Angioneurotic Edema

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Contents 1 More Specific Terms 2 Introduction 3 Etiology 4 Pathology 5 Genetics 6 Clinical-manifestations 7 Laboratory 8 Radiology 9 Complications 10 Management 11 More General Terms 12 Additional Terms 13 References

More Specific Terms

• hereditary angioedema; hereditary angioneurotic edema (HANE)

Introduction

• Periodically recurring epiosodes of noninflammatory swelling of the skin, mucous membranes, viscera, & brain, of sudden onset & lasting hours to days, occasionally with arthralgia, purpura, or fever.

Etiology

• food allergies
• urticaria
• C1 esterase inhibitor deficiency ( hereditary angioneurotic edema)
• acquired C1 esterase deficiency associated with:

• adenocarcinoma
• lymphoproliferative disorder
• autoimmune disease: lupus erythematosus

• pharmacologic agents

• angiotensin-converting enzyme ( ACE) inhibitor

• possibly stress & emotional factors

Pathology

• mast cell release of histamine & other mediators
• generally occurs in association with urticaria
• angioedema involves deeper layers of the dermis & subcutaneous tissues in contrast to urticaria which involves only the epidermis & superficial layers of the dermis
• edema is noninflammatory
• may involve the skin, mucous membranes, viscera, & brain
• hereditary form is a complement disorder & is not related to release mediators by mast cells
• acquired C1 esterase deficiency:

• autoantibodies to C1 inhibitor
• tumor-mediated complement fixation overwelming the synthetic capacity for C1 esterase
• diminished serum complement C1 esterase inhibitor, C1q, C2, C4

Genetics

• see hereditary angioedema

Clinical-manifestations

• episodic edema of extremities, face & tongue
• onset is sudden & lasting hours to days
• edema may involve skin, oropharynx, genitalia [5]
• severe cases may lead to airway obstruction [6]
• angioedema without urticaria suggests C1 esterase inhibitor deficiency
• genital edema in a child riding a bicycle suggests hereditary angioedema
• urticaria in associated with angioedema suggests other than ereditary form
• non- pitting pruritic or burning wheals, single or multiple

• face
• tongue
• larynx (common in hereditary form)
• hands
• feet
• genitalia

• pruritic lesions generally resolve without treatment
• lesions of hereditary angioedema are not pruritic
• gastrointestinal ( GI) complaints

• due to edema of intestinal mucosa
• nausea/vomiting
• diarrhea
• abdominal pain
• symptoms of intestinal obstruction in patients with hereditary form (duration 3-5 days)

• arthralgia, purpura, or fever may be present
• trauma, manual labor or dental extraction may precipitate attacks in hereditary angioedema

Laboratory

• C1 esterase deficiency in hereditary form

• normal in food allergy, drug-induced angioedema, idiopathic angioedema
• obtain functional & antigenic levels [5]

• diminished complement C4 in hereditary form

• serum C4 can be monitored to assess efficacy of treatment
• C4 in normal in food allergy, drug-induced angioedema, idiopathic angioedema
• obtain function & antigenic levels for diagnosis [5]

• diminished serum complement C2 during attacks
• diminished complement C1 in acquired form (i.e. C1q)
• complete blood count ( CBC)
• erythrocyte sedimentation rate (ESR)
• serum beta-tryptase within 4 hour confirms diagnosis; a negative value does not exclude
• urinalysis
• liver function tests
• specific testing according to presentation

• cryoglobulins, rheumatoid factor, anti SS-A, anti SS-B, Reiter protein reagin, hepatitis serologies (A, B, C), HIV, CH50

• skin biopsy: ineffective treatment or suspected vasculitis

Radiology

• chest X-ray

Complications

• cerebral edema or epiglottal edema may cause death

Management

• assess for signs of airway compromise if swelling involves airway
• assess for impending shock (see anaphylaxis)
• epinephrine

• signs of airway compromise
• impending shock
• hereditary form does not respond to epinephrine
• effective by racemic nebulized form or SC injection

• emergency tracheostomy if airway does not clear in response to administration of epinephrine
• antihistamines ( hydroxyzine, diphenhydramine)

• will not cause harm in ACE inhibitor induced angioedema
• will not cause harm in complement-mediated angioedema

• glucocorticoids
• androgens for hereditary form

• testosterone
• danazol
• stanozolol

• C1 inhibitor therapy is investigational
• epsilon-aminocaproic acid & tranexemic acid may be useful for C1-inhibitor deficiency
• stop offending drug if drug-induced

• if due to ACE inhibitor, ARB is reasonable alternative [7]

• plasma kallikrein inhibitor ecallantide ( Kalbitor) FDA-approved in 2009

More General Terms

• edema (swelling)

Additional Terms

• complement C1 inhibitor; C1 esterase inhibitor (Cinryze, Berinert)
• complement C1s

References

1. Stedman's Medical Dictionary 24th ed, Williams & Wilkins, Baltimore, 1982
2. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
3. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 864
4. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 25-26
5. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006
6. Prescriber's Letter 11(7):31 2004
7. Veterans Administration, Pharmacy, Mather, CA

angioneurotic edema; angioedema; atrophedema; Bannister's disease; Milton's disease; Quincke's disease; periodic or Quincke's edema; giant urticaria or hives; urticaria gigans, gigantea, or tuberosa.