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Acquired Angioedema Due to C1 Inhibitor Deficiency Workup

  • Author: Amanda T Moon, MD; Chief Editor: William D James, MD  more...
 
Updated: Mar 07, 2016
 

Laboratory Studies

Screening is conducted by determining the C4 level, which is decreased both during and in between clinical flares. If the C4 level is normal but clinical suspicion remains high, the C4 testing should be repeated, and, additionally, qualitative and functional values of C1 inhibitor (C1-INH) should be obtained. Antigenic levels of C1q are usually low and are useful to distinguish hereditary angioedema from acquired angioedema.[2]

Test results for acquired angioedema types I and II are as follows:

  • Low C1-INH level
  • Low C1q levels (except 1 reported case)
  • Low C4 and C2 levels

Acquired angioedema type II shows positive immunoblot assay findings for the 95-kd C1-INH cleavage product.

An enzyme-linked immunosorbent assay (ELISA) has been developed that measures the neutralizing capacity of anti-C1-INH antibodies in plasma.[9]

Other laboratory findings are related to associated illnesses.

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Abdominal Radiography

During attacks of gastrointestinal edema, abdominal ultrasonography or computed tomography scanning may show edematous thickening of the intestinal wall, a fluid layer around the bowel, and large amounts of free peritoneal fluid.

Other findings may be referable to an associated illness.

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Histologic Findings

Histologic findings are indistinguishable from other angioedema types. Features include sparse perivascular mononuclear cell infiltrate and reticular dermal, subcutaneous, or submucosal edema.

Vasodilation may be seen.

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Contributor Information and Disclosures
Author

Amanda T Moon, MD Resident Physician, Department of Dermatology, University of Rochester, Strong Memorial Hospital

Amanda T Moon, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Ru'aa Al Harithy, MBBS, FRCPC Clinical Fellow in Laser and Cosmetic Dermatology, Division of Dermatology, SunnyBrook Hospital, University of Toronto Faculty of Medicine, Canada

Ru'aa Al Harithy, MBBS, FRCPC is a member of the following medical societies: American Academy of Dermatology, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Paul Krusinski, MD Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Kathleen M Rossy, MD Staff Physician, Department of Dermatology, New York Medical College, Metropolitan Hospital

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

References
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  2. Caballero T, Baeza ML, Cabañas R, et al. Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis. J Investig Allergol Clin Immunol. 2011. 21(5):333-47; quiz follow 347. [Medline].

  3. Cugno M, Zanichelli A, Foieni F, Caccia S, Cicardi M. C1-inhibitor deficiency and angioedema: molecular mechanisms and clinical progress. Trends Mol Med. 2009 Feb. 15(2):69-78. [Medline].

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  9. Engel R, Rensink I, Roem D, Brouwer M, Kalei A, Perry D, et al. ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients. J Immunol Methods. 2015 Aug 28. [Medline].

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  14. Klossowski N, Braun SA, von Gruben V, Losem C, Plewe D, Homey B, et al. [Acquired angioedema with C1-INH deficiency and accompanying chronic spontaneous urticaria in a patient with chronic lymphatic B cell leukemia]. Hautarzt. 2015 Sep 3. [Medline].

  15. Desai HG, Shah SS. Recurrent intestinal obstruction with acquired angio-oedema, due to C1-esterase inhibitor deficiency. J Assoc Physicians India. 2014 Jun. 62 (6):524-5. [Medline].

  16. Kaur R, Williams AA, Swift CB, Caldwell JW. Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. J Asthma Allergy. 2014. 7:165-7. [Medline].

  17. Dreyfus DH, Na CR, Randolph CC, Kearney D, Price C, Podell D. Successful rituximab B lymphocyte depletion therapy for angioedema due to acquired C1 inhibitor protein deficiency: association with reduced C1 inhibitor protein autoantibody titers. Isr Med Assoc J. 2014 May. 16 (5):315-6. [Medline].

  18. Rottem M, Mader R. Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. J Rheumatol. 2010 Jan. 37(1):209. [Medline].

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