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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2804_Библиотеки_им_академика_М_И_Перельмана
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orblood-dwellinghelminthswillnotbefoundinthestool,serologictestsforantiparasiteantibodies
shouldalsobesent.Suchtestsareavailableforstrongyloidiasis,toxocariasis,andtrichinellosis.
DiagnosisatthetimeofpresentationwithLöfflersyndromecanbemadebydetectionofAscarislarvae
inrespiratorysecretionsorgastricaspiratesbutnotstool.
Peripheral blood smearandflowcytometryoflymphocytesubpopulationscanaidinthe diagnosisof
hematologic malignancy. Bone marrow biopsy for pathologic, cytogenetic, andmolecular testing on
bonemarrowand/orperipheralblood (e.g.,forFIP1L1/PDGFRA mutation)mayberequired. Serum
vitamin B12 level may be elevated in myeloproliferative neoplasms and autoimmune
lymphoproliferativesyndrome.
Evaluation for idiopathic HES should also consist of troponin measurement, echocardiogram, and
ECG.
Immunoglobulin levels are helpful if concerned for an immunodeficiency. Elevated immunoglobulin
levelscanbefoundinL-HES.
Atryptaselevelisnecessaryifmastocytosisisconsideredasacauseofeosinophilia.
IMAGING
CXRorCTfindingsmayalsohelptonarrowthedifferentialdiagnosis.
Peripheralinfiltrateswithcentralclearingareindicativeofchroniceosinophilicpneumonia.
Diffuse infiltrates in an interstitial, alveolar, or mixed pattern may be seen in acute eosinophilic
pneumoniaaswellasdrug-inducedeosinophiliawithpulmonaryinvolvement.
Transient infiltrates may be seen in Löffler syndrome, EGPA, or allergic bronchopulmonary
aspergillosis(ABPA).
CentralbronchiectasisisamajorcriterioninthediagnosisofABPA.
A diffuse miliary or nodular pattern, consolidation,or cavitation maybe found in cases of tropical
pulmonaryeosinophilia.
ACTofthesinuses,nerveconductionstudies,andtestingforp-ANCAmaybehelpfulinthediagnosis
ofeosinophilicgranulomatosiswithpolyangiitis(EGPA).
DIAGNOSTICPROCEDURES
If no other cause ofpulmonaryinfiltrates hasbeenidentified,a bronchoscopy may be necessaryfor
analysis ofbronchoalveolar lavage(BAL) fluidandlungtissue.Thepresenceofeosinophils inBAL
fluid or sputum witheosinophilic infiltration ofthe parenchyma is most typical of acute or chronic
eosinophilicpneumonia.
SkinbiopsywillaidindiagnosingthecutaneouseosinophilicdiseasesandEGPA.
TREATMENT
Mildeosinophiliawithnoevidenceofend-organdamagemaynotneedtreatment.
Oral steroids are indicatedwhen thereis evidenceoforganinvolvement.However,strongyloidiasis
mustbeexcludedbeforeadministrationofsteroidstopreventhyperinfectionsyndrome.
Whenadrugreactionissuspected,discontinuationofthedrugisbothdiagnosticandtherapeutic.Other
treatment options depend on the exact cause of eosinophilia because, with the exception of HES,
eosinophiliaisamanifestationofanunderlyingdisease.
HES: Patients with marked eosinophilia with no organ involvement may have a benign course. In
contrast, those with organ involvement and FIP1L1/PDGFRA-associated disease may have an
extremelyaggressivecoursewithouttreatment.
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Monitoringandearlyinitiationofhigh-doseglucocorticoidsshouldbepursuedinallpatientsexcept
thosewhohavetheFIP1L1/PDGFRAfusiongene.
Patients with the FIP1L1/PDGFRA fusion mutation should be started on imatinib mesylate
(Gleevec), a tyrosinekinase inhibitor.Treatmentshould be initiated promptlyin these patients to
prevent progression of cardiac disease and other end-organ damage. Imatinib has been shown to
inducediseaseremissionandhaltprogression.
11
Hydroxyurea has beenthe most frequentlyused effective second-lineagent and/or steroid-sparing
agent for HES.Interferon-α2bincombination with glucocorticoids has been usedtotreatL-HES.
HematopoieticcelltransplantationmaybeconsideredinrefractoryHES.
Mepolizumab, a humanized anti–IL-5 antibody, has shown corticosteroid-sparing effects in
FIP1L1/PDGFRA-negative,corticosteroid-responsivesubjectswithHES.
