Granulomatosis polyangiitis

BMJ Case Rep. 2019 Jul 8;12(7):e228693. doi: 10.1136/bcr-2018-228693.

Abstract

A 22-year-old female patient was admitted to hospital after being referred from the oral medicine clinic where she had been seen for persistent gingivitis and mouth ulcers. She described an insidious history of persistent fevers, dry cough and unexplained weight loss over 4-6 weeks. Imaging showed extensive bilateral pulmonary nodules with mediastinal lymphadenopathy and two lesions in the pancreas. MRI revealed these lesions to be well-defined fluid-filled cysts in the tail of the pancreas, without features of malignancy. Core biopsies taken from her lung nodules demonstrated features of vasculitis with granulomata. This was consistent with her positive immunology for c-antinuclear cytoplasmic antibodies and proteinase-3, which were sent after her fever failed to settle with antibiotic treatment. In keeping with a diagnosis of vasculitis, the patient showed a significant clinical and biochemical response to intravenous methylprednisolone and high-dose daily prednisolone thereafter.

Keywords: TB and other respiratory infections; pancreas and biliary tract; radiology; vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Administration, Intravenous
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Diagnosis, Differential
  • Female
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use
  • Granulomatosis with Polyangiitis / diagnostic imaging
  • Granulomatosis with Polyangiitis / drug therapy*
  • Granulomatosis with Polyangiitis / metabolism
  • Granulomatosis with Polyangiitis / pathology*
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lymphadenopathy / pathology*
  • Mediastinal Neoplasms / diagnostic imaging
  • Mediastinal Neoplasms / pathology
  • Methylprednisolone / administration & dosage
  • Methylprednisolone / therapeutic use
  • Myeloblastin / blood
  • Pancreas / diagnostic imaging
  • Pancreas / pathology
  • Treatment Outcome
  • Young Adult

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Glucocorticoids
  • Myeloblastin
  • Methylprednisolone