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ANGIOEDEMA, LARYNGEAL EDEMAANGIOEDEMA, LARYNGEAL EDEMADIAGNOSIS AND MANAGEMENTDIAGNOSIS AND MANAGEMENT
Henriette Farkas Semmelweis University
3 rd Department of Internal MedicineBudapest, Hungary
farkash@kut.sote.hu
UPPER AIRWAY OBSTRUCTION (UAO)AETIOLOGY
MECHANICAL CAUSESForeign body aspirationInfections Laryngeal edema Haemorrhage and haematoma Trauma Burns Neoplasm CongenitalMiscellaneous
FUNCTIONAL CAUSESCNS depression Peripheral nervous system and neuromuscular abnormalities
LARYNGEAL EDEMA
is a life-threatening condition characterized by acute or gradual onset with swelling of the laryngeal mucosa
PATIENT’S COMPLAINTS, SIGNS
• Dysphagia• Sensation of a lump in the throat• Feeling of tightness• Voice changes• Hoarseness• Roughness • Resonant barky cough• Stridor• Dyspnea• Fear of asphyxation• Aphonia • Patient is unable to breathe, speak, or cough and may hold the throat• between the thumb and index finger (the universal choking sign)• Patient is anxious and agitated
PHYSICIAN EXAMINATION
2. Physical examination• Voice changes• Hoarseness• Roughness • Resonant barky cough• Stridor• Dyspnea• Aphonia • Patient is anxious and agitated • Vigorous attempts at respiration with intercostal and supraclavicular
retraction• Patient becomes rapidly cyanosed • Respiratory efforts diminish• Loss of consciousness • Heart rate and blood pressure raised, bradycardia• Hypotension, cardiac arrest • Death is inevitable if the obstruction is not relieved within 2-5 minutes
of the onset
1. Medical history (patient or relatives)
EXAMINATION IN THE HOSPITAL
Indirect laryngoscopy
flexible fibreoptic laryngoscopy
direct laryngoscopy
Radiographic imagingAP and lateral plain neck radiographs, CT, MRI
Congenital stenosis
Infection Laryngeal oedema Tumor
Foreign bodyRecurrent paralysis
Normal larynx
THE EFFECT OF EDEMA ON THE CROSS SECTIONAL AREA OF THE SUBGLOTTIC LARYNX
Physical status
Neonate Child Adult
Normal
Subglottic area (mm2)
12 48 147
Effect of 1 mm edema
3 27 108
Reduction of airway area %
75 44 27
MANAGEMENT
Treatment consists of
• immediately ensuring an adequate airway• administration of oxygen • intravenous fluids• epinephrine, antihistamines and steroids• C1-INH concentrate, bradykinin receptor B2
antagonist, kallikrein inhibitor or FFP
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES1. Try simple manoeuvres to open airway
• Jaw thrust is used when other methods have failed. • Oro- or nasopharyngeal airway is useful in the
unconscious patient. • If the patient is not immediately intubated the coma
position should be used.
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES2. Endotracheal intubation
Method of choice for the unconscious apnoeic patient
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES3. Surgical airway
• cricothyroidotomy • percutanous tracheotomy • emergency tracheostomy
Cricothyroidotomy is an emergency procedure when intubation or tracheotomy are impossible. Relatively easy way of providing an emergency airway. (cricothyroid membrane is near the skin surface)
CRICOTHYROIDOTOMY
PERCUTANOUS TRACHEOSTOMY
PCT is cost-effective, safe, fast, and easy to perform
Emergency tracheotomy rarely required.
Formal surgical tracheotomy under local anaesthesiamay be a prudent approach under some controlled conditions.