12
Alemtuzumab,ananti-CD52antibody(CD52isexpressed onthesurfaceofeosinophils),hasbeen
showntobeeffectiveintreatmentforpatientswithrefractoryidiopathicHES.
13
Primary eosinophilia disorders should be followed by a specialist; any cases of unresolved or
unexplainedeosinophiliawarrantevaluationbyanallergist/immunologist.
UrticariaandAngioedema
GENERALPRINCIPLES
Definition
Urticaria(hives)areraised,erythematous,well-demarcatedpruriticskinlesions.Centralclearingcan
cause an annular lesion andis often seenafter antihistamine use. Anindividual lesionusuallylasts
minutestohours.
Angioedema is swelling of the deep dermis and subcutaneous tissue. It is oftenpainful rather than
pruritic andgenerallylasts less than 48hours. It canbefoundanywhere on the bodybutmostoften
involvesthetongue,lips,eyelids,throat,bowels,and/orgenitals.
Classification
Acute urticaria (with or without angioedema) is defined as the occurrence of hives and/or
angioedema lasting<6 weeks.Itcanbe causedby anallergic reaction to a medication,food,insect
sting, or exposure(contactor inhalation) to anallergen. Patientscandevelop ahypersensitivity toa
food,medication,orself-careproductthatpreviouslyhadbeenusedwithoutdifficulty.Inmanycases
ofacuteurticaria,noidentifiabletriggercanbefound.
Chronic urticaria (with or without angioedema) is defined as the occurrence of hives and/or
angioedema for >6 weeks. There are many possible causes of chronic urticaria and angioedema,
including medications,autoimmunity,self-careproducts,andphysicaltriggers.However,the etiology
remainsunidentifiedin>80%ofcases.
Epidemiology
Urticariaisacommonconditionthataffects15%–24%oftheUSpopulationatsometimeintheirlife.
Chronic idiopathic urticaria occurs in approximately 1% of the US population, and there does not
appeartobeanincreasedriskinpersonswithatopy.
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Angioedemaoccursin40%–50%ofpatientswithurticaria.
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Etiology
IgE-mediated:drugs,foods,stingingandbitinginsects,latex,inhalant,orcontactallergens
Non–IgE-mediated:narcotics,musclerelaxants,radiocontrast,vancomycin,NSAIDs,ACEinhibitors
Transfusionreactions
Infections(i.e.,viral,bacterial,parasitic)
Systemic disorders: autoimmune diseases, malignancy, mastocytosis, HES, cryoglobulinemia, and
hereditarydiseases
Physicalurticaria:dermographism,cold,cholinergic,pressure,vibratory,solar,andaquagenic
Idiopathic
Pathophysiology
Mostformsofurticariaandangioedemaarecausedbythedegranulationofmastcellsorbasophilsand
thereleaseofinflammatorymediators.Histamineistheprimarymediatorandelicitsedema(wheal)and
erythema(flare).HAEandrelatedsyndromesaremediatedbytheoverproductionofbradykininandare
notresponsivetoantihistamines.
DIAGNOSIS
Diagnosisisbasedoncompletehistoryandphysicalexaminationwithcharacteristicskinlesions.
ClinicalPresentation
Patientswithanacuteurticariaepisodepresentwithhistoryofpruritic,raised,erythematouslesions.
Individuallesionsresolveoveraperiodof1–24hours.
Angioedema usuallypresentswith painful swelling without pruritus. Theswellingcantakeupto72
hourstoresolve.
Physicalurticariaisinducedbyenvironmentalorphysicalstimuli.Thecommontriggersarecold,heat,
sweating, exercise, pressure, vibration, andsunlight. Dermographism, literally“skin writing,”is the
most common form of physical urticaria. It affects approximately 4% of the population and can be
elicitedbybrisklystrokingorscratchingskin.
HISTORY
A detailed history should elicit identifiable triggers and rule out any systemic causes. When the
individual skin lesion lasts longer than 48 hours, the diagnosis of urticarial vasculitis must be
investigatedbyaskinbiopsy.
Anychangesinenvironmentalexposures, foods, medications, personalcare products, etc. shouldbe
determined.
Itisimportanttodifferentiatefromanaphylaxis,whichaffectsorgansotherthantheskin,asthiswillbe
treateddifferently(see“Anaphylaxis”section).
PHYSICALEXAMINATION
Completeexaminationoftheaffectedandnonaffectedskin.
Urticariaappearsaserythematous,raisedlesionsthatblanchwithpressure.
