Animal models of primary biliary cirrhosis.


:Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα-/-, Scurfy, and Ae2a,b-/- mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.


Katsumi T,Tomita K,Leung PS,Yang GX,Gershwin ME,Ueno Y




Has Abstract


2015-06-01 00:00:00












  • New therapies for primary biliary cirrhosis.

    abstract::Primary biliary cirrhosis (PBC) is a rare inflammatory liver disease for which ursodeoxycholic acid (UDCA) is the only therapy approved by the U.S. Food and Drug Administration. Patients with a biochemical response to UDCA therapy have a similar survival rate compared to the general population. However, up to 40% of P...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Floreani A,Franceschet I,Perini L,Cazzagon N,Gershwin ME,Bowlus CL

    更新日期:2015-06-01 00:00:00

  • Allergy and Asthma Care in the Mobile Phone Era.

    abstract::Strategies to improve patients' adherence to treatment are essential to reduce the great health and economic burden of allergic rhinitis and asthma. Mobile phone applications (apps) for a better management of allergic diseases are growing in number, but their usefulness for doctors and patients is still debated. Contr...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,meta分析,评审


    authors: Huang X,Matricardi PM

    更新日期:2019-04-01 00:00:00

  • Mold and human health: separating the wheat from the chaff.

    abstract::The term "mold" is utilized to define the ubiquitous fungal species commonly found in household dust and observed as visible multicellular filaments. Several well-defined human diseases are known to be caused or exacerbated by mold or by exposure to their byproducts. Among these, a solid connection has been establishe...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Pettigrew HD,Selmi CF,Teuber SS,Gershwin ME

    更新日期:2010-04-01 00:00:00

  • The strategies used for treatment of experimental autoimmune neuritis (EAN): a beneficial effect of glatiramer acetate administered intraperitoneally.

    abstract::Glatiramer acetate (GA) significantly ameliorates multiple sclerosis and was initially discovered through its effects on the animal model experimental autoimmune encephalomyelitis (EAE). Guillain-Barré syndrome (GBS) is a relatively common demyelinating disease of peripheral nerves for which there is a parallel animal...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章


    authors: Aronovich R,Katzav A,Chapman J

    更新日期:2012-04-01 00:00:00

  • The clinical significance of 25OH-Vitamin D status in celiac disease.

    abstract::Reduced bone mineral density is frequently found especially in adult celiac disease (CD) and dietary guidelines favor vitamin D supplementation in adults and children with CD. Vitamin D serum levels were investigated in CD populations in order to challenge its routine supplementation. Israeli (61), Spanish (59), CD ch...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章


    authors: Lerner A,Shapira Y,Agmon-Levin N,Pacht A,Ben-Ami Shor D,López HM,Sanchez-Castanon M,Shoenfeld Y

    更新日期:2012-06-01 00:00:00

  • Autoinflammatory Pathogenesis and Targeted Therapy for Adult-Onset Still's Disease.

    abstract::Adult-onset Still's disease (AOSD) is a rare multisystem autoinflammatory disorder of unknown etiology. AOSD is generally characterized by high spiking fever, arthralgia or arthritis, skin rash, leukocytosis, and hyperferritinemia. Traditionally, AOSD has been treated with non-steroidal anti-inflammatory drugs, cortic...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Li S,Zheng S,Tang S,Pan Y,Zhang S,Fang H,Qiao J

    更新日期:2020-02-01 00:00:00

  • Do We Need Worms to Promote Immune Health?

    abstract::Many immune-mediated diseases like inflammatory bowel disease, multiple sclerosis, type 1 diabetes, asthma, and food allergy appeared to have increased in frequency in developed countries in the latter part of the twentieth century. Reports from less developed countries suggest that the "epidemic" of immune-mediated d...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Weinstock JV

    更新日期:2015-10-01 00:00:00

  • The arachidonate 12/15 lipoxygenases. A review of tissue expression and biologic function.

    abstract::12/15-Lipoxygenase is a highly regulated lipid-peroxidating enzyme whose expression and arachidonic acid metabolites are implicated in several important inflammatory conditions including airway and glomerular inflammation as well as atherosclerosis. Tissue expression of the original 12/15-lipoxygenase is well characte...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Conrad DJ

    更新日期:1999-04-01 00:00:00

  • A Review of Neutrophil Extracellular Traps (NETs) in Disease: Potential Anti-NETs Therapeutics.

    abstract::Activated 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 pat...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Mutua V,Gershwin LJ

    更新日期:2020-08-01 00:00:00

  • Update in the treatment of asthma during pregnancy.

    abstract::Asthma is the most common potentially serious medical problem to complicate pregnancy. Recent reports suggest that 7 of every 100 pregnant women suffer from asthma during pregnancy. Asthmatic women have been shown to be at an increased risk of complications during pregnancy. This may be secondary to inadequately contr...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Namazy JA,Schatz M

