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ANGIOEDEMA,%20LARYNGEAL%20EDEMA%20DIAGNOSIS%20AND%20MANAGEMENT

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Title: ANGIOEDEMA,%20LARYNGEAL%20EDEMA%20DIAGNOSIS%20AND%20MANAGEMENT


1
ANGIOEDEMA, LARYNGEAL EDEMA DIAGNOSIS AND
MANAGEMENT
  • Henriette Farkas
  • Semmelweis University
  • 3 rd Department of Internal Medicine
  • Budapest, Hungary

farkash_at_kut.sote.hu
2
UPPER AIRWAY OBSTRUCTION (UAO) AETIOLOGY
LARYNGEAL EDEMA is a life-threatening
condition characterized by acute or gradual onset
with swelling of the laryngeal mucosa
FUNCTIONAL CAUSES CNS depression Peripheral
nervous system and neuromuscular abnormalities
  • MECHANICAL CAUSES
  • Foreign body aspiration
  • Infections
  • Laryngeal edema
  • Haemorrhage and haematoma
  • Trauma
  • Burns
  • Neoplasm
  • Congenital
  • Miscellaneous

3
PATIENTS 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

4
PHYSICIAN EXAMINATION
1. Medical history (patient or relatives)
  • 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

5
EXAMINATION IN THE HOSPITAL
flexible fibreoptic laryngoscopy
Indirect laryngoscopy
direct laryngoscopy
Radiographic imaging AP and lateral plain neck
radiographs, CT, MRI
6
Congenital stenosis
Foreign body
Recurrent paralysis
Normal larynx
Infection
Laryngeal oedema
Tumor
7
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
8
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

9
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES 1. 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.

10
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES 2.
Endotracheal intubation
Method of choice for the unconscious apnoeic
patient
11
PRINCIPLES OF AIRWAY MANAGEMENT TECHNIQUES 3.
Surgical airway
  • cricothyroidotomy
  • percutanous tracheotomy
  • emergency tracheostomy

12
CRICOTHYROIDOTOMY
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)
13
PERCUTANOUS TRACHEOSTOMY
PCT is cost-effective, safe, fast, and easy to
perform
14
TRACHEOTOMY
Emergency tracheotomy rarely required. Formal
surgical tracheotomy under local anaesthesia may
be a prudent approach under some controlled
conditions.
15
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
16
PATHOMECHANISM
  • IGE MEDIATED, I TYPE HYPERSENSITIVITY

Roitt
17
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

18
SYMPTOMS
May be localized or part of a systemic
anaphylactic reaction
  • 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

19
Prick test
DIAGNOSIS
Specific IgE
Use specific monoclonal antibodies against
allergens
20
MANAGEMENT
  • ACUTE TREATMENT CONSISTS OF
  • immediately ensuring an adequate airway
  • and administration of oxygen,
  • intravenous fluids,
  • epinephrineim, iv
  • antihistamines and steroids

NEXT STEP hyposensibilisation (bee, wasp venom)
21
INFORMATION, EMERGENCY CARE KIT
  • Wear a medic alert bracelet at all times
  • Get information from patients 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

22
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
23
KININ SYSTEM
HW kininogen
Angiotensinogen
kallikrein
renin
Vasodilatation Increased vascular permeabity
Angiotensin I
bradykinin
ACE
BK metabolits
Angiotensin II
Vasoconstriction Vascular hypertrophy Aldosterone
release
24
EFFECT OF ACE INHIBITORS
kininogen
angiotensinogen
kallikrein
renin
Vasodilatation Increased vascular permeabity
bradykinin
Angiotensin I
ACE
ACE inhibitor
Angiotensin II
BK metabolits
ATII Blocker
Vasoconstriction Vascular hypertrophy Aldosterone
release
25
ACE INHIBITORS
  • 35 - 40 million patients worldwide are treated
    with ACE
  • inhibitors, ACE inhibitors are generally well
    tolerated.
  • ADVERSE EFFECTS
  • Significant adverse effects include hypotension,
    renal
  • impairment, cough, and angioedema
  • Prevalence of angioedema 0.1-0.7
  • Angiooedema can first manifest itself from a few
    hours to 10
  • years after an ACE inhibitor has been first
    taken.Therefore
  • physicians fail to recognize the association.

26
MANAGEMENT OF ACE INHIBITOR-INDUCED ANGIOEDEMA
ACUT TREATMENT
Avoid ACE inhibitors Conservative treatment
antihistamines with or without steroids
Tongue and upper airway are involved, intramus
cular adrenaline should be used, some patients
require an artificial airway C1 inhibitor
concentrate,FFP, SDP, kallikrein inhibitor or
bradykinin receptor antagonist has been
successfully
27
MANAGEMENT OF ACE INHIBITOR-INDUCED ANGIOEDEMA
LONG-TERM
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.
28
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
29
HEREDITARY ANGIOEDEMA (HAE)
Type I (Donaldson, 1963) Type II (Rosen, 1965)
Type III (Bork, Binkley, 2000, Martin 2001)
C1-inhibitor (C1-INH) deficiency
Normal C1-INH
30
HEREDITARY ANGIOEDEMA (HAE) Type I. II
  • autosomal dominant inheritance
  • deficiency of C1 inhibitor
  • onset of symptoms in childhood
  • prevalence 110 000, 150 0000
  • mortality 20-30

31
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?