Angioedemaappearsasswelling;canofteninvolvetheface,tongue,extremities,orgenitalia;andmay
beasymmetric.
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DifferentialDiagnosis
IgE-mediatedallergicreactiontodrugs,foods,insects,inhalant,orcontactallergen.
Non–IgE-mediated drug and food reactions (i.e., medications including NSAIDs, vancomycin,
radioactiveiodine,opiates,musclerelaxants,foodsincludingtomatoesandstrawberries).
Pruriticurticarialpapulesandplaquesofpregnancy.
Mast cell release syndromes(i.e., systemic mastocytosis, cutaneous mastocytosis including urticaria
pigmentosa).
Cutaneoussmall-vesselvasculitis(i.e.,urticarialvasculitis,systemiclupuserythematosus).
HES.
Toxicdrugeruptions.
Allergiccontactdermatitis(i.e.,poisonivy,poisonoak).
Cryopyrin-associated periodic syndromes including familial cold autoinflammatory syndrome and
Muckle–Wellssyndrome.
Angioedemawithouturticariashouldleadtoconsiderationofspecificentities.
Use of ACE inhibitors or angiotensin II receptor blockers (ARBs) can be associated with
angioedemaatanypointinthecourseoftherapyandcanoccurupto6weeksafterlastexposure.
HAE,orC1INHdeficiency,isinheritedinanautosomaldominantpattern;25%ofcasesarisefrom
denovomutations.
Acquired C1 INH deficiency presents similarly to HAE but is typically associated with an
underlyinglymphoproliferativedisorder,connectivetissuedisease,orotherneoplasias.
DiagnosticTesting
Epicutaneousskintestingandpatchtestingareonlyindicatedwhensymptomsareassociatedwith
specifictriggers.
LABORATORIES
Routine laboratory testing in the absence of a clinical history is rarely helpful in determining an
etiologyinchronicurticaria.Evaluationforsystemicdiseaseassociatedwithchronicurticariaincludes
CBCwithdifferential,CRPorESR,thyroid-stimulatinghormone,renalandhepaticprofiles.
Autologousserumskintesting,assaysforbasophilhistaminerelease,andautoantibodiestoIgEandthe
high-affinityIgEreceptorareavailable,buttheutilityofthesetestshasnotbeenestablished.
All patients with angioedema without urticaria should be screened with a C4 level, which is
reducedduringandbetweenattacksofHAE.IftheC4levelisreduced,aquantitativeandfunctional
C1INHassayshouldbeperformed.MeasuringC1INHlevelsaloneisnotsufficientbecause15%of
patientshavenormallevelsofadysfunctionalC1INHprotein;therefore,itisimportanttoalsoobtain
thefunctionalassay.
AcquiredC1INHdeficiencypatientshavereducedC1q,C1INHlevelandfunction,andC4levelsdue
toanautoantibodytoC1INH.
DIAGNOSTICPROCEDURES
A skin biopsyshould be performed if individual lesions persist for>24 hours torule outurticarial
vasculitis.
TREATMENT
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Theideal treatmentofacuteurticaria withor withoutangioedemaisidentificationand avoidance of
specific causes. All potential causes should be eliminated. Most cases of acuteurticaria are self-
limitedandresolvespontaneously.
Carefulconsiderationshouldbegiventotheeliminationorsubstitutionofeachprescriptionorover-
the-countermedication or supplement.Ifapatientreactstoonemedicationina class,thereaction
likelywill be triggered byall medications inthat class. Exacerbatingagents (e.g.,NSAIDs, opiates,
vancomycin, and alcohol) should be avoided because they may induce nonspecific mast cell
degranulationandexacerbateurticariacausedbyotheragents.
Medications
Ifacuteurticariaisassociatedwithadditionalsystemicsymptomssuchashypotension,laryngeal
edema,orbronchospasm,treatmentwithepinephrine(0.3–0.5mLofa1:1000solutionIM)shouldbe
administeredimmediately.See“Anaphylaxis”sectionforadditionalinformation.
ACUTEURTICARIAAND/ORANGIOEDEMA
Second-generation antihistamines such as cetirizine, fexofenadine, or loratadine should be
administered to patients until the hives have cleared. Higher than conventional, US FDA–approved
dosesmayprovidemoreefficacy.Afirst-generation antihistaminesuchashydroxyzinemaybeadded
asaneveningdoseifneededtoobtaincontrolinrefractorycases.H2antihistamines,suchasranitidine,
mayalsobeaddedtotheabovetreatment.
