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Perspectives on Mycophenolate Mofetil in the Management of Autoimmunity
AbstractBefore becoming a cornerstone in the treatment of numerous immune-mediated diseases, mycophenolate mofetil (MMF) was first introduced as an immunosuppressive agent in transplant immunology and later received the attention of rheumatologists and clinicians involved in the management of autoimmune diseases. MMF is now a widespread immunosuppressive drug for the treatment of several conditions, including lupus nephritis, interstitial lung disease associated with systemic sclerosis, and anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis while being efficacious also as rescue therapy in various orphan disea...
Source: Clinical Reviews in Allergy and Immunology - June 20, 2023 Category: Allergy & Immunology Source Type: research

The Evolving Landscape of Fecal Microbial Transplantation
AbstractThe human gastrointestinal tract houses an enormous microbial ecosystem. Recent studies have shown that the gut microbiota plays significant physiological roles and maintains immune homeostasis in the human body. Dysbiosis, an imbalanced gut microbiome, can be associated with various disease states, as observed in infectious diseases, inflammatory diseases, autoimmune diseases, and cancer. Modulation of the gut microbiome has become a therapeutic target in treating these disorders. Fecal microbiota transplantation (FMT) from a healthy donor restores the normal gut microbiota homeostasis in the diseased host. Ample ...
Source: Clinical Reviews in Allergy and Immunology - February 9, 2023 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

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

Mesenchymal Stem Cell –Based Therapy as a New Approach for the Treatment of Systemic Sclerosis
AbstractSystemic sclerosis (SSc) is an intractable autoimmune disease with unmet medical needs. Conventional immunosuppressive therapies have modest efficacy and obvious side effects. Targeted therapies with small molecules and antibodies remain under investigation in small pilot studies. The major breakthrough was the development of autologous haematopoietic stem cell transplantation (AHSCT) to treat refractory SSc with rapidly progressive internal organ involvement. However, AHSCT is contraindicated in patients with advanced visceral involvement. Mesenchymal stem cells (MSCs) which are characterized by immunosuppressive,...
Source: Clinical Reviews in Allergy and Immunology - January 15, 2022 Category: Allergy & Immunology Source Type: research

Future of Therapy for Inborn Errors of Immunity
This article aims to review the future of therapy of PI/IEI (referred to IEI throughout this paper). Historically, immune deficiencies have been characterized as monogenic disorders resulting in immune deficiencies affecting T cells, B cells, combination of T and B cells, or innate immune disorders. More recently, immunologists are also recognizing a variety of phenotypes associated with one genotype or similar phenotypes across genotypes and a role for incomplete penetrance or variable expressivity of some genes causing inborn errors of immunity [3]. The IUIS classification of immune deficiencies (IEIs) has evolved over t...
Source: Clinical Reviews in Allergy and Immunology - January 12, 2022 Category: Allergy & Immunology Source Type: research

Update on the Pathogenesis and Therapy of Atopic Dermatitis
AbstractAtopic dermatitis (AD) is a common inflammatory skin disorder characterized by recurrent eczematous lesions and intense itch. Although it most often starts in infancy and affects children, it is also highly prevalent in adults. In this article, the main aspects of AD have been updated, with a focus on the pathogenetic and therapeutic aspects. The pathogenesis of AD is complex, and it is evident that a strong genetic predisposition, epidermal dysfunction, skin microbiome abnormalities, immune dysregulation, and the neuroimmune system are critical in AD development. Mutations in the genes associated with disrupted ep...
Source: Clinical Reviews in Allergy and Immunology - August 2, 2021 Category: Allergy & Immunology Source Type: research

Improving the Efficacy of Regulatory T Cell Therapy
AbstractAutoimmunity is caused by an unbalanced immune system, giving rise to a variety of organ-specific to system disorders. Patients with autoimmune diseases are commonly treated with broad-acting immunomodulatory drugs, with the risk of severe side effects. Regulatory T cells (Tregs) have the inherent capacity to induce peripheral tolerance as well as tissue regeneration and are therefore a prime candidate to use as cell therapy in patients with autoimmune disorders. (Pre)clinical studies using Treg therapy have already established safety and feasibility, and some show clinical benefits. However, Tregs are known to be ...
Source: Clinical Reviews in Allergy and Immunology - July 5, 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