TRACHEOTOMY
CLASSIFICATION OF ANGIOEDEMA
• ALLERGIC • NSAID-induced• Idiopathic• Associated with idiopathic or autoimmune urticaria• Associated with urticaria vasculitis• Infections and infestations• Angioedema with eosinophilia• Associated with some physical urticarias and with cholinerg
urticaria• Associated with contact urticaria• Angiotensin- converting enzyme inhibitor-induced• C1-INH deficiency• Hereditary angioedema with normal C1-INH
Kaplan, Graeves 2005
PATHOMECHANISM
IGE MEDIATED, I TYPE HYPERSENSITIVITY
Roitt
COMMON ALLERGENS
• Foods such as peanut, milk, nuts, shellfish, fish, eggs, and other foods.
• Medications including penicillin and related or unrelated antibiotics, may produce allergic reactions.
• Insect sting venom • Less common causes are latex rubber in surgical gloves and enema
devices
SYMPTOMS
• in acute allergic laryngeal edema, angioedema of the lips and supraglottis, glottis, and infraglottis results in airway obstruction
• systemic reaction consists of a variable combination of urticaria (79%), bronchospasm (70%), shock, cardiovascular collapse and abdominal pain
May be localized or part of a systemic anaphylactic reaction
Prick test
DIAGNOSIS
Specific IgE Use specific monoclonal antibodies against allergens
MANAGEMENT
ACUTE TREATMENT CONSISTS OF
• immediately ensuring an adequate airway• and administration of oxygen, • intravenous fluids, • epinephrine:im, iv• antihistamines and steroids
NEXT STEP
hyposensibilisation (bee, wasp venom)
INFORMATION, EMERGENCY CARE KIT
• Wear a medic alert bracelet at all times
• Get information from patient’s doctor and the pharmacist before taking any medication
• Read all food labels carefully
• Carry with the patient an emergency care kit so that it can be self-administered epinephrine
CLASSIFICATION OF ANGIOEDEMA• Allergic • NSAID-induced• Idiopathic• Associated with idiopathic or autoimmune urticaria• Associated with urticaria vasculitis• Infections and infestations• Angioedema with eosinophilia• Associated with some physical urticarias and with cholinerg
urticaria• Associated with contact urticaria• ANGIOTENSIN- CONVERTING ENZYME INHIBITOR-
INDUCED• Acquired C1-INH deficiency• Hereditary angioedema
Kaplan, Graeves 2005
VasodilatationIncreased vascular permeabity
HW kininogen
bradykinin
BK metabolits
Angiotensinogen
Angiotensin I
Angiotensin II
VasoconstrictionVascular hypertrophyAldosterone release
ACE
reninkallikrein
KININ SYSTEM
VasodilatationIncreased vascular permeabity
kininogen
bradykinin
BK metabolits
angiotensinogen
Angiotensin I
Angiotensin II
VasoconstrictionVascular hypertrophyAldosterone release
ACE
reninkallikrein
EFFECT OF ACE INHIBITORS
ACE inhibitor
ATII Blocker
ACE INHIBITORS
35 - 40 million patients worldwide are treated with ACEinhibitors, ACE inhibitors are generally well tolerated.
ADVERSE EFFECTS:Significant adverse effects include hypotension, renalimpairment, cough, and angioedemaPrevalence of angioedema: 0.1-0.7%
Angiooedema can first manifest itself from a few hours to 10years after an ACE inhibitor has been first taken.Thereforephysicians fail to recognize the association.
MANAGEMENT OF ACE INHIBITOR-INDUCED ANGIOEDEMA
Avoid ACE inhibitors
Conservative treatment antihistamines with orwithout steroids
Tongue and upper airway are involved,intramuscular adrenaline should be used, some patients require an artificial airway
C1 inhibitor concentrate,FFP, SDP, kallikrein inhibitor or bradykinin receptor antagonisthas been successfully
ACUT TREATMENT
MANAGEMENT OF ACE INHIBITOR-INDUCED ANGIOEDEMA
Should not be switched to another ACE inhibitor.
Calcium channel blockers and/or thiazides are appropriate as alternative antihypertensives. Beta blockers are contraindicated in the initial setting.
Alternative medications: are AT II receptor blockers Several cases of angioedema associated with ATII receptor although the overall incidence appears lower than that with ACE inhibitors.