Oral corticosteroids should be reserved for patients with moderate to severe symptoms.
Corticosteroidswillnothaveanimmediateeffectbutmaypreventrelapse.
If a patientpresentswith systemic symptoms, self-administeredepinephrineshouldbe prescribedfor
useinthecaseofanaphylaxis.
CHRONICURTICARIA
Useofasecond-generationH1antihistamineonceortwicedaily(uptofourtimesdaily).
If noresponse,thencanaddH2antihistamine,leukotrienereceptor antagonist,and/or first-generation
H1antihistamineordoxepintobetakenatbedtime.
If continuedurticaria, thencanchooseanalternative suchasomalizumab (FDA approved for patient
withchronicurticariathatfailH1antihistaminetherapy),cyclosporine,hydroxychloroquine,dapsone,
orotherimmunosuppressants.
Optimaldurationoftherapyhasnotbeenestablished;taperingmedicationsafter3–6monthsof
symptomcontrolhasbeensuggested.
HEREDITARYANDACQUIREDANGIOEDEMA(DISORDEROFC1INHIBITOR)
Acute attacks: C1 inhibitorconcentrate, icatibant,andecallantide are first-lineagents.Ifnoneofthe
first-lineagentsareavailable,freshfrozenplasmacanbeused.Alsopursuesymptomatictherapyand
rehydration.
Preventative medications include C1 inhibitor replacement via IV or SC, lanadelumab, berotralstat,
attenuatedandrogens,andantifibrinolytics.
Referral
Allpatientswithchronicurticariaorahistoryofanaphylaxisshouldbereferredtoanallergyspecialist
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forevaluationtoidentifypotentialallergicandautoimmunetriggers.
Immunodeficiency
GENERALPRINCIPLES
Definition
Primary immunodeficiencies (PIDs) are disorders of the immune system that result in an increased
susceptibilitytoinfection.
Secondary immunodeficiencies are also disorders of increased susceptibility to infection but are
attributabletoanexternalsource.
Classification
PIDscanbeorganizedbythedefectiveimmunecomponentswithconsiderableheterogeneityineach
disorder.
Predominantlyantibodydeficiencies:Thedefectisprimarilyintheabilitytomakeantibodies.
Commonvariableimmunedeficiency(CVID)
X-linked(Bruton)agammaglobulinemia
IgGsubclassdeficiency
Specificantibodydeficiency
Hyper-IgMsyndrome
SelectiveIgAdeficiency
Combinedimmunodeficienciesandsyndromes:Thedefectresultsindeficienciesinbothcellularand
humoralimmuneresponses.
Severecombinedimmunodeficiencies
DiGeorgesyndrome
Hyper-IgE(Job)syndrome
Defects of innate immunity: Defects in germline-encoded receptors and downstream signaling
pathways.
DeficiencyofToll-likereceptorsignaling
Mendeliansusceptibilitytomycobacterialdiseases(MSMD)
Naturalkiller(NK)celldeficiency
Phagocyticcelldeficiencies
Chronicgranulomatousdisease(CGD)
Complementdeficiencies
Diseases of immune dysregulation: Autoimmunity and lymphoproliferation are characteristic
manifestationsinthesedisorders.
Epidemiology
Secondary immunodeficiency syndromes, particularly HIV/AIDS, are the most common
immunodeficiencydisorders.
TheestimatedprevalenceofPIDsisapproximately1in1200livebirths.
MostPIDspresentinginadulthoodarehumoralimmunedefectsaffectingantibodyproduction.
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Etiology
PredominantlyantibodyimmunedeficienciesarethoughttobecausedbydefectsinB-cellmaturation.
Combined immunodeficiencies are caused by defective T-cell–mediated immunity and associated
antibodydeficiency.
AvarietyofgeneticmutationshavebeenassociatedwithspecificPIDsyndromes.
Secondary immunodeficiencies can be caused by medications (chemotherapy, immunomodulatory
agents, corticosteroids), infectious agents (e.g., HIV), malignancy, antibody loss (e.g., nephrotic
syndrome, protein losing enteropathy, or consumption during a severe underlying infection),
autoimmune disease (e.g., systemic lupus erythematosus, rheumatoid arthritis), malnutrition (vitamin
D),andotherunderlyingdiseases(e.g.,diabetesmellitus,cirrhosis,uremia).
