The eye is a common site of granulomatosis with polyangiitis. A collaborative study

BMC Ophthalmol. 2023 Jan 18;23(1):26. doi: 10.1186/s12886-022-02743-x.

Abstract

Background: Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%).

Methods: Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination.

Results: The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined.

Conclusions: The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.

Keywords: ANCA-associated vasculitis; Episcleritis; Granulomatosis with polyangiitis; Orbital inflammatory disease; Rituximab; Scleritis.

MeSH terms

  • Eye
  • Glucocorticoids / therapeutic use
  • Granulomatosis with Polyangiitis* / complications
  • Granulomatosis with Polyangiitis* / diagnosis
  • Humans
  • Orbital Diseases* / diagnosis
  • Orbital Diseases* / etiology
  • Retrospective Studies
  • Scleritis* / diagnosis
  • Scleritis* / drug therapy
  • Scleritis* / etiology
  • Vision Disorders

Substances

  • Glucocorticoids