Chronic Granulomatous Disease: a Comprehensive Review
AbstractChronic granulomatous disease (CGD) is a primary immunodeficiency of phagocyte function due to defective NADPH oxidase (phox). Compared with the common types ofCYBB/gp91phox,NCF1/p47phox, andCYBA/p22phox deficiency,NCF4/p40phox deficiency is a mild and atypical form of CGD without invasive bacterial or fungal infections. It can be diagnosed using serum-opsonizedE.coli as a stimulus in dihydrorhodamine (DHR) assay. Patients withCYBC1/Eros deficiency, a new and rare form of CGD, present as loss of respiratory burst and gp91phox expression in phagocytes. Neutrophils from patients with CGD are deficient in neutrophil e...
Source: Clinical Reviews in Allergy and Immunology - June 9, 2020 Category: Allergy & Immunology Source Type: research

Clinical Management of Primary Biliary Cholangitis —Strategies and Evolving Trends
AbstractPBC is a chronic progressive autoimmune disorder involving the destruction of intrahepatic small bile ducts, cholestasis, fibrosis, and ultimately cirrhosis if left untreated. It is largely driven by the autoimmune response, but bile acids and the intestinal microbiota are implicated in disease progression as well. The only drugs licensed for PBC are UDCA and OCA. UDCA as a first-line and OCA as a second-line therapy are safe and effective, but the lack of response in a significant portion of patients and inadequate control of symptoms such as fatigue and pruritus remain as concerns. Liver transplantation is an end...
Source: Clinical Reviews in Allergy and Immunology - November 10, 2019 Category: Allergy & Immunology Source Type: research

Clinical, Immunological, and Genetic Features in Patients with Activated PI3K δ Syndrome (APDS): a Systematic Review
AbstractActivated phosphoinositide 3-kinase delta syndrome (APDS) is a novel primary immunodeficiency (PID) caused by heterozygous gain of function mutations in PI3K δ catalytic p110δ (PIK3CD) or regulatory p85 α (PIK3R1) subunits leading to APDS1 and APDS2, respectively. Patients with APDS present a spectrum of clinical manifestations, particularly recurrent respiratory infections and lymphoproliferation. We searched PubMed, Web of Science, and Scopus databases for APDS patients and screened for eligibility criteria. A total of 243 APDS patients were identified from 55 articles. For all patients, demographic, clinical,...
Source: Clinical Reviews in Allergy and Immunology - May 20, 2019 Category: Allergy & Immunology Source Type: research

Geoepidemiology of Primary Biliary Cholangitis: Lessons from Switzerland
AbstractNo data on primary biliary cholangitis (PBC) are available in Switzerland. We established a national patient cohort to obtain information on PBC phenotypes and disease course in Switzerland. Local databases in all university hospitals and in two large secondary centers were searched for case finding. In addition, all primary care physicians, gastroenterologists, rheumatologists, and dermatologists were invited to contribute patients from their own medical records. PBC diagnosis was centrally reviewed. Five hundred one PBC patients were identified, 474 were included in data analysis, and 449 of them were enrolled by...
Source: Clinical Reviews in Allergy and Immunology - November 27, 2017 Category: Allergy & Immunology Source Type: research

Dermatological Complications After Solid Organ Transplantation
AbstractOrgan transplant recipients (OTRs) are a population at high risk for cutaneous adverse events. Their early recognition and appropriate treatment is an important component of the clinical management of OTRs and should be optimally dealt with by dermatologists working in the context of a transplant dermatology clinic. Skin examination should be a standard procedure before performing organ transplantation to assess conditions which may be difficult to manage after the transplant procedure has been performed or which may represent a contraindication to transplantation, e.g., malignant melanoma. It also offers an opport...
Source: Clinical Reviews in Allergy and Immunology - November 25, 2017 Category: Allergy & Immunology Source Type: research

Common Variable Immunodeficiency and Liver Involvement
AbstractCommon variable immunodeficiency (CVID) is a primary B-cell immunodeficiency disorder, characterized by remarkable hypogammaglobulinemia. The disease can develop at any age without gender predominance. The prevalence of CVID varies widely worldwide. The underlying causes of CVID remain largely unknown; primary B-cell dysfunctions, defects in T cells and antigen-presenting cells are involved. Although some monogenetic defects have been identified in some CVID patients, it is likely that CVID is polygenic. Patients with CVID develop recurrent and chronic infections (e.g., bacterial infections of the respiratory or ga...
Source: Clinical Reviews in Allergy and Immunology - August 7, 2017 Category: Allergy & Immunology Source Type: research

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