PMID- 29393169
OWN - NLM
STAT- MEDLINE
DCOM- 20180503
LR  - 20180503
IS  - 1423-0097 (Electronic)
IS  - 1018-2438 (Linking)
VI  - 175
IP  - 3
DP  - 2018
TI  - Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical 
      Phenotypes.
PG  - 126-135
LID - 10.1159/000486312 [doi]
AB  - Recurrent angioedema (AE) without wheals is increasingly recognized as a clinical 
      entity and a frequent cause of admission to the emergency room. The Hereditary 
      Angioedema Working Group (HAWK) classification allowed the scientific community 
      to go beyond the semantic confusion that dominated this topic for decades. This 
      classification distinguishes hereditary and acquired forms of AE, either related 
      or unrelated to C1 inhibitor deficiency. Recently, additional mechanisms have 
      been involved in the AE pathogenesis, including the uncontrolled activation of 
      factor XII, generation of vasoactive mediators that induce dysregulation of 
      endothelial functions, and bidirectional interactions between mast cell-derived 
      mediators and the plasma contact system. Thus, recurrent AE can be determined by 
      multiple and concurrent mechanisms that may generate distinct clinical phenotypes 
      of the disease. Frequency, severity, and the location of attacks are quite 
      different from patient to patient and, even in the same patient, they may change 
      throughout the course of life. The severity of the clinical phenotype strongly 
      influences the burden of the disease and patients' quality of life. Despite major 
      advances in our understanding of recurrent AE, many unsolved questions remain, 
      leaving several unmet needs for patients and caregivers. This review is focused 
      on a description of different AE phenotypes and the concurrent mechanisms leading 
      to their pathogenesis. A better definition of cellular and molecular pathways 
      responsible for the distinct AE phenotypes may help to improve diagnosis and may 
      lead to a personalized approach to prophylaxis and treatment of the disease.
CI  - (c) 2018 S. Karger AG, Basel.
FAU - Bova, Maria
AU  - Bova M
AD  - Department of Translational Medicine, University Federico II, Naples, Italy.
AD  - Department of Medicine, Division of Allergy and Clinical Immunology, University 
      of Salerno, Baronissi, Italy.
FAU - De Feo, Giulia
AU  - De Feo G
AD  - Department of Medicine, Division of Allergy and Clinical Immunology, University 
      of Salerno, Baronissi, Italy.
FAU - Parente, Roberta
AU  - Parente R
AD  - Department of Medicine, Division of Allergy and Clinical Immunology, University 
      of Salerno, Baronissi, Italy.
FAU - De Pasquale, Tiziana
AU  - De Pasquale T
AD  - Division of Allergy, Civitanova Marche Hospital, Civitanova Marche, Italy.
FAU - Gravante, Carmela
AU  - Gravante C
AD  - Department of Translational Medicine, University Federico II, Naples, Italy.
FAU - Pucci, Stefano
AU  - Pucci S
AD  - Division of Allergy, Civitanova Marche Hospital, Civitanova Marche, Italy.
FAU - Nettis, Eustachio
AU  - Nettis E
AD  - Department of Allergy and Clinical Immunology, University of Bari, Bari, Italy.
FAU - Triggiani, Massimo
AU  - Triggiani M
AD  - Department of Medicine, Division of Allergy and Clinical Immunology, University 
      of Salerno, Baronissi, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20180126
PL  - Switzerland
TA  - Int Arch Allergy Immunol
JT  - International archives of allergy and immunology
JID - 9211652
RN  - Acquired angioedema
SB  - IM
MH  - *Angioedema/diagnosis/etiology/physiopathology/therapy
MH  - *Angioedemas, Hereditary/diagnosis/etiology/physiopathology/therapy
MH  - Humans
MH  - Phenotype
OTO - NOTNLM
OT  - Acquired angioedema
OT  - Differential diagnosis
OT  - Hereditary angioedema
OT  - Mediators
OT  - Pathogenesis
OT  - Phenotypes
EDAT- 2018/02/03 06:00
MHDA- 2018/05/04 06:00
CRDT- 2018/02/03 06:00
PHST- 2017/07/17 00:00 [received]
PHST- 2017/12/13 00:00 [accepted]
PHST- 2018/02/03 06:00 [pubmed]
PHST- 2018/05/04 06:00 [medline]
PHST- 2018/02/03 06:00 [entrez]
AID - 000486312 [pii]
AID - 10.1159/000486312 [doi]
PST - ppublish
SO  - Int Arch Allergy Immunol. 2018;175(3):126-135. doi: 10.1159/000486312. Epub 2018 
      Jan 26.