Churg-Strauss syndrome concomitant with chronic symmetrical dacryoadenitis suggesting Mikulicz's disease

Intern Med. 2012;51(17):2457-61. doi: 10.2169/internalmedicine.51.7725. Epub 2012 Sep 1.


A case of Churg-Strauss syndrome complicated by chronic symmetrical dacryoadenitis suggestive of Mikulicz's disease is herein presented. A 72-year-old Japanese man, who had been previously diagnosed with asthma, presented with weakness of the left leg and purpura on the lower extremities. A neurological examination showed multiple mononeuropathies and a laboratory examination revealed elevated eosinophil counts, IgE levels and the presence of Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCAs). Churg-Strauss syndrome was diagnosed, although the patient also exhibited bilateral swelling of the lachrymal glands. Furthermore, elevated serum IgG4 levels, an infiltration of a relatively large number of IgG4-positive plasmacytes in the nasal mucosa and hypocomplementemia were also observed. These findings were consistent with a diagnosis of Mikulicz's disease (MD). Oral prednisolone (30 mg) was administered and the swelling of the lachrymal glands resolved. Churg-Strauss syndrome may be accompanied by Mikulicz's disease (an IgG4-related disease), and common pathogeneses between Churg-Strauss syndrome and IgG4-related disease may exist.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Churg-Strauss Syndrome / diagnosis*
  • Churg-Strauss Syndrome / drug therapy
  • Churg-Strauss Syndrome / epidemiology*
  • Dacryocystitis / diagnosis*
  • Dacryocystitis / drug therapy
  • Dacryocystitis / epidemiology*
  • Humans
  • Immunoglobulin E / blood
  • Immunoglobulin G / blood
  • Immunoglobulins, Intravenous / therapeutic use
  • Lacrimal Apparatus / pathology
  • Male
  • Methylprednisolone / therapeutic use
  • Mikulicz' Disease / diagnosis*
  • Mikulicz' Disease / drug therapy
  • Mikulicz' Disease / epidemiology*
  • Plasma Cells / pathology
  • Prednisolone / therapeutic use
  • Treatment Outcome


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunoglobulin G
  • Immunoglobulins, Intravenous
  • Immunoglobulin E
  • Prednisolone
  • Methylprednisolone