    更新日期:2004-06-01 00:00:00

  • Health Effects of Trimellitic Anhydride Occupational Exposure: Insights from Animal Models and Immunosurveillance Programs.

    abstract::Acid anhydrides are used by chemical industries as plasticizers. Trimellitic acid (TMA) is an acid anhydride widely utilized in factories to produce paints, varnishes, and plastics. In addition to causing direct irritant effects, TMA can augment antibody responses in exposed factory workers leading to occupational ast...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Ghosh D,Bernstein JA

    更新日期:2020-08-01 00:00:00

  • Infectious serologies and autoantibodies in hepatitis C and autoimmune disease-associated mixed cryoglobulinemia.

    abstract::Mixed cryoglobulinemia (MC) syndrome is an immune complex-mediated vasculitis characterized by the clinical triad of purpura, weakness, and arthralgias, the morbidity of which is mainly related to kidney and peripheral nervous system dysfunction as well as to the development of a secondary lymphoma (Ferri et al. Autoi...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Lidar M,Lipschitz N,Agmon-Levin N,Langevitz P,Barzilai O,Ram M,Porat-Katz BS,Bizzaro N,Damoiseaux J,Tervaert JW,deVita S,Bombardieri S,Shoenfeld Y

    更新日期:2012-04-01 00:00:00

  • Seasonal variation in bronchial hyperreactivity (BHR) in allergic patients.

    abstract::As summarized in Table 1, the literature consistently supports the hypothesis that allergic asthmatic patients have seasonal BHR changes that parallel allergen exposure. These seasonal changes appear to be preventable by treatment with corticosteroids (systemic, inhaled, or nasal), disodium cromoglycate, and immunothe...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Tilles SA,Bardana EJ Jr

    更新日期:1997-07-01 00:00:00

  • A review of non-cystic fibrosis pediatric bronchiectasis.

    abstract::With the implementation of vaccination programs and the use of antibiotics, developed countries have seen a decline in infection-related pediatric bronchiectasis. However, significant morbidity from bronchiectasis is still seen and both infectious and noninfectious causes of bronchiectasis in the pediatric population ...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Boren EJ,Teuber SS,Gershwin ME

    更新日期:2008-04-01 00:00:00

  • Forkhead Box O1 Regulates Macrophage Polarization Following Staphylococcus aureus Infection: Experimental Murine Data and Review of the Literature.

    abstract::The functions of macrophages that lead to effective host responses are critical for protection against Staphylococcus aureus. Deep tissue-invading S. aureus initially countered by macrophages trigger macrophage accumulation and induce inflammatory responses through surface receptors, especially toll-like receptor 2 (T...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,meta分析,评审


    authors: Wang YC,Ma HD,Yin XY,Wang YH,Liu QZ,Yang JB,Shi QH,Sun B,Gershwin ME,Lian ZX

    更新日期:2016-12-01 00:00:00

  • The acute management of asthma.

    abstract::Patients presenting to the emergency department (ED) or clinic with acute exacerbation of asthma (AEA) can be very challenging varying in both severity and response to therapy. High-dose, frequent or continuous nebulized short-acting beta2 agonist (SABA) therapy that can be combined with a short-acting muscarinic anta...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Albertson TE,Sutter ME,Chan AL

    更新日期:2015-02-01 00:00:00

  • IL-23/IL-17 Axis in Inflammatory Rheumatic Diseases.

    abstract::In 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 ...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Li H,Tsokos GC

    更新日期:2021-02-01 00:00:00

  • Autoimmunity in 2018.

    abstract::In the vast database of peer-reviewed articles, the number of 2018 papers published retrieved using the "autoimmunity" keyword remained unchanged compared with the brilliant results of 2017 while returning above a 5% share within the immunology field, after the brisk decrease of this ratio in 2017. As in the past 12 y...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章


    authors: Selmi C

    更新日期:2019-06-01 00:00:00

  • Irritant Contact Dermatitis.

    abstract::Contact dermatitis accounts for 95% of occupational skin disorders. Irritant contact dermatitis (ICD) is often caused by cumulative exposure to weak irritants, accounting for 80% of all cases of contact dermatitis. ICD can co-exist with atopic dermatitis (AD) and allergic contact dermatitis (ACD). Patients with AD and...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Bains SN,Nash P,Fonacier L

    更新日期:2019-02-01 00:00:00

  • Diagnostics and treatment of cryoglobulinaemia: it takes two to tango.

    abstract::Cryoglobulins are immunoglobulins that reversibly precipitate in the cold. They come in different flavours and, as such, are differentially associated with lymphoproliferative diseases (type I), or systemic autoimmune diseases, and/or infectious diseases (type II/III). The clinical manifestations of cryoglobulinaemia ...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Damoiseaux J,Cohen Tervaert JW

