Infectious diseases associated with complement

CLINICAL MICROBIOLOGY REVIEWS, JUlY 1991, p. 359-395 0893-8512/91/030359-37$02.00/0 Copyright © 1991, American Society for Microbiology

Vol. 4, No. 3

Infectious Diseases Associated with Complement Deficiencies
J. E. FIGUEROA AND P. DENSEN* Department of Internal Medicine, VA Medical Center and University of Iowa College of Medicine,* Iowa City, Iowa 52242

INTRODUCTION............................................................... THE COMPLEMENT SYSTEM ............................................................... Biochemistry of Complement Proteins ...............................................................

359 360 360 360 Biosynthesis ............................................................... Activation of the classical pathway............................................................... 360 Activation of the alternative pathway ............................................................... 362 363 Assembly of the membrane attack complex ............................................................... 364 Complement receptors ............................................................... 364 Complement Regulation............................................................... Families of Complement Proteins ............................................................... 366 366 Complement-Mediated Functions ............................................................... 367 Microbial Interactions with Complement ............................................................... COMPLEMENT DEFICIENCY STATES............................................................... 368 Plasma Complement Proteins ............................................................... 368 368 Acquired deficiencies ............................................................... 368 Hereditary deficiencies ............................................................... 368 (i) Frequency ............................................................... (ii)Functional consequences of specific deficiencies ..............................................................369 (iii) Diseases associated with complement deficiencies ...........................................................376 (a) Deficiencies of classical-pathway proteins ............................................................... 376 (b) Deficiencies of alternative-pathway proteins............................................................... 377 378 (c) C3 deficiency ............................................................... (d) Deficiencies of late-complement components ............................................................... 378 Deficiencies of Membrane Complement Proteins ............................................................... 379 Leukocyte adhesion deficiency(CDll-CD18 deficiency or CR3 deficiency) ...................................379 PNH ............................................................... 379 INFECTION IN COMPLEMENT DEFICIENCY STATES ............................................................380 380 Epidemiology ............................................................... Meningococcal Disease in Complement Deficiency............................................................... 380 380 Pathogenesis of meningococcal disease ............................................................ Meningococcal disease in late-complement-component deficiency states .......................................381 382 Meningococcal disease in properdin deficiency ............................................................ IMMUNE RESPONSE TOMENINGOCOCCAL DISEASE ...........................................................382 Normal Individuals ............................................................. 382 383 Complement-Deficient Individuals ............................................................ Prevention of Infection in Complement-Deficient Individuals .......................................................384 Vaccination...