Dacryoadenitis with Ptosis and Diplopia as the Initial Presentation of Granulomatosis with Polyangiitis

Intern Med. 2017 Oct 1;56(19):2649-2653. doi: 10.2169/internalmedicine.8761-16. Epub 2017 Sep 6.

Abstract

A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine. Dacryoadenitis in the form of an orbital inflammatory pseudotumor may be an initial presenting feature of GPA, sometimes as the limited phenotype, and occasionally progressing to systemic disease.

Keywords: Wegener's granulomatosis; idiopathic orbital inflammation; inflammatory pseudotumor; magnetic resonance imaging; orbital.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Aged
  • Asian People
  • Azathioprine / therapeutic use*
  • Dacryocystitis / etiology*
  • Diplopia / etiology*
  • Female
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / drug therapy*
  • Humans
  • Orbital Diseases / diagnosis
  • Orbital Diseases / drug therapy*
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Azathioprine