90. ANoA has been classically known to be associated with sc...
Source: Clinical Reviews in Allergy and Immunology - March 8, 2022 Category: Allergy & Immunology Source Type: research

A Narrative Review of Pathogenetic and Histopathologic Aspects, Epidemiology, Classification Systems, and Disease Outcome Measures in Systemic Sclerosis
AbstractSystemic sclerosis (SSc) is a rare systemic autoimmune disease, characterized by the presence of three main actors: vasculopathy, immune activation, and fibrosis. This pathologic process is then translated in a clinical picture with great variability among different patients in terms of type of organ involvement, disease severity and prognosis. This heterogeneity is a main feature of SSc, which, in addition to the presence of early phases of the disease characterized by mild symptoms, can explain the high difficulty in establishing classification criteria, and in defining patients ’ subsets and disease outcomes. ...
Source: Clinical Reviews in Allergy and Immunology - March 7, 2022 Category: Allergy & Immunology Source Type: research

The Role of Autoantibody Testing in Modern Personalized Medicine
AbstractPersonalized medicine (PM) aims individualized approach to prevention, diagnosis, and treatment. Precision Medicine applies the paradigm of PM by defining groups of individuals with akin characteristics. Often the two terms have been used interchangeably. The quest for PM has been advancing for centuries as traditional nosology classification defines groups of clinical conditions with relatively similar prognoses and treatment options. However, any individual is characterized by a unique set of multiple characteristics and therefore the achievement of PM implies the determination of myriad demographic, epidemiologi...
Source: Clinical Reviews in Allergy and Immunology - March 4, 2022 Category: Allergy & Immunology Source Type: research

Autoantibody Markers of Increased Risk of Malignancy in Patients with Dermatomyositis
AbstractDermatomyositis is a chronic inflammatory disease involving the skin and muscles. It most commonly occurs in adults with preponderance in females, but pediatric occurrence is also possible. The risk of malignancy in adult patients with dermatomyositis was reported to be 4.66-fold higher compared to that in the general population. A significantly increased risk of malignancy was reported within the first 12 months following the diagnosis of dermatomyositis (standardized incidence ratio equaled 17). One of the characteristic laboratory findings associated with dermatomyositis is the presence of circulating autoantibo...
Source: Clinical Reviews in Allergy and Immunology - February 11, 2022 Category: Allergy & Immunology Source Type: research

Spectrum of Genetic T-Cell Disorders from 22q11.2DS to CHARGE
AbstractImproved genetic testing has led to recognition of a diverse group of disorders of inborn errors of immunity that present as primarily T-cell defects. These disorders present with variable degrees of immunodeficiency, autoimmunity, multiple organ system dysfunction, and neurocognitive defects. 22q11.2 deletion syndrome, commonly known as DiGeorge syndrome, represents the most common disorder on this spectrum. In most individuals, a 3  Mb deletion of 22q11 results in haploinsufficiency of 90 known genes and clinical complications of varying severity. These include cardiac, endocrine, gastrointestinal, renal, palata...
Source: Clinical Reviews in Allergy and Immunology - February 8, 2022 Category: Allergy & Immunology Source Type: research

The Role of Patch Testing in Evaluating Delayed Hypersensitivity Reactions to Medications
AbstractConfirming drug imputability is an important step in the management of cutaneous adverse drug reactions (CADR). Re-challenge is inconvenient and in many cases life threatening. We review the literature on ideal patch testing technique for specific CADRs. Testing should be performed approximately 3  months after the resolution of the eruption using standard patch testing techniques. Commercially available patch test preparations are available for a minority of drugs, so in most cases, testing should be performed with the drug at various recommended concentrations and in different vehicles. Te sting to all known exc...
Source: Clinical Reviews in Allergy and Immunology - February 3, 2022 Category: Allergy & Immunology Source Type: research

An Italian Multicenter Study on Anti-NXP2 Antibodies: Clinical and Serological Associations
AbstractThe identification of anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some reports, with cancer. Historically, these associations with anti-NXP2 antibodies have been detected by immunoprecipitation (IP), but in the last few years commercial immunoblotting assays have been released. The aim of this collaborative project was to analyse the clinical features associated to anti-NXP2 antibodies, both with commercial line blot (LB) and IP. Myositis-specific and myositis-associated autoantibodies were detected in single centres by commercial line bl...
Source: Clinical Reviews in Allergy and Immunology - January 29, 2022 Category: Allergy & Immunology Source Type: research

Road Less Traveled: Drug Hypersensitivity to Fluoroquinolones, Vancomycin, Tetracyclines, and Macrolides
AbstractWhile fluoroquinolones, vancomycin, macrolides, and tetracyclines are generally safe antibiotics, they can induce both immediate and delayed hypersensitivity reactions (HSRs). Historically, less has been published on allergies to these antibiotics compared to beta lactams, but the prevalence of non-beta lactam HSRs is increasing. To fluoroquinolones, immediate HSRs are more common than delayed reactions. Both IgE and non-IgE mechanisms, such as the mast cell receptor Mas-related G protein-coupled receptor X2 (MRGPRX2), have been implicated in fluoroquinolone-induced anaphylaxis. Skin testing for fluoroquinolones is...
Source: Clinical Reviews in Allergy and Immunology - January 29, 2022 Category: Allergy & Immunology Source Type: research

Advanced Imaging Supports the Mechanistic Role of Autoimmunity and Plaque Rupture in COVID-19 Heart Involvement
AbstractThe cardiovascular system is frequently affected by coronavirus disease-19 (COVID-19), particularly in hospitalized cases, and these manifestations are associated with a worse prognosis. Most commonly, heart involvement is represented by myocarditis, myocardial infarction, and pulmonary embolism, while arrhythmias, heart valve damage, and pericarditis are less frequent. While the clinical suspicion is necessary for a prompt disease recognition, imaging allows the early detection of cardiovascular complications in patients with COVID-19. The combination of cardiothoracic approaches has been proposed for advanced ima...
Source: Clinical Reviews in Allergy and Immunology - January 28, 2022 Category: Allergy & Immunology Source Type: research

Heart Involvement in Systemic Sclerosis: the Role of Magnetic Resonance Imaging
AbstractSystemic sclerosis (SSc) is a severe connective tissue disease characterized by diffuse vascular damage and aberrant activation of immune system, resulting in inflammation and fibrosis of skin and internal organs, including the heart. Cardiac involvement is frequent in SSc, even though often unrecognized due to the occult nature at early stages and to the lack of a defined diagnostic algorithm. Once clinically evident, heart involvement is associated with a poor prognosis, representing the leading cause of death in about one third of SSc patients. Thus, its early recognition and monitoring are of crucial importance...
Source: Clinical Reviews in Allergy and Immunology - January 24, 2022 Category: Allergy & Immunology Source Type: research

A Contemporary Update on the Diagnosis of Systemic Lupus Erythematosus
AbstractSystemic lupus erythematosus (SLE) is a complicated autoimmune disease with female susceptibility. It is characterized by over-activation of the immune system and deposit of autoimmune complex in multiple organs. High heterogeneity, unpredictable disease course of SLE  as well as the lack of specific and sensitive biomarkers posed diagnostic challenges to clinicians. Despite the complicated clinical presentation and pathogenesis of SLE, research regarding this disease has made many significant breakthroughs over the past decades. Some new learning can potential ly be translated into clinical practice. In addition...
Source: Clinical Reviews in Allergy and Immunology - January 22, 2022 Category: Allergy & Immunology Source Type: research