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When the Immune System Fails: Mechanisms of Immune Dysfunction

1Normal Immune Defense as a Layered System2Barrier and Innate Failure: When the First Lines Collapse3B-Cell and Antibody Failure4T-Cell and Thymic Failure5Regulatory Failure: Autoimmunity and Allergy
Regulatory Failure: Autoimmunity and Allergy

How Self-Reactive Effectors Damage Tissue

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Watch the pathway on the left. An autoantibody binds a self antigen on a cell surface, and from there three things can happen. Complement is activated, generating C3a and C5a that call in neutrophils and assembling the membrane attack complex that punches a hole in the cell. The Fc portion of the bound antibody engages Fc receptors on macrophages, which either engulf the opsonized cell or release lytic enzymes and reactive oxygen species. And in some cases, as in Graves disease, the antibody does not destroy the cell at all — it mimics the natural ligand and chronically stimulates it. Now watch the pathway on the right. A CD8 T cell recognizes self peptide on MHC class I and kills the presenting cell with perforin and granzyme, exactly as it would kill a virus-infected cell; in type 1 diabetes this is what destroys the beta cells. A CD4 T cell recognizing self peptide on MHC class II does not usually kill directly — it recruits and activates macrophages, and the cytokines it releases sustain a self-amplifying inflammatory lesion. The mechanism is the standard antimicrobial repertoire; only the target has changed.
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Once tolerance fails, the effector machinery that normally clears pathogens is redirected at self tissue. The damage is not a new mechanism; it is the standard effector repertoire applied to the wrong target.

Autoantibodies cause damage through the same pathways used against microbes. IgG bound to self antigen on a cell surface activates complement, generating C3a and C5a that recruit neutrophils and assembling the membrane attack complex that lyses the cell. The Fc portion of bound IgG engages Fc receptors on macrophages and neutrophils, driving phagocytosis of the opsonized cell or release of lytic enzymes and reactive oxygen species. In Graves disease, an autoantibody against the TSH receptor does not destroy the cell at all — it mimics the ligand and chronically stimulates the thyroid, an example of agonist autoantibody. In myasthenia gravis, anti-acetylcholine receptor antibodies accelerate receptor internalization and activate complement at the neuromuscular junction, reducing signal transmission.

Self-reactive T cells cause damage by direct cytotoxicity and by cytokine-driven inflammation. CD8 cytotoxic T cells recognizing self peptide on MHC class I kill the presenting cell through perforin and granzyme, exactly as they would kill a virus-infected cell. In type 1 diabetes, this destroys insulin-producing beta cells in the pancreatic islets. CD4 helper T cells recognizing self peptide on MHC class II do not usually kill directly; they recruit and activate macrophages and neutrophils, producing the chronic inflammatory infiltrate seen in rheumatoid synovium or multiple sclerosis plaques. The cytokines these cells release — IFN-gamma, TNF, IL-17 — sustain the inflammation and recruit more effectors, so the lesion becomes self-amplifying.

Two features distinguish this from immunodeficiency. First, the damage is antigen-specific and directed at a defined self target, so it produces organ-specific disease rather than broad susceptibility to infection. Second, because the effector arm is intact, the inflammation is active and often chronic, waxing and waning with the availability of the self antigen and the strength of the regulatory brake.

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