COMPLEMENTS

• The complement system plays an important role in innate immune response by binding to (opsonizing) and destroying pathogens, and recruiting other immune cells.
• Common complement tests including CH50 and serum C3 and C4 levels.
   CH50: or total hemolytic complement (THC) is a functional assay used to detect any deficiency of the complements of the classical pathway
   Serum C3 and C4 levels: used to identify certain autoimmune diseases causing a decrease in complement levels due to immune complexes formation
• Hypocomplementemia (low serum C3 and/or C4 levels) might result from decreased production (in inherited complement deficiencies or liver failure) or increased consumption by immune complexes (in various systemic autoimmune diseases and glomerular diseases).


HYPOCOMPLEMENTEMIA

✿ Immune complexes formation
  ✧ systemic lupus erythematosus)
  ✧ antiphospholipid syndrome
  ✧ immune hemolytic anemia
  ✧ thrombotic thrombocytopenic purpura
  ✧ IgG4-related diseases
  ✧ hereditary angioedema (c1 inhibitor deficiency)
  ✧ systemic vasculitis
    ☼ polyarteritis nodosa
    ☼ hypocomplementemic urticarial vasculitis
  ✧ cryoglobulinemia
    ☼ hcv/hbv infection
    ☼ monoclonal gammopathy (mgus, myeloma)
    ☼ hypocomplementemic urticarial vasculitis
  ✧ glomerular disease
    ☼ poststreptococcal glomerulonephritis
    ☼ membranoproliferative glomerulonephritis
    ☼ goodpasture's syndrome
    ☼ c3 nephritic factor
    ☼ c4 nephritic factor
    ☼ c3 glomerulopathy
    ☼ dense deposit disease
    ☼ IgA nephropathy
  ✧ serum sickness
    ☼ biologics (rituximab, infliximab)
    ☼ thrombolytics (streptokinase)
    ☼ anti-toxins (rabies, botulinum, snake venoms)
    ☼ insect bites
  ✧ serum sickness-like reactions
    ☼ drugs (cefalor, penicillin, tmp/smx)
    ☼ vaccinations (rabies)
    ☼ viral infections (hbv/hcv, parvovirus)


✿ Decreased production
  ✧ hereditary complement deficiencies
  ✧ liver failure
  ✧ severe malnutrition

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