Sulfonamide Hypersensitivity
AbstractSulfonamides, particularly antimicrobial sulfonamides, have been implicated as a common cause of a spectrum of hypersensitivity reactions. Immediate IgE-mediated reactions have been reported but are much less common than delayed cutaneous reactions. Delayed cutaneous reactions range from benign exanthems to severe cutaneous reactions such as Stevens Johnson syndrome, toxic epidermal necrolysis, or drug reaction with eosinophilia and systemic symptoms. Sulfonamides can be subclassified as antimicrobial sulfonamides and non-antimicrobial sulfonamides, which are also distinguished by key structural differences, result...
Source: Clinical Reviews in Allergy and Immunology - July 1, 2021 Category: Allergy & Immunology Source Type: research

Correction to: Mitigating Disparity in Healthcare Resources Between Countries for Management of Hereditary Angioedema
(Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - July 1, 2021 Category: Allergy & Immunology Source Type: research

Precision Medicine in the Treatment of Primary Immune Deficiency Patients With Disorders of Immune Dysregulation
AbstractThere are now more than 450 described monogenic germline mutations for inborn errors of immunity that result in the loss of expression, loss of function (LOF), or gain in function (GOF) of the encoded protein. Molecular characterization of these inborn errors of immunity has not only allowed us to characterize on a genetic basis these immune deficiency disorders but has provided a better understanding of the immunobiology of these inborn errors of immunity. More recently, these advances have allowed us to apply targeted therapy or precision medicine in their treatment. Of particular interest related to this review ...
Source: Clinical Reviews in Allergy and Immunology - June 24, 2021 Category: Allergy & Immunology Source Type: research

Nickel Hypersensitivity to Atrial Septal Occluders: Smoke Without Fire?
AbstractNickel is one of the most common contact allergens worldwide; it is used as the main component of the devices used for atrial septal defects (ASDs) and patent foramen ovale (PFO) closure. Developing nickel hypersensitivity after PFO/ASD occlusion is significantly rarer described in medical literature than typical nickel contact sensitization. The exact pathophysiological mechanism of this “device syndrome” remains unknown, and many question the real incidence or even the existence of this clinical entity. Nevertheless, it has been associated with a wide spectrum of symptoms, including chest pain, migraines, pal...
Source: Clinical Reviews in Allergy and Immunology - June 15, 2021 Category: Allergy & Immunology Source Type: research

Autoantibodies in Neuropsychiatric Systemic Lupus Erythematosus (NPSLE): Can They Be Used as Biomarkers for the Differential Diagnosis of This Disease?
AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neurop...
Source: Clinical Reviews in Allergy and Immunology - June 11, 2021 Category: Allergy & Immunology Source Type: research

A Contemporary Review of Behcet ’s Syndrome
AbstractBehcet ’s syndrome (BS) is a chronic systemic inflammatory vasculitis with a wide range of clinical manifestations including recurrent oral and genital ulcers; cutaneous lesions; and ophthalmic, neurologic, and gastrointestinal involvement. BS has a global distribution but is particularly prevalent in so -called Silk Road populations. Disease onset is usually around the third or fourth decade of life, and the sex ratio is roughly 1:1. Both environmental and genetic factors contribute to the etiology of BS, although the detailed mechanisms remain unclear. At present, there is no laboratory examinatio n with diagno...
Source: Clinical Reviews in Allergy and Immunology - June 2, 2021 Category: Allergy & Immunology Source Type: research

High Estrogen States in Hereditary Angioedema: a Spectrum
AbstractSex differences have been well documented within hereditary angioedema (HAE) over the past several decades. Females often experience more frequent and more intense attacks compared to their male counterparts. Additionally, elevated estrogen levels —as seen in pregnancy and use of oral contraceptives—is a widely known trigger for angioedema attacks. In this review article, we will outline how estrogens’ downstream effects increase bradykinin, a potent vasodilator and key mediator of HAE. Estrogen-dependent HAE is a rare disorder that pro vides insight into the relationship between HAE and estrogens. Females af...
Source: Clinical Reviews in Allergy and Immunology - June 1, 2021 Category: Allergy & Immunology Source Type: research

Global View on Ant Venom Allergy: from Allergenic Components to Clinical Management
This article provides a global view on allergic reactions to the venoms of stinging ants and the contemporary approach to diagnose and manage ant venom allergy. (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - June 1, 2021 Category: Allergy & Immunology Source Type: research

Roles of Immune Cells in Hereditary Angioedema
AbstractHereditary angioedema (HAE) is a rare genetic disease, characterized by recurrent and unexpected potentially life-threatening mucosal swelling. HAE may be further classified into HAE with C1 ‐inhibitor deficiency (C1‐INH‐HAE) and HAE with normal C1‐INH activity (nlC1‐INH‐HAE), mostly due to mutations leading to increased vascular permeability. Recent evidence implicates also the innate and adaptive immune responses in several aspects of angioedema pathophysiology. Monocytes/ macrophages, granulocytes, lymphocytes, and mast cells contribute directly or indirectly to the pathophysiology of angioedema. Imm...
Source: Clinical Reviews in Allergy and Immunology - May 29, 2021 Category: Allergy & Immunology Source Type: research

Significance of Mast Cell Formed Extracellular Traps in Microbial Defense
AbstractMast cells (MCs) are critically involved in microbial defense by releasing antimicrobial peptides (such as cathelicidin LL-37 and defensins) and phagocytosis of microbes. In past years, it has become evident that in addition MCs may eliminate invading pathogens by ejection of web-like structures of DNA strands embedded with proteins known together as extracellular traps (ETs). Upon stimulation of resting MCs with various microorganisms, their products (including superantigens and toxins), or synthetic chemicals, MCs become activated and enter into a multistage process that includes disintegration of the nuclear mem...
Source: Clinical Reviews in Allergy and Immunology - May 22, 2021 Category: Allergy & Immunology Source Type: research

Mitigating Disparity in Health-care Resources Between Countries for Management of Hereditary Angioedema
AbstractHereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent episodes of skin and mucosal edema. The main treatment goal is to enable a “normal life” for all patients. However, due to high costs, there are limited options for the management of HAE in most developing and low-income countries. As a result, most of the recommended first-line treatments are not available. In this review, we attempt to highlight the disparities in he alth-care resources for the management of patients with HAE amongst different countries. Data was collected from HAE experts in countries who provide tabulated info...
Source: Clinical Reviews in Allergy and Immunology - May 18, 2021 Category: Allergy & Immunology Source Type: research

Blood Clotting and the Pathogenesis of Types I and II Hereditary Angioedema
AbstractThe plasma contact system is the initiator of the intrinsic pathway of coagulation and the main producer of the inflammatory peptide bradykinin. When plasma is exposed to a negatively charged surface the two enzymes factor XII (FXII) and plasma prekallikrein (PK) bind to the surface alongside the co-factor high molecular weight kininogen (HK), where PK is non-covalently bound to. Here, FXII and PK undergo a reciprocal activation feedback loop that leads to full contact system activity in a matter of seconds. Although naturally occurring negatively charged surfaces have shown to be involved in the role of the contac...
Source: Clinical Reviews in Allergy and Immunology - May 6, 2021 Category: Allergy & Immunology Source Type: research

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