Wegener's granulomatosis with dural involvement as the initial clinical manifestation

Intern Med. 1997 Jul;36(7):514-8. doi: 10.2169/internalmedicine.36.514.

Abstract

We treated a patient with an atypical presentation of Wegener's granulomatosis (WG) with dural involvement as the initial clinical manifestation. A 37-year-old man had a dural lesion without lower respiratory tract or renal manifestations in the initial clinical course. His only initial symptom was headache, and at disease onset computed tomography (CT) and magnetic resonance imaging (MRI) of the head revealed bilateral abnormal subdural masses. The diagnosis of WG was made based on the results of needle biopsy of the nasal polyps and the finding of positive circulating antineutrophil cytoplasmic antibodies (c-ANCA). He achieved remission on daily prednisone and cyclophosphamide with the later addition of sulfamethoxazole-trimethoprim.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • Autoimmune Diseases / pathology*
  • Biopsy
  • Cyclophosphamide / therapeutic use
  • Dura Mater / immunology
  • Dura Mater / pathology*
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology
  • Granulomatosis with Polyangiitis / pathology*
  • Headache / etiology*
  • Hematoma, Subdural / diagnostic imaging
  • Hematoma, Subdural / etiology*
  • Hematoma, Subdural / pathology
  • Hematoma, Subdural / surgery
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Nasal Polyps / etiology
  • Nasal Polyps / pathology
  • Prednisone / therapeutic use
  • Tomography, X-Ray Computed
  • Trimethoprim, Sulfamethoxazole Drug Combination / therapeutic use

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Trimethoprim, Sulfamethoxazole Drug Combination
  • Cyclophosphamide
  • Prednisone