Ocular Manifestations of Granulomatosis with Polyangiitis

Cesk Slov Oftalmol. 2019 Spring;74(5):167-174. doi: 10.31348/2018/5/1.


Granulomatosis with polyangiitis (GPA), formerly known as Wegeners granulomatosis, is an autoimmune vasculitis of small vessels, presenting as necrotizing granulomatous inflammation especially of the upper and lower respiratory tract and necrotizing glomerulonephritis. GPA affects more often Caucasians in northern states, predominantly is affected the age-range group of 50 - 60 years. GPA may affect any organ; the eye symptoms are stated in the range of 16-78 %. The eye symptoms are very variable, and in up to 27 % they are the first sign of undiagnosed GPA. The etiology of GPA was not until now explained. Anti-neutrophil cytoplasmic antibodies (ANCA) play important role in the pathogenesis of this disease. GPA is ranked among ANCA associated vasculitis. The GPA is diagnosed on the basis of clinical signs and symptoms of systemic vasculitis, laboratory and histological tests and imaging studies. Immunomodulative therapy made a contribution to the improvement of GPA prognosis in the last decades; biological treatment reaches the prominence of the GPA treatment procedures. Good collaboration with other specialties is necessary for the early diagnosis and treatment of this life and vision threating disease. The ophthalmologist in the collaboration with specialists of other medical branches may take an important part in the GPA diagnostics, monitoring of the diseases course, or adverse affects of the medication. This paper pays attention to the eye symptoms of the GPA; the literature is supplemented with own photographs of GPA eye symptoms in patients followed up at the Department of Ophthalmology, First medical faculty, Charles University and General Faculty Hospital in Prague, Czech Republic, E.U. Key words: Granulomatosis with polyangiitis (GPA), orbit, scleritis, peripheral ulcerative keratitis (PUK), immunomodulation.

Keywords: Granulomatosis with polyangiitis (GPA); immunomodulation; orbit; peripheral ulcerative keratitis (PUK); scleritis.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Czech Republic
  • Eye Diseases* / etiology
  • Granulomatosis with Polyangiitis* / complications
  • Humans
  • Middle Aged
  • Orbit


  • Antibodies, Antineutrophil Cytoplasmic