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Eosinophilic vasculitis in connective tissue disease

K R Chen1, W P Su, M R Pittelkow

  • 1Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.

Journal of the American Academy of Dermatology
|August 1, 1996
PubMed
Summary

Eosinophilic vasculitis in connective tissue disease (CTD) involves eosinophil granule major basic protein (MBP) deposition, leading to vessel damage. This variant form of vasculitis is linked to hypocomplementemia and diminished perivascular mast cells.

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Area of Science:

  • Rheumatology
  • Immunology
  • Pathology

Background:

  • Connective tissue disease (CTD) commonly presents with neutrophilic and lymphocytic vascular inflammation.
  • A distinct subset of eight CTD patients exhibited eosinophilic vasculitis, prompting further investigation.
  • This study aimed to characterize this eosinophilic variant of vasculitis in CTD patients.

Purpose of the Study:

  • To characterize a distinct variant of vasculitis in connective tissue disease (CTD) characterized by eosinophilic infiltration.
  • To compare the clinical and laboratory findings of eosinophilic vasculitis with neutrophilic vasculitis in CTD patients.

Main Methods:

  • Compared clinical and laboratory data from eight CTD patients with eosinophilic vasculitis and nine with neutrophilic vasculitis.

Related Experiment Videos

  • Utilized indirect immunofluorescence to detect eosinophil major basic protein (MBP), neutrophil elastase, and mast cell tryptase in lesional tissue.
  • Assayed serum MBP levels and eosinophil survival-enhancing activity in a subset of patients.

    • Main Results:

    • Eosinophilic vasculitis patients presented with hypocomplementemia and peripheral blood eosinophilia, elevated serum MBP, and prolonged eosinophil survival.
    • Immunofluorescence revealed prominent angiocentric eosinophil MBP staining and diminished mast cell tryptase in eosinophilic vasculitis.
    • Neutrophilic vasculitis patients lacked peripheral eosinophilia, showed variable hypocomplementemia, and had prominent neutrophil elastase staining.

    Conclusions:

    • Eosinophilic vasculitis in CTD with hypocomplementemia is associated with vessel wall destruction mediated by cytotoxic eosinophil granule MBP.
    • Eosinophils are implicated as key mediators of vascular damage in this specific vasculitis subtype.
    • Diminished perivascular mast cells suggest mast cell degranulation may play a role in the pathogenesis.