Churg-Strauss syndrome with a clinical condition similar to IgG4-related kidney disease: a case report

Intern Med. 2012;51(10):1233-8. doi: 10.2169/internalmedicine.51.6074. Epub 2012 May 15.

Abstract

A 68-year-old Japanese woman with asthma of recent onset and a long history of membranous glomerulonephropathy (MN) was admitted because of multifocal pulmonary infiltrates, marked eosinophilia, mild renal dysfunction, a rash on her feet, and right median nerve paralysis. Although MPO- and PR3-ANCA were negative, skin biopsy demonstrated leukocytoclastic vasculitis and Churg-Strauss Syndrome (CSS) was diagnosed. She also had salivary gland swelling and a high serum IgG4 level. Renal biopsy revealed MN with eosinophil-rich tubulointerstitial nephropathy. Her symptoms resolved after the start of corticosteroid therapy. The present case shows that ANCA-negative CSS can have a clinical condition similar to IgG4-related kidney disease.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Churg-Strauss Syndrome / complications*
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / immunology*
  • Female
  • Glomerulonephritis, Membranous / complications
  • Glomerulonephritis, Membranous / diagnosis
  • Glomerulonephritis, Membranous / immunology
  • Humans
  • Immunoglobulin G / blood
  • Immunoglobulin G / metabolism*
  • Kidney Diseases / complications*
  • Kidney Diseases / diagnosis
  • Kidney Diseases / immunology*
  • Nephritis, Interstitial / complications
  • Nephritis, Interstitial / diagnosis
  • Nephritis, Interstitial / immunology

Substances

  • Immunoglobulin G