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Related Concept Videos

Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

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Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum...
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Complement System01:27

Complement System

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The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
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Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

648
Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
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Drug Toxicity: Allergic Reactions01:30

Drug Toxicity: Allergic Reactions

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Drug-related allergies are immune-mediated responses triggered by the administration of pharmacological agents. These hypersensitivity reactions are classified based on the immune mechanisms involved. The four primary types—Type I, II, III, and IV—are mediated by different immunological pathways and exhibit distinct clinical manifestations.Type I Hypersensitivity/ IgE-Mediated Reactions: Immunoglobulin E (IgE) immediately mediates Type I hypersensitivity reactions. Upon initial...
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Hypersensitivities01:30

Hypersensitivities

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Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
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Granulocyte-dependent Autoantibody-induced Skin Blistering
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[Hypocomplementemic urticarial vasculitis].

M Jachiet1, B Flageul1, J-D Bouaziz1

  • 1Service de dermatologie, faculté de médecine, hôpital Saint-Louis, université Paris-Diderot, Assistance publique-Hôpitaux de Paris, 75010 Paris, France.

La Revue De Medecine Interne
|May 2, 2017
PubMed
Summary
This summary is machine-generated.

Hypocomplementemic urticarial vasculitis (HUV) is a rare small vessel vasculitis. Current treatments include hydroxychloroquine, colchicine, immunosuppressants, and rituximab, though optimal management strategies require further definition.

Keywords:
Anti-C1q antibodiesAnticorps anti-C1qCorticosteroidsCorticothérapieLupus érythémateux systémiqueRituximabSystemic lupus erythematosusVasculariteVasculitis

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Area of Science:

  • Rheumatology
  • Immunology
  • Dermatology

Background:

  • Hypocomplementemic urticarial vasculitis (HUV), also known as anti-C1q vasculitis, is a rare systemic small vessel vasculitis of unknown cause.
  • Key features include urticarial lesions, hypocomplementemia, and systemic involvement (musculoskeletal, ocular, GI, pulmonary, renal).

Purpose of the Study:

  • To review the current understanding of Hypocomplementemic urticarial vasculitis (HUV).
  • To summarize the limited published data on therapeutic management strategies for HUV.

Main Methods:

  • Literature review of published data on HUV.
  • Analysis of reported therapeutic interventions and outcomes.

Main Results:

  • Low C1q levels appear more sensitive than anti-C1q antibodies for HUV diagnosis.
  • Hydroxychloroquine and colchicine show promise as first-line treatments.
  • For refractory cases, corticosteroids with immunosuppressants (azathioprine, mycophenolate mofetil, cyclophosphamide) or rituximab demonstrate efficacy.

Conclusions:

  • The optimal treatment strategy for HUV remains undefined.
  • Further research is needed to establish evidence-based guidelines for HUV management.