32
HEREDITARY ANGIOEDEMA (HAE) TYPE I II
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
33
PATHOMECHANISM
edema
34
DIAGNOSIS OF HAE
  • Family history
  • Clinical symptoms
  • Measurement of complement

asymptomatic
Confirm the diagnosis in uncertain cases Prenatal
diagnostics
Agostoni A Medicine 1992
35
SUBCUTANEOUS SYMPTOMS
Farkas H Acta Dermato-Venereol 2001
36
SUBCUTANEOUS SYMPTOMS
Bork K Am J Med 2006
37
SUBMUCOSAL SYMPTOMS Upper airway mucosa
Pharyngeal edema
Laryngeal edema
Bork K Transfus Apher Sci 2003 Tsunoda
Laryngoscope 2000
38
SUBMUCOSAL SYMPTOMS Intestinal mucosa
  • intense, colicky abdominal pain
  • nausea and vomiting
  • postattack (watery) diarrhea
  • can mimic an acute abdomen

Unnecessary surgical intervention
Bork K Am J Gastroenterol 2006
39
ABDOMINAL ULTRASOUND
Nonspecific but extremely sensitive method
ascites
edematous intestinal wall
  • 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.

Farkas H Eur J Gastroenterol Hepatol 2001 Acta
Paediatr 2002
40
COMPLEMENT PROFILES IN C1-INH DEFICIENCIES
Type C1q C1-INH antigen C1-INH function C4 Anti-C1-INH
HAE-I N L L L -
HAE-II N N/H L L -
AAE-I L L L L -
AAE-II (autoimm.) L N/L L L
Agostoni A J Allergy Clin Immunol. 2004
41
MANAGEMENT
COUNSELING EDUCATION
TREATMENT
FOLLOW-UP
Bowen J Allergy Clin Immunol 2004
42
COUNSELING EDUCATION
Individualized information to patient and parents
Written information to school, pediatrician,
family practitioner
Multilanguage infocard, hne service, hospital for
emergencies
Patient diary
Drug for emergency
Patient organizations, websites
Farkas H Transfus Apher Sci 2003
43
TREATMENT
stress
minor trauma
infection
Elimination of precipitating factors
drugs
hormons (estrogens)
Prophylaxis with drugs
Management of attack
Farkas H Lancet 2001 Bouillet L Dermatology 2003

44
PROPHYLAXIS WITH DRUGS
Long-term
antifibrinolytic agents (tranexamic acid)
1 attack per month or history of
life-threatening attacks
attenuated androgens
C1-INH concentrate
Short-term
attenuated androgens 5 days before the
intervention and for 2 days after
Before surgery and instrumentation on the
oropharynx, head and neck
C1-INH concentrate, SDP, FFP
Cicardi M J Allergy Clin Immunol 1991 Farkas H J
Oral Maxillofac Surg 1999
45
SIDE EFFECTS
ATTENUATED ANDROGENS
ANTIFIBRINOLYTIC AGENTS
Tranexamic acid Epsilon-amino-caproic acid
Danazol, stanozolol, oxandrolone
-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 -transamina
se?, hepatocellular adenoma and liver carcinoma
thrombosis postural hypotension muscular pain and
weekness creatine kinase ? aldolase
? rhabdomyolysis myositis fatigue
Cicardi M J Allergy Clin Immunol. 1997 Széplaki
G J Allergy Clin Immunol. 2005
46
FOLLOW-UP
SIDE EFFECTS
MINIMAL EFFECTIVE DOSE
blood cell count liver renal function,
lipid profile, CK urinalysis
abdominal ultrasound HIV, Hepatitis A,B,C,
serology vaccination to Hepatitis B
ADJUSTMENT OF THERAPY
ALTERATION IN STATUS
Gompels M Clin Exp Immunol 2005 Farkas H Lancet
1999
47
MANAGEMENT OF ATTACK
pharyngeal/laryngeal face, lips edema abdominal
attack severe edema of extremities, trunk neck
genitals
SEVERE
C1-INH concentrate, FFP,SDF
Edema of the extremities, Mild abdominal attack
tranexamic acid or danazol for 2-3 days
MILD
Corticosteroids, antihistamines, and epinephrine
are INEFFECTIVE
Farkas H JACI 2007 Prematta M Ann Allergy Asthma
Immunol 2007 Bork K Transfusion 2005
48
FUTURE TREATMENT OPTIONS
Trauma
Prekallikrein
Kallikrein inhibitor, DX88
XIIa Faktor
ACE
Kallikrein
hC1-INH, rhC1-INH
Bradykinin
Des-Arg9-Bradykinin
HMW-Kininogen
Bradykinin receptor antagonist Icatibant
edema
Bork K J Allergy Clin Immunol 2007 Van Doom M J
Allergy Clin Immunol 2005 Levy J Expert Opin
Investig Drugs 2006
49
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