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Pediatric Blood & Cancer Oct 2006Bleeding and isolated thrombocytopenia in idiopathic thrombocytopenic purpura (ITP) are phenomena of a transient or long-term disturbed immune response. Since 1980, ITP... (Review)
Review
Bleeding and isolated thrombocytopenia in idiopathic thrombocytopenic purpura (ITP) are phenomena of a transient or long-term disturbed immune response. Since 1980, ITP has become a model for targeted therapeutic immunomodulation with still unclear mechanisms of action. The systematic analysis of ITP aims to determine more clearly defined patient subgroups for targeted therapy and to explore the complex immune disturbances in this autoimmune disorder.
Topics: Autoimmune Diseases; Biomedical Research; Humans; Inflammation; Purpura, Thrombocytopenic, Idiopathic
PubMed: 16933261
DOI: 10.1002/pbc.20969 -
Critical Reviews in Immunology 2019Childhood exposure to traumatic events, termed early life stress (ELS), is now widely recognized for causing long-term negative health effects that may not manifest... (Review)
Review
Childhood exposure to traumatic events, termed early life stress (ELS), is now widely recognized for causing long-term negative health effects that may not manifest until adulthood. Allostatic load (AL) describes the cumulative "wear-and-tear" effects of chronic stress on the body that may adversely affect human health by accelerating other disease processes. Recent epidemiological studies have reported higher stress levels in industrialized countries and trends of increasing prevalence in autoimmune diseases during recent decades. To elucidate mechanisms of stress-related immune dysregulation, most animal studies up to now have focused on AL and stress-triggered events occurring in adults but have not explored ELS in the context of autoimmune disorders. We have identified a current gap in understanding the impact of ELS on immune system ontogeny and its potential for priming genetically susceptible individuals who are at increased risk for autoimmune diseases later in life, through mechanisms involving neuroendocrine-immune cross talk. In this review, we highlight the intersection between stress and immune function, with a focus on ELS as consequential for increased autoimmune disorder risks later in life.
Topics: Adult; Animals; Autoimmune Diseases; Child; Gene-Environment Interaction; Genetic Predisposition to Disease; Humans; Prevalence; Stress, Psychological
PubMed: 32422015
DOI: 10.1615/CritRevImmunol.2020033244 -
Revista Colombiana de Psiquiatria... 2022Since 1980, there have been known cases of childhood neuropsychiatric syndromes in the world and its concept has evolved with changes in the definitions in 1995 (PITANDs...
INTRODUCTION
Since 1980, there have been known cases of childhood neuropsychiatric syndromes in the world and its concept has evolved with changes in the definitions in 1995 (PITANDs - paediatric infection-triggered autoimmune neuropsychiatric disorders), 1998 (PANDAS - paediatric autoimmune neuropsychiatric syndrome associated with streptococci infection), 2010 (PANS - paediatric acute-onset neuropsychiatric syndrome) and 2012 (CANS - childhood acute neuropsychiatric syndrome). Despite being known for more than 20 years, it is still an illness that often goes unnoticed by many health professionals.
OBJECTIVE
To sensitise the medical community about the identification of the disease and reduce the morbidity associated with a late diagnosis.
CLINICAL CASE
A 6-year-old schoolgirl brought to the emergency department due to her refusal to eat. In the hospital treatment, a clinical history was identified with PANS-PANDAS diagnostic criteria. She exhibited a relapsing-remitting clinical course, as described in the literature, with poor response to first-line treatments.
CONCLUSIONS
In all school-age child presenting with obsessive compulsive disorder or eating disorders, with other symptoms or not, a possible link to PANS-CANS should be evaluated and ruled out.
