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Hereditary Angioedema Attacks: Local Swelling at Multiple Sites

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Abstract

Hereditary angioedema (HAE) patients experience recurrent local swelling in various parts of the body including painful swelling of the intestine and life-threatening laryngeal oedema. Most HAE literature is about attacks located in one anatomical site, though it is mentioned that HAE attacks may also involve multiple anatomical sites simultaneously. A detailed description of such multi-location attacks is currently lacking. This study investigated the occurrence, severity and clinical course of HAE attacks with multiple anatomical locations. HAE patients included in a clinical database of recombinant human C1-inhibitor (rhC1INH) studies were evaluated. Visual analog scale scores filled out by the patients for various symptoms at various locations and investigator symptoms scores during the attack were analysed. Data of 219 eligible attacks in 119 patients was analysed. Thirty-three patients (28 %) had symptoms at multiple locations in anatomically unrelated regions at the same time during their first attack. Up to five simultaneously affected locations were reported. The observation that severe HAE attacks often affect multiple sites in the body suggests that HAE symptoms result from a systemic rather than from a local process as is currently believed.

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Abbreviations

B1R:

Bradykinin receptor B1

B2R:

Bradykinin receptor B2

C1INH:

C1-inhibitor

CRP:

C-reactive protein

fC1INH:

Functional C1INH

HAE:

Hereditary angioedema

HK:

High-molecular-weight kininogen

ISS:

Investigator symptom score

OFPL:

Oro-facial-pharyngeal-laryngeal

RCT:

Randomized controlled trial

rhC1INH:

Recombinant human C1-inhibitor

TNF:

Tumour necrosis factor

VAS:

Visual analog scale

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Authors and Contributors

All authors participated in the study design and protocol for data analysis. The analyses were carried out by Zonne Hofman who also prepared the first draft of the manuscript. Erik Hack and Anurag Relan contributed to the interpretation of the data and revised the manuscript. All authors read and approved the final manuscript.

Conflict of Interest

Zonne Hofman declares that she has no conflict of interest. Erik Hack has received consultancy fees from Pharming Technologies in the past. Anurag Relan is an employee of Pharming Technologies.

Funding

The studies described in this paper were not funded. The data utilized for these studies are from clinical trials sponsored by Pharming Technologies NV.

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Authors and Affiliations

  1. Laboratory for Translational Immunology, University Medical Center Utrecht, Lundlaan 6, P.O. Box 85090, 3508, GA, Utrecht, The Netherlands

    Zonne L. M. Hofman & C. Erik Hack

  2. Pharming Technologies NV, Leiden, The Netherlands

    Anurag Relan

Authors
  1. Zonne L. M. Hofman
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  2. Anurag Relan
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  3. C. Erik Hack
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Corresponding author

Correspondence to Zonne L. M. Hofman.

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Hofman, Z.L.M., Relan, A. & Hack, C.E. Hereditary Angioedema Attacks: Local Swelling at Multiple Sites. Clinic Rev Allerg Immunol 50, 34–40 (2016). https://doi.org/10.1007/s12016-014-8463-6

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  • DOI: https://doi.org/10.1007/s12016-014-8463-6

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