DIAGNOSIS
ClinicalPresentation
The hallmark of PID is recurrent infections. Clinical suspicion should be increased by recurrent
sinopulmonaryinfection,deep-seatedinfections,opportunisticinfections,ordisseminatedinfectionsin
anotherwisehealthypatient.
SpecificPIDsareoftenassociatedwithparticulartypesofpathogens(e.g.,catalase-positiveinfections
inCGDorMSMD).
RecurrenturinarytractinfectionsareonlyrarelyassociatedwithPID.
PatientswithPIDsmayalso presentwithautoimmunity, immunedysregulation,allergicdiseases,and
malignancies.
SelectiveIgAdeficiencyisthemostcommonimmunedeficiency,withaprevalenceof1in300–500
people.
Mostpatientsare asymptomatic.Some maypresentwith recurrentsinusandpulmonaryinfections.
TherapyisdirectedatearlytreatmentwithantibioticsbecauseIgAreplacementisnotavailable.
Associated autoimmune diseases are observed in 20%–30% of cases. Absolute IgA-deficient
patients(i.e.,<7mg/dL)areatriskfordevelopingaseveretransfusionreactiontobloodproducts
including IV immunoglobulin (IVIG), because of thepresence of IgE anti-IgA antibodies in some
individuals;therefore, thesepatientsshould betransfused withwashed redblood cellsor receive
bloodproductsonlyfromIgA-deficientdonors.
CVID is the most common symptomatic PID, occurring with a frequency of 1/25,000. It includes a
heterogeneousgroupofdisordersinwhichmostpatientspresentinthesecondtofourthdecadeoflife
withrecurrentsinusandpulmonaryinfectionsandarediscoveredtohavelowanddysfunctionalIgG,
IgA,and/orIgMantibodieswithpoorresponsetoimmunizations.
B-cellnumbersareoftennormal,butthereisdecreasedabilitytoproduceimmunoglobulinbecause
ofthelackofisotype-switchedmemoryBcells.SomepatientsmayalsoexhibitT-celldysfunction.
CVID is largely idiopathic, although there are molecular defects in the B-cell signaling and
developmentpathways(e.g.,TACI,ICOS,BAFF-R,andCD19)withsomeformsofthedisorder.
PatientswithCVIDareparticularlysusceptibletoinfectionwithencapsulatedorganisms.
Patientsmayhaveassociatedgastrointestinaldiseaseorautoimmuneabnormalities(mostcommonly
autoimmune hemolytic anemia, idiopathic thrombocytopenic purpura, pernicious anemia, and
rheumatoidarthritis).
Thereisanincreasedincidenceofmalignancy,especiallylymphoidandgastrointestinalmalignancy.
TherapyconsistsofIVIGor subcutaneousimmunoglobulin(SCIG)replacement therapyaswell as
prompttreatmentofinfectionswithantibiotics.
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Specific antibody deficiency is defined as poor or absent antibody responses to polysaccharide
antigens(i.e.,23-valentpneumococcalvaccine)inthesettingofnormallevelsofimmunoglobulinsand
IgGsubclasses.
B-cell numbers and response to protein antigens (i.e., tetanus toxoid and diphtheria toxoid) are
usuallynormal.
Patients have increased susceptibility to sinopulmonary infections. Allergic diseases are also
common.
Therapeutic approaches include adequate antibiotic treatment for infections, pneumococcal
conjugatevaccine,andimmunoglobulinreplacement.
X-linked (Bruton) agammaglobulinemia clinically manifests very similarly to severe CVID and is
typicallydiagnosedinchildhood,butcanpresentinadulthood.
PatientsusuallyhavelowlevelsofallimmunoglobulintypesandverylowlevelsofBcells.
SpecificgeneticdefectisinBrutontyrosinekinase,whichisinvolvedinB-cellmaturation.
Subclassdeficiency. Deficiencies ofeachof the IgGsubclasses (IgG1, IgG2,IgG3, andIgG4) have
beendescribed.
ThesepatientspresentwithsimilarcomplaintsastheCVIDpatients.
Total IgG levels may be normal. A strong association with IgA deficiency exists. There is
disagreementas towhetherthis isaseparateentityfrom CVID.Inmostcases,thereisnoneedto
evaluateIgGsubclasslevels.
Isolatedsubclassdeficiencywithoutrecurrentinfectionsisofunknownclinicalsignificance.
Hyper-IgMsyndromeischaracterizedbylowIgAandIgGlevelswithnormalorincreasedIgMand
poor antibody function. There are several gene mutations reported, which cause defective class
switchinginimmunoglobulins.Dependingonthemutation,somepatientsmayhavepoorT-cellfunction
aswell,leadingtoincreasedopportunisticinfections.
