Gell & Coombs · immune pathways

Hypersensitivity & Autoimmunity

USMLE Step 1 · concise, high‑yield, clinically oriented

🔬 Overview

Hypersensitivity refers to tissue injury caused by an exaggerated or misdirected immune response. The first encounter with an antigen sensitizes the immune system; subsequent exposure triggers a damaging reaction. The four Gell & Coombs types are classified by the effector mechanism and the type of immune response.

Key concept: autoimmune diseases occur when self‑tolerance fails. Hypersensitivity reactions can be against foreign antigens (allergy) or self‑antigens (autoimmunity).

⚡ Type I · Immediate (IgE‑mediated)

fast mast cells atopy Response within minutes of re‑exposure. Mediated by IgE bound to FcεRI on mast cells, basophils, and eosinophils.

Pathophysiology

  • Sensitization: first exposure → Th2 cells produce IL‑4 and IL‑13 → B cells class‑switch to IgE.
  • Effector phase: allergen cross‑links IgE on mast cells → degranulation → histamine, proteases, prostaglandins, leukotrienes.
  • Late‑phase: 2–4 hours later, arachidonic acid metabolites and cytokines cause inflammation, leukocyte recruitment.

Clinical examples

  • Allergic rhinitis (hay fever)
  • Asthma (bronchoconstriction, mucus)
  • Anaphylaxis (insect stings, drugs)
  • Food allergies (urticaria, GI)
  • Wheal‑and‑flare skin tests
High‑yield: systemic anaphylaxis → laryngeal edema, hypotension, bronchospasm. Immediate epinephrine is first‑line.
Allergen Th2 → IL‑4, IL‑13 B cell IgE Mast cell sensitization Cross‑link → degranulation

Mediators: histamine (vasodilation, smooth muscle), heparin, prostaglandin D₂, leukotrienes C₄/D₄/E₄ (bronchospasm), LTB₄ (neutrophil chemotaxis).

🧬 Type II · Antibody‑mediated (cytotoxic / non‑cytotoxic)

IgG/IgM cell surface ECM Antibodies against cell‑surface or extracellular matrix antigens. Tissue damage localised to the antigen‑bearing tissue.

Mechanisms

  • Opsonisation & complement‑mediated lysis: Fc receptor phagocytosis, MAC formation.
  • Inflammatory cell recruitment: C3a, C5a, and FcγR trigger neutrophil/macrophage infiltration.
  • Functional alteration: antibody binding to receptor (e.g., TSH receptor) or enzyme (e.g., AChR) alters signalling without cell death.

Cytotoxic examples

  • Autoimmune hemolytic anemia (anti‑RBC)
  • HDNB (Rh incompatibility)
  • Goodpasture syndrome (anti‑collagen IV)
  • Transfusion reactions (ABO)

Non‑cytotoxic examples

  • Myasthenia gravis (anti‑AChR → muscle weakness)
  • Graves disease (anti‑TSHR → hyperthyroidism)
  • Pernicious anemia (anti‑intrinsic factor)
Classic HDNB: Rh‑negative mother with Rh‑positive fetus → maternal IgG crosses placenta in subsequent pregnancy → hemolysis. Prevent with RhoGAM (anti‑D) at 28 weeks and within 72h post‑partum.

🌀 Type III · Immune complex‑mediated

soluble Ag–Ab vasculitis nephritis Circulating immune complexes deposit in small vessels → complement activation, neutrophil recruitment, tissue injury. Usually systemic.

Key diseases

  • SLE (dsDNA, Sm, nucleoproteins) → nephritis, arthritis, malar rash
  • Post‑streptococcal glomerulonephritis (planted streptococcal Ag) → “lumpy‑bumpy” deposits
  • Serum sickness (foreign proteins) → arthritis, vasculitis, nephritis
  • Polyarteritis nodosa (HBV Ag) → systemic vasculitis
Pattern: immune complexes deposit where blood is filtered — glomeruli, joints, skin. “Lumpy‑bumpy” immunofluorescence.

🛡️ Type IV · T‑cell mediated (delayed)

CD4+ Th1/Th17 CD8+ CTL 48–72 h No antibody involvement. Tissue damage from cytokine‑activated macrophages, neutrophils, or direct cytotoxicity.

Th1 / Th17 mediated

  • Tuberculin test (PPD) – induration
  • Contact dermatitis (poison ivy, nickel)
  • Rheumatoid arthritis (synovial inflammation)
  • Crohn disease (Th1/Th17 driven)
  • Type 1 diabetes (β‑cell destruction)

CD8+ CTL mediated

  • Viral infections (hepatitis)
  • Graft rejection
  • Some autoimmune diseases (e.g., DM1)
Clinical pearl: Type IV reactions are delayed and are often diagnosed by skin testing (PPD) or patch testing. Corticosteroids and immunosuppressants are mainstays.

⚖️ Autoimmunity · pathogenesis

Autoimmunity arises from failure of central (thymus, bone marrow) and peripheral tolerance. Self‑reactive lymphocytes escape deletion and become activated.

Breakdown of tolerance

  • Anergy: self‑antigen recognition without co‑stimulation → lymphocyte inactivation.
  • Deletion: apoptosis via Fas/FasL or caspase pathways.
  • Suppression: Treg cells secrete IL‑10, TGF‑β; high CTLA‑4 sequesters B7 (CD80/86).
  • B cell tolerance: anergic B cells express high IgD, excluded from follicles → apoptosis.
Key regulators: CTLA‑4 (inhibitory), PD‑1, and Treg‑derived cytokines. Defects in these pathways are linked to autoimmunity.

Genetics & environmental triggers

HLA associations are the strongest genetic risk factors. Examples:

DiseaseHLA allele
Rheumatoid arthritisDR4
Type 1 diabetesDR3 / DR4
Ankylosing spondylitisB27
Celiac diseaseDQ2 / DQ8
SLEDR2 / DR3

Non‑HLA genes (e.g., PTPN22, CTLA‑4, IL‑2R) also contribute. Infections (molecular mimicry) and tissue injury expose self‑antigens, driving chronic progression.

💎 Clinical Pearls & High‑Yield Facts

  • Bee sting anaphylaxis → type I. Urticaria, bronchospasm, hypotension. First‑line: epinephrine.
  • Hashimoto thyroiditis → type IV (T‑cell mediated) with autoantibodies as markers (anti‑TPO, anti‑thyroglobulin).
  • Goodpasture syndrome → type II: anti‑collagen IV antibodies → linear immunofluorescence on kidney/lung biopsy.
  • Serum sickness → type III: fever, rash, arthralgias ~1‑2 weeks after antiserum or drug.
  • Contact dermatitis (poison ivy) → type IV. Treat with topical steroids.
  • HDNB prevention: RhoGAM at 28 weeks and within 72h of delivery/termination.
  • Autoimmunity associations: female preponderance, HLA‑linked, often relapsing‑remitting.

USMLE Tip: Distinguish type II (antibody to cell surface) from type III (soluble immune complexes). Type II is localised to the target tissue; type III is systemic (vessels, glomeruli). Type IV has no antibody involvement — DTH or CTL.

📋 Quick comparison table (click to expand)
TypeEffectorMechanismExamples
IIgE / mast cellsDegranulation, histamine, leukotrienesAnaphylaxis, asthma, hay fever
IIIgG/IgMOpsonisation, complement, functional blockadeHDNB, Goodpasture, myasthenia gravis
IIIIgG/IgM complexesComplement & neutrophil infiltrationSLE, serum sickness, PAN
IVT cells (Th1, Th17, CTL)Cytokine release, macrophage activation, cytolysisPPD, contact dermatitis, DM1