Immunology 334Y

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Inherited Complement Component Deficiencies

• C1q associated with lupus (SLE)

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Inherited Complement Component Deficiencies

• C1q
• C1r associated with lupus (SLE
• C2
• C1s

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Inherited Complement Component Deficiencies

• C1 Inhibitor Deficiency
• Hereditary Angioneurotic Edema
• - activation of complement
• and release of vasoactive fragments
• - recurrent swelling of lips and pharynx

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Pathogenesis of Hereditary Angioedema
Cicardi, M. et. al. N Engl J Med
19963341666-1667
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Mean Functional C1 Inhibitor Levels and C4 Levels
in Patients with Hereditary Angioedema after
Infusions of C1 Inhibitor Concentrate or Placebo
Waytes, A. T. et. al. N Engl J Med
19963341630-1634
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Inherited Complement Component Deficiencies

• C5
• C6
• C7
• C8

• Neisseria infections
• N. meningitidis meningitis
• N. gonorrhea gonorrhea

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Inherited Complement Component Deficiencies

• CD59 and DAF Deficiency
• Paroxysmal Nocturnal Hemoglobinuria
• -complement-mediated destruction
• of red blood cells

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Analysis of Lactate Dehydrogenase Levels, a
Biochemical Indicator of Hemolysis, in 11
Patients with Paroxysmal Nocturnal Hemoglobinuria
up to 25 Weeks before and during 12 Weeks of
Eculizumab Treatment
Hillmen, P. et. al. N Engl J Med 2004350552-559
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Incidence of Paroxysms during Eculizumab Treatment
Hillmen, P. et. al. N Engl J Med 2004350552-559
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Facial Rash in a Patient with a Hereditary C3
Deficiency
Walport, M. J. N Engl J Med 20013441140-1144
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Complement levels and lupus activity

• Disease quiet ?
• Fever ?
• Arthritis, skin rash, and protein in urine
• PREDNISONE Rx
• STARTED
• Fever, rash gone, urine
• normal ?

• C3, C4 levels normal
• Slight ? C3, C4
• Marked ? C3, C4
• C3, C4 levels normal

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