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Defects of the Innate Immune System and Related Immune Deficiencies
AbstractThe innate immune system is the host ’s first line of defense against pathogens. Toll-like receptors (TLRs) are pattern recognition receptors that mediate recognition of pathogen-associated molecular patterns. TLRs also activate signaling transduction pathways involved in host defense, inflammation, development, and the production of inflammatory cytokines. Innate immunodeficiencies associated with defective TLR signaling include mutations inNEMO,IKBA,MyD88, andIRAK4. Other innate immune defects have been associated with susceptibility to herpes simplex encephalitis, viral infections, and mycobacterial disease, a...
Source: Clinical Reviews in Allergy and Immunology - August 1, 2022 Category: Allergy & Immunology Source Type: research

Agammaglobulinemia: from X-linked to Autosomal Forms of Disease
AbstractInterruptions or alterations in the B cell development pathway can lead to primary B cell immunodeficiency with resultant absence or diminished immunoglobulin production. While the most common cause of congenital agammaglobulinemia is X-linked agammaglobulinemia (XLA), accounting for approximately 85% of cases, other genetic forms of agammaglobulinemia have been identified. Early recognition and diagnosis of these conditions are pivotal for improved outcomes and prevention of sequelae and complications. The diagnosis of XLA is often delayed, and can be missed if patient has a mild phenotype. The lack of correlation...
Source: Clinical Reviews in Allergy and Immunology - August 1, 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 - August 1, 2022 Category: Allergy & Immunology Source Type: research

Novel Genetic Discoveries in Primary Immunodeficiency Disorders
AbstractThe field of Immunology is one that has undergone great expansion in recent years. With the advent of new diagnostic modalities including a variety of genetic tests (discussed elsewhere in this journal), the ability to diagnose a patient with a primary immunodeficiency disorder (PIDD) has become a more streamlined process. With increased availability of genetic testing for those with suspected or known PIDD, there has been a significant increase in the number of genes associated with this group of disorders. This is of great importance as a misdiagnosis of these rare diseases can lead to a delay in what can be crit...
Source: Clinical Reviews in Allergy and Immunology - August 1, 2022 Category: Allergy & Immunology Source Type: research

The Treatment of Primary Immune Deficiencies: Lessons Learned and Future Opportunities
AbstractPrimary immunodeficiency is a group of disorders associated with susceptibility to infectious agents and the development of various comorbidities. Many primary immunodeficiencies are complicated by immune dysregulation, autoinflammation, or autoimmunity which impacts multiple organ systems. Major advances in the treatment of these disorders have occurred over the last half-century, and deeper molecular understanding of many disorders combined with clinically available genetic testing is allowing for use of precision therapy for several primary immunodeficiencies. Patients with antibody deficiencies who rely on immu...
Source: Clinical Reviews in Allergy and Immunology - July 1, 2022 Category: Allergy & Immunology Source Type: research

A Summary on the Genetics of Systemic Lupus Erythematosus, Rheumatoid Arthritis, Systemic Sclerosis, and Sj ögren’s Syndrome
AbstractSystemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, and Sj ögren’s syndrome are four major autoimmune rheumatic diseases characterized by the presence of autoantibodies, caused by a dysregulation of the immune system that leads to a wide variety of clinical manifestations. These conditions present complex etiologies strongly influenced by multiple enviro nmental and genetic factors. The human leukocyte antigen (HLA) region was the first locus identified to be associated and still represents the strongest susceptibility factor for each of these conditions, particularly the HLA class II genes, ...
Source: Clinical Reviews in Allergy and Immunology - June 24, 2022 Category: Allergy & Immunology Source Type: research

Systemic Sclerosis-Specific Antibodies: Novel and Classical Biomarkers
AbstractDisease-specific autoantibodies are considered the most important biomarkers for systemic sclerosis (SSc), due to their ability to stratify patients with different severity and prognosis. Anti-nuclear antibodies (ANA), occurring in subjects with isolated Raynuad ’s phenomenon, are considered the strongest independent predictors of definite SSc and digital microvascular damage, as observed by nailfold videocapillaroscopy. ANA are present in more than 90% of SSc, but ANA negativity does not exclude SSc diagnosis: a little rate of SSc ANA negative exists and shows a distinct subtype of disease, with less vasculopath...
Source: Clinical Reviews in Allergy and Immunology - June 18, 2022 Category: Allergy & Immunology Source Type: research

