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A Comprehensive Approach to Urticaria: From Clinical Presentation to Modern Biological Treatments Through Pathogenesis

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Cell Biology and Translational Medicine, Volume 12

Part of the book series: Advances in Experimental Medicine and Biology ((CBTMED,volume 1326))

Abstract

Urticaria is characterized by the cutaneous presence of wheals (hives), angioedema or both. Acute and chronic urticaria are distinguished based on a duration of less or more than 6 weeks. Chronic urticaria can be further classified into a spontaneous form and several inducible types triggered by specific external stimuli. Lifetime prevalence of urticaria may be up to 20%, with the acute form being way more common than the chronic one. Exacerbating factors (e.g. infections, drugs, food) and immune system alterations have been investigated as main triggers of mast cell activation, which in turn leads to increased vascular permeability and extravasation of inflammatory cells. While diagnostic workup is focused upon history taking, several emerging biomarkers correlate with severity and/or prognosis of the disease and can be necessary to differentiate chronic spontaneous urticaria from other disorders, such as vasculitis and autoinflammatory diseases. Treatment of acute urticaria is based upon H1 antihistamines and short courses of steroids. While H1 antihistamines are also used in chronic spontaneous urticaria, omalizumab is the standard of care in patients who are unresponsive to these. Recently, several new drugs have entered clinical trials to offer a therapeutic possibility for patients unresponsive to omalizumab. Numerous target molecules, such as mediators of mast cells activation, are under investigation. Amongst these, new anti-IgE therapies and possibly IL-5 pathway blockade seem to have reached enough data to move to advanced clinical trials.

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Abbreviations

AAC:

area above the curve

ACEi:

Angiotensin converting enzyme inhibitors

ANA:

anti-nuclear antibodies

AOSD:

Adult onset Still’s disease

ASST:

autologous serum skin test

AU:

Acute urticaria

BHRA:

basophil histamine release assay

BTK:

Bruton’s tyrosine kinase

C5aR:

Complement 5a receptor

CAPS:

Cryopirin-associated periodic syndromes

CIndU:

Chronic Inducible Urticaria

COX-1:

cyclooxygenase 1

CRP:

c-reactive protein

CRTH2:

chemoattractant receptor homologous molecule expressed on the Th2 cell

CSU:

Chronic Spontaneous Urticaria

CU:

Chronic urticaria

CU-Q20L:

Chronic Urticaria Quality of Life

DARPins:

designed ankyrin repeat proteins

ESR:

erythrocyte sedimentation rate

F1 + 2:

Prothrombin fragment 1 + 2

FcεRI:

high-affinity IgE receptor

FcεRIα:

high-affinity IgE receptor alpha chain

HLA:

Human Leukocyte Antigen

HUV:

hypocomplementemic urticarial vasculitis

IFNγ:

interferon gamma

IL-1:

interleukin 1

IL-18:

interleukin 18

IL-24:

interleukin 24

IL-25:

interleukin 25

IL-33:

interleukin 33

IL-4:

interleukin 4

IL-5:

interleukin 5

IL-6:

interleukin 6

Il-6sR:

interleukin 6 soluble receptor

InH-AAE:

idiopathic nonhistaminergic acquired angioedema

ITAMs:

immunoreceptor tyrosine-based activation motifs

LCN2:

serum Lipocalin-2

MCT:

tryptase-positive chymase-negative mast cells

MCTC:

tryptase-positive chymase-positive mast cells

MMP-9:

matrix metalloproteinase-9

MPV:

mean platelet volume

NGF:

nerve growth factor

NSAID:

non-steroidal anti-inflammatory drug

PG:

Prostaglandins.

PGD2:

prostaglandin D2

sgp130:

soluble glycoprotein 130

Siglec:

Sialic acid-binding immunoglobulin-like lectin

SYK:

spleen tyrosine kinase

Th1:

T helper 1

Th2:

T helper 2

TPO:

thyroid peroxidase

TSH:

thyroid stimulating hormone

TSLP:

thymic stromal lymphopoietin

UAS7:

weekly urticaria activity score

USS:

Urticaria Severity Score (USS)

UV:

urticarial vasculitis

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Authors and Affiliations

  1. Internal medicine, division of Clinical Immunology, Humanitas Clinical and Research Center – IRCCS, Milan, Italy

    Marco Folci, Giacomo Ramponi & Enrico Brunetta

  2. Department of Biomedical Sciences, Humanitas University, Milan, Italy

    Marco Folci & Enrico Brunetta

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  1. (emeritus), Ottawa Hospital Research Institute, Ottawa, ON, Canada

    Kursad Turksen

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Folci, M., Ramponi, G., Brunetta, E. (2020). A Comprehensive Approach to Urticaria: From Clinical Presentation to Modern Biological Treatments Through Pathogenesis. In: Turksen, K. (eds) Cell Biology and Translational Medicine, Volume 12. Advances in Experimental Medicine and Biology(), vol 1326. Springer, Cham. https://doi.org/10.1007/5584_2020_612

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