Hyper-IgEsyndrome (Job syndrome) is characterizedbyrecurrent pyogenic infections ofthe skin
andlowerrespiratorytract.Thissyndromecanresultinsevereabscessandempyemaformation.Some
formsofthediseaseareassociatedwithautosomaldominantmutationofSTAT3.
ThemostcommonorganisminvolvedisS.aureus,butotherbacteriaandfungihavebeenreported.
Patientspresentwithrecurrentinfectionsandhaveassociatedpruriticdermatitis,coarse(lion-like)
facies,growthretardation,scoliosis,retentionofprimaryteeth,andhyperkeratoticnails.
Laboratorydatareveal thepresence ofnormal levels ofIgG, IgA,andIgM butmarkedlyelevated
levelsofIgE.Amarkedincreaseintissueandbloodeosinophilsmayalsobeobserved.
ComplementdeficienciesareabroadcategoryofPIDcharacterizedbyrecurrentinfectionstoarange
ofpathogens.
Recurrent disseminated Neisseria infections are associated with a deficiency in the terminal
complementsystem(C5–C9).
Systemic lupus–like disorders and recurrent infection with encapsulated organisms have been
associatedwithdeficienciesinothercomponentsofcomplement.
CH50 and AH50 are useful to screen for deficiencies of the classical pathway and alternative
pathway,respectively.
CGDischaracterizedbydefectivekillingofintracellularpathogensbyneutrophils.
Patients usually present with frequent infection, often with abscesses, from S. aureus and other
catalase-positive organisms. Aspergillus is a particularly troublesome pathogen for patients with
CGD.
Diagnosisismadebydemonstrationofdefectiverespiratoryburstwithflowcytometryassayusing
dihydrorhodamine.
MSMDis causedbydefects in Th1immunity andis associated with mutationsingenes involved in
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interferon-γandIL-12 signaling. Characteristicinfectionsincludemycobacterial infections(including
typicalandatypicalMycobacterium)andSalmonellainfections.
DiagnosticTesting
Frequentsinopulmonaryinfections,recurrentandinvasiveinfectionsrequiringIVantimicrobialagents,
infectionswithunusualpathogens,andfamilyhistoryofPIDarewarningsignsforPIDs.
Initialevaluationshouldfocusonidentifyingpossiblesecondarycausesofrecurrentinfectionsuchas
allergy, medications,andanatomic abnormalities. Workup begins with a CBC with differential, HIV
test, quantitative immunoglobulin levels, and complement levels. Often the evaluation will need to
include enumeration of lymphocytes by flow cytometryif B-, T-, or NK-cell defects are suspected.
Otherspecializedtestsincludinggenetictestingmaybeneededtomakeadefinitivediagnosis.
If clinical suspicion is high for an underlying antibody-predominant PID, B-cell function can be
assessed by measuring immunoglobulin response to vaccinations. Preimmunization and
postimmunization titers for both a protein antigen (i.e., tetanus) and a polysaccharide antigen (i.e.,
Pneumovax, theunconjugated 23-valent vaccine) are measuredbecause proteinsandpolysaccharide
antigensarehandleddifferentlybytheimmunesystem.
Titersofspecificantibodiesaremeasuredbeforeandat4–8weeksafterimmunization.
Genetictestingcanalsobeperformed.
TREATMENT
KilledorsubcomponentvaccinesaresafeformostpatientswithPID,althoughsomepatientsmaynot
producefullresponse.LiveattenuatedvaccinesmaybecontraindicatedinsomeindividualswithPID
andtheirfamilies.
ProphylacticantibioticsmaybeconsideredinsomePIDsyndromestopreventinfections.
IgAdeficiency:Nospecifictreatmentisavailable.However,thesepatientsshouldbepromptlytreated
atthefirstsignofinfectionwithanantibioticthatcoversStreptococcuspneumoniaeorHaemophilus
influenzae.
CVID should be treated with immunoglobulin replacement in forms of IVIG or SCIG. Numerous
preparationsofimmunoglobulinareavailable,allofwhichundergoviralinactivationsteps.Possible
side effects include myalgias, vomiting, chills, and lingering headache (due to immune complex–
mediatedasepticmeningitis).
Referral
PatientsinwhomaPIDisbeingseriouslyconsideredshouldundergoevaluationbyanallergist/clinical
immunologistwithexpertiseindiagnosingandtreatingPID.
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