Inflammaging and Osteoarthritis
AbstractOsteoarthritis is a highly prevalent disease particularly in subjects over 65  years of age worldwide. While in the past it was considered a mere consequence of cartilage degradation leading to anatomical and functional joint impairment, in recent decades, there has been a more dynamic view with the synovium, the cartilage, and the subchondral bone producing inflammatory med iators which ultimately lead to cartilage damage. Inflammaging is defined as a chronic, sterile, low-grade inflammation state driven by endogenous signals in the absence of infections, occurring with aging. This chronic status is linked to the...
Source: Clinical Reviews in Allergy and Immunology - June 18, 2022 Category: Allergy & Immunology Source Type: research

T Cell Abnormalities in X-Linked Agammaglobulinaemia: an Updated Review
AbstractX-linked agammaglobulinaemia (XLA) is a primary immunodeficiency (PID) resulting from a defect in the B cell development. It has conventionally been thought that T cells play a major role in the development and function of the B cell compartment. However, it has also been shown that B cells and T cells undergo bidirectional interactions and B cells also influence the structure and function of the T cell compartment. Patients with XLA offer a unique opportunity to understand the effect of absent B cells on the T cell compartment. In this review, we provide an update on abnormalities in the T cell compartment in pati...
Source: Clinical Reviews in Allergy and Immunology - June 16, 2022 Category: Allergy & Immunology Source Type: research

Correlates of Protection Against Respiratory Syncytial Virus Infection in Infancy
AbstractThe highest morbidity and mortality from respiratory syncytial virus (RSV) infection occurs in young infants. Immunization of expectant mothers during pregnancy has the potential to substantially reduce the burden of RSV disease in a majority of infants. Correlates of protection (COP) are important in guiding the development of maternal RSV vaccines and the design of maternal RSV vaccine trials, as immune response to candidate vaccines should mirror protective RSV immunity at birth. Here, we review the literature reporting correlations between RSV immune measures at birth and clinical RSV outcomes during infancy. L...
Source: Clinical Reviews in Allergy and Immunology - June 11, 2022 Category: Allergy & Immunology Source Type: research

Germinal Center-Related G Protein-Coupled Receptors in Antibody-Mediated Autoimmune Skin Diseases: from Basic Research to Clinical Trials
AbstractGerminal center (GC) reaction greatly contributes to the humoral immune response, which begins in lymph nodes or other secondary lymphoid organs after follicular B cells are activated by T-dependent antigens. The GCs then serve as a platform for follicular B cells to complete clonal expansion and somatic hypermutation and then interact with follicular dendritic cells (FDC) and follicular helper T cells (Tfh). Through the interaction between the immune cells, significant processes of the humoral immune response are accomplished, such as antibody affinity maturation, class switching, and production of memory B cells ...
Source: Clinical Reviews in Allergy and Immunology - June 8, 2022 Category: Allergy & Immunology Source Type: research

Serum Biomarkers for Autoimmune Hepatitis Type 1: the Case for CD48 and a Review of the Literature
In conclusion , sCD48 and sCD48-based predictive scores predict histological inflammation and fibrosis in AIH-1. Detecting sCD48 might help in the clinical management of AIH. (Source: Clinical Reviews in Allergy and Immunology)
Source: Clinical Reviews in Allergy and Immunology - June 3, 2022 Category: Allergy & Immunology Source Type: research

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis
AbstractScleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a mortality of 20% at 6  months. Once the leading cause of mortality in scleroderma (SSc), it remains a serious complication, often necessitating level three care for patients affected. Whilst renal outcomes have significantly improved following the advent of angiotensin-converting enzyme inhibitor (ACEi) therapy, SRC rem ains a precarious challenge for clinicians, due to lack of preventative measures and the fact that patients can rapidly decline despite best medical management. Large cohort studies spanning decades...
Source: Clinical Reviews in Allergy and Immunology - June 1, 2022 Category: Allergy & Immunology Source Type: research

Autoimmunity in Primary Immunodeficiencies (PID)
AbstractPrimary immunodeficiency (PID) may impact any component of the immune system. The number of PID and immune dysregulation disorders is growing steadily with advancing genetic detection methods. These expansive recognition methods have changed the way we characterize PID. While PID were once characterized by their susceptibility to infection, the increase in genetic analysis has elucidated the intertwined relationship between PID and non-infectious manifestations including autoimmunity. The defects permitting opportunistic infections to take hold may also lead the way to the development of autoimmune disease. In some...
Source: Clinical Reviews in Allergy and Immunology - June 1, 2022 Category: Allergy & Immunology Source Type: research