Topics: Female; Child; Humans; Obsessive-Compulsive Disorder; Streptococcal Infections; Autoimmune Diseases; Syndrome
PubMed: 36446706
DOI: 10.1016/j.rcpeng.2020.11.013 -
Proceedings of the Japan Academy.... 2018IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multi-organ involvement.... (Review)
Review
IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder recognized as a novel clinical entity with either synchronous or metachronous multi-organ involvement. Patients with IgG4-RD show diffuse or focal organ enlargement and mass-forming or nodular/thickened lesions with abundant infiltration of IgG4-positive plasmacytes and fibrosis, and such patients respond well to steroid treatment. It should be differentiated from mimics by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line drug is corticosteroids, or rituximab in high-risk patients for steroid intolerance. Although relapse rates are high, standardized protocols for relapsed cases have not been approved yet. Based on genetic factors, disease-specific or -related antigens, abnormal innate and adaptive immunity may be involved, although the precise pathogenic mechanism and long-term outcome still remain unclear.
Topics: Autoimmune Diseases; Humans; Immunoglobulin G4-Related Disease; Pancreatitis
PubMed: 30541967
DOI: 10.2183/pjab.94.027 -
Frontiers in Immunology 2023Autoimmune blistering diseases (AIBD) are paradigms of autoantibody-mediated organ-specific autoimmune disorders that involve skin and/or mucous membranes. Compared to... (Review)
Review
Autoimmune blistering diseases (AIBD) are paradigms of autoantibody-mediated organ-specific autoimmune disorders that involve skin and/or mucous membranes. Compared to other autoimmune diseases, the pathogenicity of autoantibodies in AIBD is relatively well described. Pemphigus is a potentially lethal autoantibody driven autoimmune disorder with a strong HLA class II association. It is mainly characterized by IgG against the desmosomal adhesion molecules desmoglein 3 (Dsg3) and Dsg1. Several murine pemphigus models were developed subsequently, each allowing the analysis of a characteristic feature, such as pathogenic IgG or Dsg3-specific T or B cells. Thus, the models can be employed to preclinically evaluate potentially novel therapies. We here thoroughly summarize past and recent efforts in developing and utilizing pemphigus mouse models for pathomechanistic investigation and therapeutic interventions.
Topics: Mice; Animals; Pemphigus; Autoantibodies; Skin; Autoimmune Diseases; Blister; Disease Models, Animal; Immunoglobulin G
PubMed: 37180099
DOI: 10.3389/fimmu.2023.1169947 -
Anales de Medicina Interna (Madrid,... Jul 2004Autoimmune hepatitis (AIH) is a hepatocellular inflammation that is characterised by a wide range of histopathologic (periportal interface hepatitis with plasma cell... (Review)
Review
Autoimmune hepatitis (AIH) is a hepatocellular inflammation that is characterised by a wide range of histopathologic (periportal interface hepatitis with plasma cell infiltration and piecemeal necrosis), biochemical (hypertransaminasemia, hypergammaglobulinaemia) and autoimmune (several autoantibodies presence) features. This relatively rare disorder frequently affects middle-aged women. There is no pathognomonic marker for AIH diagnosis, therefore it requires a careful rule out of other causes of liver disease together with the detection of a suggestive pattern of clinical and laboratory abnormalities. Scoring system for AIH diagnosis proposed by International Autoimmune Hepatitis Group has been used as a tool in clinical practice but is not sufficiently exclusive in terms of defining prognosis or treatment. AIH has been classified in two subtypes according to autoantibodies detected: 1 and 2, but this classification results in poor clinical implications. Previously known as subtype 3 is at the present included in subtype 1 because no clinical significant differences has been found between them. Aetiology, and molecular mechanisms still remain to be elucitaded in this disease, although viruses, drugs and molecular mimicry act presumably as a trigger in genetically predisposed patients (associated with HLA-DR3 and DR4 haplotypes). On the other hand, immunosuppressive therapy (corticosteroid or azathioprine) generally offers favourable response. Our aim is to review this disease from different points of view, considering: clinical, histopathological, etiologic, genetic, biochemical, autoimmune, treatment and prognosis features.
