Immune Complex Mediated Diseases

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Immune complexes that cause disease may be composed of antibodies bound to either self antigens or foreign antigens. The pathologic features of diseases caused by immune complexes reflect the site of immune complex deposition and are not determined by the cellular source of the antigen. Therefore, immune complex-mediated diseases tend to affect multiple tissues and organs, although some are particularly susceptible, such as kidneys and joints.

The occurrence of diseases caused by immune complexes was suspected as early as 1911 by an astute physician named Clemens von Pirquet. At that time, diphtheria infections were being treated with serum from horses immunized with the diphtheria toxin, which is an example of passive immunization against the toxin by the transfer of serum containing antitoxin antibodies. von Pirquet noted that joint inflammation (arthritis), rash, and fever developed in patients injected with the antitoxin-containing horse serum. Two clinical features of this reaction suggested that it was not due to the infection or a toxic component of the serum itself. First, these symptoms appeared even after the injection of horse

TABLE 18-2 Examples of Diseases Caused by Cell- or Tissue-Specific Antibodies

Disease

Target Antigen

Mechanisms of Disease

Clinicopathologic Manifestations

Autoimmune hemolytic anemia

Erythrocyte membrane proteins (Rh blood group antigens, I antigen)

Opsonization and phagocytosis of erythrocytes, complement-mediated lysis

Hemolysis, anemia

Autoimmune thrombocytopenic purpura

Platelet membrane proteins (gpNb-INa integrin)

Opsonization and phagocytosis of platelets

Bleeding

Pemphigus vulgaris

Proteins in intercellular junctions of epidermal cells (desmoglein)

Antibody-mediated activation of proteases, disruption of intercellular adhesions

Skin vesicles (bullae)

Vasculitis caused by ANCA

Neutrophil granule proteins, presumably released from activated neutrophils

Neutrophil degranulation and inflammation

Vasculitis

Goodpasture's syndrome

Non-collagenous NC1 protein of basement membrane in glomeruli and lung

Complement- and Fc receptor-mediated inflammation

Nephritis, lung hemorrhage

Acute rheumatic fever

Streptococcal cell wall antigen; antibody cross-reacts with myocardial antigen

Inflammation, macrophage activation

Myocarditis, arthritis

Myasthenia gravis

Acetylcholine receptor

Antibody inhibits acetylcholine binding, downmodulates receptors

Muscle weakness, paralysis

Graves' disease (hyperthyroidism)

TSH receptor

Antibody-mediated stimulation of TSH receptors

Hyperthyroidism

Insulin-resistant diabetes

Insulin receptor

Antibody inhibits binding of insulin

Hyperglycemia, ketoacidosis

Pernicious anemia

Intrinsic factor of gastric parietal cells

Neutralization of intrinsic factor; decreased absorption of vitamin b12

Abnormal erythropoiesis, anemia

ANCA, antineutrophil cytoplasmic antibodies; TSH, thyroid-stimulating hormone.

Anti-basement membrane antibody-mediated glomerulonephritis

Immune complex mediated glomerulonephritis

Graves Disease Antibodies

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Arthritis Joint Pain

Arthritis Joint Pain

Arthritis is a general term which is commonly associated with a number of painful conditions affecting the joints and bones. The term arthritis literally translates to joint inflammation.

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Responses

  • ALICE LEEPER
    Is rheumatoid arthritis an immune complex mediated disease?
    2 years ago
  • russom saare
    How did horse serum cause immune complex disease?
    11 months ago
  • marmaduke
    Which of the following is an example of an immunecomplexmediated disease?
    21 hours ago

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