LONG-TERM
CLASSIFICATION OF ANGIOEDEMA
• Allergic • NSAID-induced• Idiopathic• Associated with idiopathic or autoimmune urticaria• Associated with urticaria vasculitis• Infections and infestations• Angioedema with eosinophilia• Associated with some physical urticarias and with cholinerg
urticaria• Associated with contact urticaria• Angiotensin- converting enzyme inhibitor-induced• ACQUIRED C1-INH DEFICIENCY• HEREDITARY ANGIOEDEMA
Kaplan, Graeves 2005
Type I Type I (Donaldson, 1963)(Donaldson, 1963)
Type II Type II (Rosen, 1965)(Rosen, 1965)
Type IIIType III(Bork, (Bork, Binkley, 2000, Martin Binkley, 2000, Martin 20020011))
HEREDITARY ANGIOEDEMA (HAE)
C1-inhibitor (C1-INH) deficiency
Normal C1-INH
HEREDITARY ANGIOEDEMA (HAE) Type I. II
• autosomal dominant inheritance
• deficiency of C1 inhibitor
• onset of symptoms: in childhood
• prevalence: 1:10 000, 1:50 0000
• mortality: 20-30 %
HEREDITARY ANGIOEDEMA (HAE)Type III
• Mainly in vomen• Percipitating factors: oral contraceptive therapy,
hormone replacement treatment, pregnancy• Symptoms: similar to HAE Type I and II• Subgroup: missense mutation in factor XII gene• Normal C1-INH function• Not respond antihistamine treatment• Tranexamic acid, C1-INH concentrate?
Autosomal dominant
inheritance
Deficiency of C1-inhibitor
(C1-INH)
Two phenotype HAE I and II
Prevalence: 1/50 000
Mortality: 20-30%
Osler Am J Med Sci 1888; Donaldson Am J Med 1963; Rosen J Clin Invest 1971
HEREDITARY ANGIOEDEMA (HAE) TYPE I & IIHEREDITARY ANGIOEDEMA (HAE) TYPE I & II
PATHOMECHANISM
FXII FXIIa
Prekallikrein
Kallikrein
HWK
BradBradyykininkinin
Plasminogén
Plasmin
C1
C1rs
C42
C2 kininC2 kinin
C1C1--INHINHC1C1--INHINH
edema
Family historyFamily history
CClinicallinical symptoms symptoms
Measurement of Measurement of complement complement
• pedigree analysis
• genetic testing in HAE
DIAGNOSIS OF HAE
asymptomatic
Confirm the diagnosisin uncertain casesPrenatal diagnostics
Agostoni A Medicine 1992
Farkas H Acta Dermato-Venereol 2001
SUBCUTANEOUS SYMPTOMS
SUBCUTANEOUS SYMPTOMS
Bork K Am J Med 2006
SUBMUCOSAL SYMPTOMS
Upper airway mucosa
Pharyngeal edema Laryngeal edema
Bork K Transfus Apher Sci 2003; Tsunoda Laryngoscope 2000
SUBMUCOSAL SYMPTOMS Intestinal mucosa
intense, colicky abdominal pain nausea and vomiting postattack (watery) diarrhea can mimic an „acute abdomen”
Unnecessary surgicalintervention
Bork K Am J Gastroenterol 2006
ABDOMINAL ULTRASOUNDNonspecific but extremely sensitive method
ascites edematous intestinal wall
Farkas H Eur J Gastroenterol Hepatol 2001; Acta Paediatr 2002
In patients with known HAE Differential diagnosis
Recurrent abdominal complaints with ascites HAE should be considered if all other differential diagnostic options have
been ruled out.
