Mast cells, cortistatin, and its receptor, MRGPRX2, are linked to the pathogenesis of chronic prurigo

J Allergy Clin Immunol. 2022 Jun;149(6):1998-2009.e5. doi: 10.1016/j.jaci.2022.02.021. Epub 2022 Mar 10.

Abstract

Background: Chronic prurigo (CPG) is characterized by intensive itch and interactions among nerves, neuropeptides, and mast cells (MCs). The role of some neuropeptides such as cortistatin (CST) and its receptor, Mas-related G protein-coupled receptor X2 (MRGPRX2), in CPG remains poorly investigated.

Objectives: We evaluated first whether CST activates human skin MCs, and second whether CST and MRGPRX2 are expressed in the skin of CPG patients, and by which cells.

Methods: Skin prick tests and microdialysis with CST were performed in 6 and 1 healthy volunteers, respectively. Degranulation of human skin MCs was assessed using β-hexosaminidase and histamine release assays. Skin samples from 10 patients with CPG and 10 control subjects were stained for CST, MCs, and MRGPRX2 (protein and mRNA) using immunohistochemistry, immunofluorescence, and/or in situ hybridization. Flow cytometry was used to assess CST in human skin MCs. MRGPRX2 levels were measured in serum by ELISA.

Results: CST induced concentration-dependent degranulation of human skin MCs in vivo and ex vivo. Skin lesions of CPG patients exhibited markedly higher numbers of CST-expressing cells, CST-expressing MCs, MRGPRX2-expressing cells, and MRGPRX2 mRNA-expressing cells than nonlesional skin. MCs were the main MRGPRX2 mRNA-expressing cells in the lesions of most CPG patients (70%). Stimulation of human skin MCs with anti-IgE led to a release of CST. The number of MRGPRX2-expressing cells correlated with disease severity (r = 0.649, P = .04). MRGPRX2 serum levels in CPG patients correlated with disease severity (r = 0.704, P = .023) and quality-of-life impairment (r = 0.687, P = .028).

Conclusions: CST and MRGPRX2 may contribute to the pathogenesis of CPG and should be evaluated in further studies as potential biomarkers and novel therapeutic targets.

Keywords: Chronic prurigo; MRGPRX2; cortistatin; degranulation; mast cell; severity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Degranulation
  • Humans
  • Mast Cells / physiology
  • Nerve Tissue Proteins / genetics
  • Neuropeptides*
  • Prurigo*
  • RNA, Messenger
  • Receptors, G-Protein-Coupled / genetics
  • Receptors, Neuropeptide / genetics

Substances

  • MRGPRX2 protein, human
  • Nerve Tissue Proteins
  • Neuropeptides
  • RNA, Messenger
  • Receptors, G-Protein-Coupled
  • Receptors, Neuropeptide
  • cortistatin