    更新日期:2014-12-01 00:00:00

  • The clinical significance of antityrosinase antibodies in melanoma and related hypopigmentary lesions.

    abstract::Antityrosinase antibody is a newly detected antibody in the sera of patients with melanoma or vitiligo. The serum level of the antibody is measured by enzyme-linked immunosorbent assay (ELISA). The autoantigen is tyrosinase itself, the enzyme that participates in pigment (melanin) formation by both melanocytes and mel...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Merimsky O,Shoenfeld Y,Fishman P

    更新日期:1998-10-01 00:00:00

  • A Comprehensive Review of Celiac Disease/Gluten-Sensitive Enteropathies.

    abstract::Celiac disease is a complex immune-mediated gluten-sensitive enteropathy with protean clinical manifestations. It is manifest in genetically predisposed individuals who ingest gluten in varying amounts. In broad terms, it is thought to affect 1% of the population in the USA. More specifically, the prevalence increases...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: McAllister BP,Williams E,Clarke K

    更新日期:2019-10-01 00:00:00

  • Management of haemophilia A-inhibitor patients: clinical and regulatory perspectives.

    abstract::Inhibitors to factor VIII (FVIII) are alloantibodies directed against epitopes able to neutralise FVIII procoagulant activity. They may render FVIII replacement therapy ineffective. They represent the most severe complication of haemophilia A. At least three mechanisms of FVIII neutralisation activity by anti-FVIII an...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Tellier Z,André MH,Polack B

    更新日期:2009-10-01 00:00:00

  • A conspicuous role for B cells In Sjögren's syndrome.

    abstract::Although the relative contributions of T cells and B cells in Sjögren's syndrome (SS) are far from being settled, recent studies have suggested a crucial role for B cells in its pathophysiology. Early investigations have focused on the ability of B cells to produce autoantibodies, and new studies have enlarged the ran...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Youinou P,Devauchelle V,Hutin P,Le Berre R,Saraux A,Pers JO

    更新日期:2007-06-01 00:00:00

  • Obstructive sarcoidosis.

    abstract::Sarcoidosis is a systemic granulomatous disease that frequently involves the lung. Although classically thought of as a restrictive lung disease, airway obstruction has become a recognized feature of the disease in the past years. Sarcoidosis can affect the airway at any level and when the involvement includes small a...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Laohaburanakit P,Chan A

    更新日期:2003-10-01 00:00:00

  • Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and Novel Therapeutic Approaches.

    abstract::Pemphigus is a group of rare, potentially devastating autoimmune diseases of the skin and mucous membranes with high morbidity and potentially lethal outcome. The major clinical variant, pemphigus vulgaris (PV) is caused by a loss of intercellular adhesion of epidermal keratinocytes which is induced by IgG autoantibod...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Pollmann R,Schmidt T,Eming R,Hertl M

    更新日期:2018-02-01 00:00:00

  • Concluding remarks: can we explain the association of beta-agonists with asthma mortality? A hypothesis.

    abstract::Beta-agonists have clearly demonstrated benefits for the treatment of both acute and chronic asthma. Therefore, it is perhaps surprising that many of the articles in this issue have focused on concerns about their safety. Much of this concern can be traced back to the "beta-agonist controversy"--the association of hig...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章


    authors: Hancox RJ

    更新日期:2006-10-01 00:00:00

  • Sex in Respiratory and Skin Allergies.

    abstract::A bulk of literature demonstrated that respiratory allergy, and especially asthma, is prevalent in males during childhood, while it becomes more frequent in females from adolescence, i.e., after menarche, to adulthood. The mechanisms underlying the difference between females and males are the effects on the immune res...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Ridolo E,Incorvaia C,Martignago I,Caminati M,Canonica GW,Senna G

    更新日期:2019-06-01 00:00:00

  • Tumor Necrosis Factor-Alpha and Pregnancy: Focus on Biologics. An Updated and Comprehensive Review.

    abstract::Tumor necrosis factor-α (TNF-α) is a central regulator of inflammation, and TNF-α antagonists may be effective in treating inflammatory disorders in which TNF-α plays a major pathogenic role. TNF-α has also been associated with inflammatory mechanisms related to implantation, placentation, and pregnancy outcome. TNF-α...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Alijotas-Reig J,Esteve-Valverde E,Ferrer-Oliveras R,Llurba E,Gris JM

    更新日期:2017-08-01 00:00:00

  • The Heart and APS.

    abstract::The heart is a target organ in antiphospholipid syndrome (APS). Endocardial disease, intracardiac thrombosis, myocardial involvement including coronary heart disease and microvascular thrombosis, as well as pulmonary hypertension have all been described in APS patients. Valvular involvement is the most common manifest...

    journal_title:Clinical reviews in allergy & immunology

    pub_type: 杂志文章,评审


    authors: Amigo MC

    更新日期:2007-04-01 00:00:00