Facts on File Encyclopedia of Health and Medicine

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common variable immunodeficiency (CVID)
An immune disorder in which the IMMUNE SYSTEM
lacks the ability to produce adequate antibodies to
protect the body from INFECTION. Though there are
normal numbers of B-lymphocytes in the BLOOD
circulation, these ANTIBODY-producing cells are
lacking IMMUNOGLOBULING (IgG), a protein essen-
tial for ANTIGENrecognition and antibody produc-
tion. IgG is the foundation for most antibodies
that the immune system produces.


Symptoms and Diagnostic Path

Symptoms of CVID can show up any time after
about age 10 though most commonly appear in the
late 20s and early 30s. Generally the person has the
same type of infection repeatedly, as the immune
system is not producing antibodies to protect
against the infectious agent. The key symptom is a
progressive pattern of recurrent or chronic infec-
tions. Infections are most commonly upper respira-
tory, SINUSES, THROAT, and middle EAR—typically
BRONCHITIS, PNEUMONIA, PHARYNGITIS, SINUSITIS, and
OTITISmedia. Infections may be viral, bacterial, fun-
gal, or parasitic. Some people also have gastroin-
testinal infections. The severity of infection varies
among people who have CVID as well as within an
individual from infection to infection. The diagnos-
tic path includes blood tests to measure IgG and
antibody levels. Immunoglobulin A (IgA) and
immunoglobulin M (IgM) levels may also be low.


Treatment Options and Outlook

The main therapeutic course is reducing exposure
to known infection (such as common viral infec-
tions) and treatment with ANTIBIOTIC MEDICATIONSat
the first sign of infection. GAMMAGLOBULINinjec-
tions can bolster the immune system, though the
gammaglobulin (which comes from blood or
PLASMAdonors) may not contain the specific anti-
bodies the person needs. With effective medical
management and diligent prevention efforts, most
people who have CVID can enjoy relatively nor-
mal lifestyles and life expectancy.


Risk Factors and Preventive Measures
Most CVID is acquired, though researchers do not
know what causes it to occur. There are no known
measures for preventing CVID. CVID occurs more


frequently in people who have AUTOIMMUNE DISOR-
DERSsuch as RHEUMATOID ARTHRITISor SYSTEMIC LUPUS
ERYTHEMATOSUS(SLE). Prompt diagnosis and appro-
priate treatment are essential for preventing
substantial lung damage that can occur from
recurrent infections.
See also BACTERIA; FUNGUS; HAND WASHING; LUNGS;
PARTIAL COMBINED IMMUNODEFICIENCY(PCID); PARASITE;
PERSONAL HYGIENE; SEVERE COMBINED IMMUNODEFI-
CIENCY(SCID); LIVING WITH IMMUNE DISORDERS; VIRUS.

complement cascade The series of events that
take place when an ANTIBODYbinds with an ANTI-
GEN, activating the complements. Complements
are proteins that participate in immune and
inflammatory processes, acting primarily to kill
antibody-marked cells. The biochemical interac-
tions that take place with their activation ulti-
mately lead to the formation of a protein structure
called the terminal complement component or the
membrane attack complex. The membrane attack
complex penetrates the cell membrane of the anti-
body-marked cell. This penetration kills the cell
and coats it in proteins that mark it for PHAGOCYTO-
SIS, the process through which LEUKOCYTE(white
BLOODcell) scavenger cells, called phagocytes, con-
sume the debris that remains after the attacked
cell dies.
There are about 30 complement proteins, also
called complement factors, in the blood circula-
tion. They remain inactive until antibody–antigen
bindings or certain other immune responses acti-
vate them. Doctors classify activated complements
into nine major molecular complexes identified as
C1–C9. C1–C4 form the preliminary pathways
leading to the formation of the membrane attack
complex. C5–C9 collectively form the membrane
attack complex. Other proteins interact with the
complements to keep their actions in check. Disin-
tegration of the complement complexes begins
immediately after their activation to prevent them
from damaging other cells.
Blood tests can measure complement activity in
the body. Complement activity is often increased
in the presence of cancer and decreased with cer-
tain AUTOIMMUNE DISORDERSsuch as SYSTEMIC LUPUS
ERYTHEMATOSUS (SLE). Complement activity also
diminishes in GRAFT VS. HOST DISEASE.

complement cascade 255
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