Topics: Autoantibodies; Autoimmune Diseases; Hepatitis; Humans; Prognosis
PubMed: 15347241
DOI: 10.4321/s0212-71992004000700008 -
British Journal of Haematology Oct 2004Acquired Glanzmann's thrombasthenia (GT) is an uncommon accompaniment to immune thrombocytopenic purpura. Rarely, GT may present as an acquired autoimmune disorder of... (Review)
Review
Acquired Glanzmann's thrombasthenia (GT) is an uncommon accompaniment to immune thrombocytopenic purpura. Rarely, GT may present as an acquired autoimmune disorder of platelet function, with rapid onset of a moderate-to-severe bleeding tendency, a prolonged bleeding time, but with a normal platelet count and normal platelet glycoprotein (GP) expression. This is caused by an autoantibody with specificity for platelet GP IIb/IIIa or an epitope close to that of the GP, resulting in partial or complete refractoriness of the patient's platelets to ADP, collagen and arachidonic acid. We describe two patients with acquired GT and a normal platelet count, who presented with severe bleeding. The first patient responded gradually to immunosuppressive treatment but eventually developed non-Hodgkin's lymphoma. The second patient had no other underlying conditions and remitted spontaneously within 2 years.
Topics: Aged; Aged, 80 and over; Autoantibodies; Autoimmune Diseases; Bleeding Time; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Platelet Count; Platelet Glycoprotein GPIIb-IIIa Complex; Remission, Spontaneous; Thrombasthenia
PubMed: 15461628
DOI: 10.1111/j.1365-2141.2004.05173.x -
Clinical and Experimental Rheumatology Mar 2023Dermatomyositis (DM) is an autoimmune disorder in which clinically amyopathic DM, characterised by hallmark cutaneous findings in the absence of clinical weakness,... (Review)
Review
Dermatomyositis (DM) is an autoimmune disorder in which clinically amyopathic DM, characterised by hallmark cutaneous findings in the absence of clinical weakness, represents 20% of patients. This review will highlight current concepts and recent advances made in DM from a dermatological perspective, with a discussion of skin-predominant DM and its distinct challenges regarding diagnosis and management as well as their implications in clinical trials. An update will be presented with respect to classification criteria, pathogenesis in cutaneous DM, myositis-specific autoantibodies and their associations with cutaneous findings, skin-specific outcome measures and new therapeutics with their efficacy in skin disease.
Topics: Humans; Dermatomyositis; Skin; Myositis; Autoimmune Diseases; Autoantibodies
PubMed: 36622138
DOI: 10.55563/clinexprheumatol/ue71ku -
Thyroid : Official Journal of the... Jan 2002
Review
Topics: Animals; Autoimmune Diseases; Connective Tissue Diseases; Eye Diseases; Graves Disease; Humans; Muscles; Orbit
PubMed: 11838728
DOI: 10.1089/105072502753451940 -
BMC Medicine Apr 2013Connective tissue diseases (CTDs) are a heterogeneous group of disorders that share certain clinical presentations and a disturbed immunoregulation, leading to... (Review)
Review
Connective tissue diseases (CTDs) are a heterogeneous group of disorders that share certain clinical presentations and a disturbed immunoregulation, leading to autoantibody production. Subclinical or overt renal manifestations are frequently observed and complicate the clinical course of CTDs. Alterations of kidney function in Sjögren syndrome, systemic scleroderma (SSc), auto-immune myopathies (dermatomyositis and polymyositis), systemic lupus erythematosus (SLE), antiphospholipid syndrome nephropathy (APSN) as well as rheumatoid arthritis (RA) are frequently present and physicians should be aware of that.In SLE, renal prognosis significantly improved based on specific classification and treatment strategies adjusted to kidney biopsy findings. Patients with scleroderma renal crisis (SRC), which is usually characterized by severe hypertension, progressive decline of renal function and thrombotic microangiopathy, show a significant benefit of early angiotensin-converting-enzyme (ACE) inhibitor use in particular and strict blood pressure control in general. Treatment of the underlying autoimmune disorder or discontinuation of specific therapeutic agents improves kidney function in most patients with Sjögren syndrome, auto-immune myopathies, APSN and RA.In this review we focus on impairment of renal function in relation to underlying disease or adverse drug effects and implications on treatment decisions.
Topics: Autoimmune Diseases; Connective Tissue Diseases; Humans; Immunologic Factors; Kidney Diseases
PubMed: 23557013
DOI: 10.1186/1741-7015-11-95