COMPLEMENT PROFILES IN C1-INH DEFICIENCIES
TypeType C1qC1qC1-INHC1-INH
antigenantigenC1-INH C1-INH
functionfunction C4C4 Anti-Anti-C1-INHC1-INH
HAE-IHAE-I NN LL LL LL --
HAE-IIHAE-II NN N/HN/H LL LL --
AAE-IAAE-I LL LL LL LL --
AAE-II AAE-II
(autoimm.)(autoimm.)LL N/LN/L LL LL ++++++
Agostoni A J Allergy Clin Immunol. 2004
COUNSELING & EDUCATION
FOLLOW-UP
TREATMENT
MANAGEMENT
Bowen J Allergy Clin Immunol 2004
Individualized information to patient and parents
Written information to school, pediatrician, & family practitioner
Multilanguage infocard, hne service, hospital for emergencies
Drug for emergency
Patient diary
Patient organizations, websites
Farkas H Transfus Apher Sci 2003
COUNSELING & EDUCATION
TREATMENT
Elimination of precipitating factors
Prophylaxis with drugs
Management of attack
minor trauma
stress
drugs hormons
(estrogens)
infection
Farkas H Lancet 2001; Bouillet L Dermatology 2003
Long-term antifibrinolytic agents (tranexamic acid)
attenuated androgens
attenuated androgens5 days before the intervention and for2 days after
Short-term
Before surgery and
instrumentation on the
oropharynx, head and neck
C1-INH concentrate
C1-INH concentrate, SDP, FFP
• ≥1 attack per month or• history of life-threatening attacks
PROPHYLAXIS WITH DRUGS
Cicardi M J Allergy Clin Immunol 1991; Farkas H J Oral Maxillofac Surg 1999
SIDE EFFECTS
-seborrhoea, acne, hirsutism, weight gain, hair loss, deeping of the voice, menstrual irregularities,decrease breast size, myalgia, fatigue, headaches, SHBG ↓-inzulin resistance↑, plasma glucagon↑, LDL, cholesterol↑, a HDL-cholesterol↓, Apo-AI and Apo-AII↓, TBG↓-erythrocytosis, polycythaemia, thrombocytosis, eosinophylia, leukopenia, Prot. C, S ↑, antithrombin III ↑, haematuria-transaminase↑, hepatocellular adenoma and liver carcinoma
ATTENUATED ANDROGENS ANTIFIBRINOLYTIC AGENTS
thrombosispostural hypotensionmuscular pain and weeknesscreatine kinase ↑aldolase ↑rhabdomyolysis myositisfatigue
Tranexamic acidEpsilon-amino-caproic acid
Danazol, stanozolol, oxandrolone
Cicardi M J Allergy Clin Immunol. 1997; Széplaki G J Allergy Clin Immunol. 2005
SIDE EFFECTS MINIMAL EFFECTIVE DOSE
FOLLOW-UP
blood cell count liver & renal function, lipid profile, CK urinalysis abdominal ultrasound HIV, Hepatitis A,B,C, serology vaccination to Hepatitis B
ADJUSTMENT OF THERAPYALTERATION IN STATUS
Gompels M Clin Exp Immunol 2005; Farkas H Lancet 1999
MANAGEMENT OF ATTACK
pharyngeal/laryngealface, lips edemaabdominal attacksevere edema ofextremities, trunkneck & genitals
Edema of theextremities,Mild abdominal attack
tranexamic acid or danazol for 2-3 days
MILD
C1-INH concentrate, FFP,SDFSEVERE
Corticosteroids, antihistamines, and epinephrine are INEFFECTIVE
Farkas H JACI 2007; Prematta M Ann Allergy Asthma Immunol 2007; Bork K Transfusion 2005
XIIa Faktor
Trauma
Kallikrein
Prekallikrein
HMW-KininogenBradykinin
hC1-INH, rhC1-INH
ACE
Des-Arg9-Bradykinin
Bradykinin receptor Bradykinin receptor antagonist antagonist IcatibantIcatibant
Kallikrein inhibitor, DX88
edema
Bork K J Allergy Clin Immunol 2007; Van Doom M J Allergy Clin Immunol 2005; Levy J Expert Opin Investig Drugs 2006
FUTURE TREATMENT OPTIONS
WWW.HAENET.HU
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